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About 16,718 results
Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice G...
https://www.atsjournals.org/doi/10.1164/rccm.201807-1255ST
Raghu, G.

Aug 31st, 2018 - This document provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). It represents a collaborative effort between the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society.

Evaluation of patients with fibrotic interstitial lung disease: A Canadian Thoracic Society ...
https://cts-sct.ca/wp-content/uploads/2018/02/Evaluation-of-pts-w_ILD-paper.pdf
Kerri A. Johannson

Aug 21st, 2017 - Fibrotic ILD represents a large heterogeneous group of disorders that are challenging to diagnose. The evaluation of patients with fibrotic ILD should include a thorough history, physical examination, basic serological testing, pulmonary function tests and HRCT of the chest.

Use of Animal Models for the Preclinical Assessment of Potential Therapies for Pulmonary Fib...
http://www.thoracic.org/statements/resources/interstitial-lung-disease/animal-models-pulm-fibrosis.pdf
R. Gisli Jenkins

Apr 30th, 2017 - Numerous compounds have shown efficacy in limiting development of pulmonary fibrosis using animal models, yet few of these compounds have replicated these beneficial effects in clinical trials. Given the challenges associated with performing clinical trials in patients with idiopathic pulmonary fibrosis (IPF), it is imperative that preclinical data packages be robust in their analyses and inter.

An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary ...
http://www.thoracic.org/statements/resources/interstitial-lung-disease/IPF-Full-length.pdf
Ganesh Raghu

Jul 14th, 2015 - After considering the confidence in effect estimates, the importance of outcomes studied, desirable and undesirable consequences of treatment, cost, feasibility, acceptability of the intervention, and implications to health equity, recommendations were made for or against specific treatment interventions.

Discriminating long myelitis of neuromyelitis optica from sarcoidosis.
https://doi.org/10.1002/ana.24582
Annals of Neurology; Flanagan EP, Kaufmann TJ et. al.

Dec 17th, 2015 - To compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS). We identified adult patients evaluated between 1996 and 2015 with SCS or NMOSD whose first myelitis episode was accompanied by a spinal cord lesion spanning ≥3 vertebral segments. All NMOSD patients were positive for aquaporin-4-immunoglobulin G, and all sarcoidosis...

A randomized, investigator-masked, double-blind, placebo-controlled trial on thalidomid...
https://doi.org/10.1378/chest.14-0015
Chest Droitcourt C, Rybojad M et. al.

Jun 19th, 2014 - Thalidomide use in cutaneous sarcoidosis is based on data from small case series or case reports. The objective of this study was to evaluate the efficacy and safety of thalidomide in severe cutaneous sarcoidosis. This study consisted of a randomized, double-bind, parallel, placebo-controlled, investigator-masked, multicenter trial lasting 3 months and an open-label study from month 3 to month ...

OFEV® (nintedanib) capsules
https://www.ofevhcp.com/

Visit the official HCP website for OFEV (nintedanib), a treatment option for your patients with idiopathic pulmonary fibrosis (IPF), by Boehringer Ingelheim. Adverse reactions may occur. Please see full prescribing information, including patient information, and important safety information on this website.

ESBRIET® (pirfenidone) CAPSULES
https://www.esbriethcp.com/

Find HCP information about Esbriet® (pirfenidone) and idiopathic pulmonary fibrosis, a progressive and irreversible fibrotic interstitial lung disease. Indication Esbriet® (pirfenidone) is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Select Important Safety Information Elevated liver enzymes

Clinical Effectiveness of Antifibrotic Medications for Idiopathic Pulmonary Fibrosis.
https://doi.org/10.1164/rccm.201902-0456OC
American Journal of Respiratory and Critical Care Medicine; Dempsey TM, Sangaralingham LR et. al.

May 31st, 2019 - Rationale: Since their approval, there has been no real-world or randomized trial evidence evaluating the effect of the antifibrotic medications pirfenidone and nintedanib on clinically important outcomes such as mortality and hospitalizations. Objectives: To evaluate the clinical effectiveness of the antifibrotic medications pirfenidone and nintedanib in patients with idiopathic pulmonary fibr...

Nintedanib plus Sildenafil in Patients with Idiopathic Pulmonary Fibrosis.
https://doi.org/10.1056/NEJMoa1811737
The New England Journal of Medicine; Kolb M, Raghu G et. al.

Sep 17th, 2018 - Nintedanib is an approved treatment for idiopathic pulmonary fibrosis (IPF). A subgroup analysis of a previously published trial suggested that sildenafil may provide benefits regarding oxygenation, gas exchange as measured by the diffusion capacity of the lungs for carbon monoxide (DlCO), symptoms, and quality of life in patients with IPF and severely decreased DlCO. That idea was tested in th...

Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoi...
https://doi.org/10.1001/jamaneurol.2018.2295
JAMA Neurology; Stern BJ, Royal W et. al.

Aug 31st, 2018 - The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhanc...

Is 18F-FDG PET/CT useful for the differential diagnosis of solitary pulmonary nodules i...
https://doi.org/10.1007/s12149-018-1273-9
Annals of Nuclear Medicine; Lee SH, Sung C et. al.

Jul 5th, 2018 - Idiopathic pulmonary fibrosis (IPF) is associated with an increased incidence of lung cancer, but patients with IPF often have poor pulmonary function and are vulnerable to pneumothorax and so using an invasive procedure to diagnose a single nodule detected on chest CT risks a critical adverse outcome. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) is...

Clinical Characteristics and Outcome of Hepatic Sarcoidosis: A Population-Based Study 1...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5629110
The American Journal of Gastroenterology; Ungprasert P, Crowson CS et. al.

Sep 5th, 2017 - Data on clinical manifestations and outcome of hepatic sarcoidosis are scarce. This study aimed to use a population-based cohort of patients with incident sarcoidosis to better describe the characteristics of hepatic sarcoidosis. A cohort of incident cases of sarcoidosis in Olmsted County, MN, USA, from 1976 to 2013 was identified from the database. Diagnosis was verified by individual medical ...

Unifying mechanism for different fibrotic diseases.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5422830
Proceedings of the National Academy of Sciences of the United States of America; Wernig G, Chen SY et. al.

Apr 20th, 2017 - Fibrotic diseases are not well-understood. They represent a number of different diseases that are characterized by the development of severe organ fibrosis without any obvious cause, such as the devastating diseases idiopathic pulmonary fibrosis (IPF) and scleroderma. These diseases have a poor prognosis comparable with endstage cancer and are uncurable. Given the phenotypic differences, it was...

Outcome in advanced non-small cell lung cancer patients with successful rechallenge aft...
https://doi.org/10.1007/s00280-017-3261-5
Cancer Chemotherapy and Pharmacology; Kashiwabara K, Semba H et. al.

Mar 4th, 2017 - Several non-small cell lung cancer (NSCLC) cases of successful rechallenge with epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor (TKI) after recovery from gefitinib or erlotinib-induced interstitial lung disease (ILD) have been reported, but it is not clear whether the rechallenge affects the outcome. We retrospectively evaluated the difference in the outcome between advanced N...

A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
https://doi.org/10.1056/NEJMoa1402582
The New England Journal of Medicine; King TE, Bradford WZ et. al.

May 19th, 2014 - In two of three phase 3 trials, pirfenidone, an oral antifibrotic therapy, reduced disease progression, as measured by the decline in forced vital capacity (FVC) or vital capacity, in patients with idiopathic pulmonary fibrosis; in the third trial, this end point was not achieved. We sought to confirm the beneficial effect of pirfenidone on disease progression in such patients. In this phase 3 ...

Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.
https://doi.org/10.1056/NEJMoa1402584
The New England Journal of Medicine; Richeldi L, du Bois RM et. al.

May 19th, 2014 - Nintedanib (formerly known as BIBF 1120) is an intracellular inhibitor that targets multiple tyrosine kinases. A phase 2 trial suggested that treatment with 150 mg of nintedanib twice daily reduced lung-function decline and acute exacerbations in patients with idiopathic pulmonary fibrosis. We conducted two replicate 52-week, randomized, double-blind, phase 3 trials (INPULSIS-1 and INPULSIS-2) ...

Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4116664
The New England Journal of Medicine; , Martinez FJ et. al.

May 19th, 2014 - Acetylcysteine has been suggested as a beneficial treatment for idiopathic pulmonary fibrosis, although data from placebo-controlled studies are lacking. In our initial double-blind, placebo-controlled trial, we randomly assigned patients who had idiopathic pulmonary fibrosis with mild-to-moderate impairment in pulmonary function to receive a three-drug regimen of prednisone, azathioprine, and ...

A practical approach to cutaneous sarcoidosis.
https://doi.org/10.1007/s40257-014-0079-3
American Journal of Clinical Dermatology; Wanat KA, Rosenbach M

May 13th, 2014 - Sarcoidosis is a chronic inflammatory disorder that has the potential to affect multiple organs, including the skin. Its cutaneous manifestations are varied and can provide clues to underlying systemic manifestations. Unfortunately, they also can be disfiguring. Therapy is usually directed at the organ system most severely affected, which often may help cutaneous disease. However, cutaneous dis...

Sirolimus as an alternative treatment in patients with granulomatous-lymphocytic lung d...
https://doi.org/10.1111/pai.12890
Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology; Deyà-Martínez A, Esteve-Solé A et. al.

Mar 13th, 2018 - One of the most frequent non-infectious complications of humoral immunodeficiencies with a CVID-like pattern is a particular form of inflammatory lung disease which is called granulomatous-lymphocytic interstitial lung disease (GLILD). Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and diff...

International management platform for children's interstitial lung disease (chILD-EU).
https://doi.org/10.1136/thoraxjnl-2017-210519
Thorax Griese M, Seidl E et. al.

Oct 23rd, 2017 - Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term...

Filamin A (FLNA) mutation-A newcomer to the childhood interstitial lung disease (ChILD)...
https://doi.org/10.1002/ppul.23695
Pediatric Pulmonology; Shelmerdine SC, Semple T et. al.

Sep 12th, 2017 - Interstitial lung disease (ILD) in infants represents a rare and heterogenous group of disorders, distinct from those occurring in adults. In recent years a new entity within this category is being recognized, namely filamin A (FLNA) mutation related lung disease. Our aims are to describe the clinical and radiological course of patients with this disease entity to aid clinicians in the prognost...

Autoimmune diseases induced by biological agents. A review of 12,731 cases (BIOGEAS Reg...
https://doi.org/10.1080/14740338.2017.1372421
Expert Opinion on Drug Safety; Pérez-De-Lis M, Retamozo S et. al.

Aug 31st, 2017 - Biological drugs are therapies designed to target a specific molecule of the immune system that have been linked with the development of autoimmune diseases. Areas covered: The BIOGEAS Registry currently collects information about nearly 13,000 reported cases of autoimmune diseases developed in patients exposed to biologics, including more than 50 different systemic and organ-specific autoimmun...

HRCT findings of childhood follicular bronchiolitis.
https://doi.org/10.1007/s00247-017-3951-5
Pediatric Radiology; Weinman JP, Manning DA et. al.

Aug 27th, 2017 - Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We foun...

Immune-checkpoint inhibitors associated with interstitial lung disease in cancer patients.
https://doi.org/10.1183/13993003.00050-2017
The European Respiratory Journal; Delaunay M, Cadranel J et. al.

Aug 11th, 2017 - Immunotherapy is becoming a standard of care for many cancers. Immune-checkpoint inhibitors (ICI) can generate immune-related adverse events. Interstitial lung disease (ILD) has been identified as a rare but potentially severe event.Between December 2015 and April 2016, we conducted a retrospective study in centres experienced in ICI use. We report the main features of ICI-ILD with a focus on c...

Lung cancer in lung transplantation: incidence and outcome.
https://doi.org/10.1136/postgradmedj-2017-134868
Postgraduate Medical Journal; Pérez-Callejo D, Torrente M et. al.

Aug 5th, 2017 - Malignancies are one of the causes of mortality after lung transplantation. However, little is known about lung cancer outcome after lung transplantation. We performed a retrospective search of the lung transplantation database at our institution to identify patients diagnosed with lung cancer after lung transplantation. Out of 633 lung transplant patients, lung cancer was detected in 23 of the...

Current approaches to the management of idiopathic pulmonary fibrosis.
https://doi.org/10.1016/j.rmed.2017.05.017
Respiratory Medicine; Raghu G, Richeldi L

Jul 24th, 2017 - Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment ear...

Reliability of Cardiovascular Risk Calculators to Estimate Accurately the Risk of Cardi...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5564423
The American Journal of Cardiology; Ungprasert P, Matteson EL et. al.

Jul 14th, 2017 - Chronic inflammation is an independent risk factor for cardiovascular disease (CVD), but most risk calculators, including the Framingham risk score (FRS) and the American College of Cardiology (ACC)/American Heart Association (AHA) risk score do not account for it. These calculators underestimate cardiovascular risk in patients with rheumatoid arthritis and systemic lupus erythematosus. To date...

Cough and airway disease: The role of ion channels.
https://doi.org/10.1016/j.pupt.2017.06.009
Pulmonary Pharmacology & Therapeutics; Bonvini SJ, Belvisi MG

Jul 3rd, 2017 - Cough is the most common reason for patients to visit a primary care physician, yet it remains an unmet medical need. It can be idiopathic in nature but can also be a troublesome symptom across chronic lung diseases such as asthma, COPD and idiopathic pulmonary fibrosis (IPF). Chronic cough affects up to 12% of the population and yet there are no safe and effective therapies. The cough reflex i...

Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-re...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5493376
PloS One; Enomoto Y, Nakamura Y et. al.

Jun 30th, 2017 - Radiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD). A total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, whi...

Emphysema in active farmer's lung disease.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5470683
PloS One; Soumagne T, Chardon ML et. al.

Jun 14th, 2017 - Farmer's lung (FL) is a common type of hypersensitivity pneumonitis. It is often considered that fibrosis is the most frequent finding in chronic FL. Nevertheless, three cohort studies have suggested that some patients with chronic FL may develop emphysema. We aimed to evaluate the current prevalence of emphysema in active FL, to describe the radiological and functional features of emphysema in...

Cutaneous sarcoidosis.
https://doi.org/10.1097/MCP.0000000000000402
Current Opinion in Pulmonary Medicine; Noe MH, Rosenbach M

Jun 9th, 2017 - Cutaneous sarcoidosis occurs in up to 30% of patients with sarcoidosis and skin findings are often the initial presenting symptom. Cutaneous sarcoidosis is a rare skin disease and many aspects of the disease presentation and treatment are not well understood. This review will highlight developments in the epidemiology, clinical presentation, diagnosis and treatment of cutaneous sarcoidosis over...

Treatment-Related Toxicity in Patients With Early-Stage Non-Small Cell Lung Cancer and ...
https://doi.org/10.1016/j.ijrobp.2017.03.010
International Journal of Radiation Oncology, Biology, Physics; Chen H, Senan S et. al.

Jun 5th, 2017 - Definitive treatment for patients with early-stage non-small cell lung cancer (ES-NSCLC) is usually well tolerated. Patients with ES-NSCLC and coexisting interstitial lung disease (ILD) are at increased risk of severe treatment-related toxicity after definitive therapy. The main objective of this systematic review is to provide a pooled estimation of treatment-related mortality and ILD-specific...

Palisaded neutrophilic and granulomatous dermatitis as a novel cause of hypercalcemia: ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5457875
Medicine Kono M, Hasegawa T et. al.

May 25th, 2017 - Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a benign, inflammatory dermatosis with distinct histopathological features often observed in patients with systemic diseases. There were no reports of PNGD without underlying systemic diseases as an underlying cause of hypercalcemia. Herein, we report a case of a 62-year-old man with hypercalcemia due to PNGD, but with no underlying ...

The role of serum amyloid A staining of granulomatous tissues for the diagnosis of sarc...
https://doi.org/10.1016/j.rmed.2017.03.009
Respiratory Medicine; Huho A, Foulke L et. al.

Apr 21st, 2017 - Previous studies demonstrated that SAA staining of sarcoidosis granulomas was qualitatively and quantitatively different from other granulomatous diseases. These data suggest that positive SAA staining of granulomatous tissue may have adequate specificity to establish a diagnosis of sarcoidosis. Our objective was to determine the diagnostic specificity of SAA staining for sarcoidosis relative t...

Idiopathic pulmonary fibrosis.
https://doi.org/10.1016/S0140-6736(17)30866-8
Lancet (London, England); Richeldi L, Collard HR et. al.

Apr 3rd, 2017 - Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease h...

Pathologic Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensiti...
https://doi.org/10.1016/j.chest.2017.02.011
Chest Wang P, Jones KD et. al.

Feb 22nd, 2017 - The ability of specific histopathologic features to predict mortality or lung transplantation in patients with chronic hypersensitivity pneumonitis (HP) is unknown. Patients with chronic HP diagnosed by surgical lung biopsy were identified from an ongoing longitudinal cohort. The surgical lung biopsy slides were evaluated prospectively by an experienced thoracic pathologist using a standardized...

Proteomic Analysis of Kveim Reagent Identifies Targets of Cellular Immunity in Sarcoido...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5256960
PloS One; Eberhardt C, Thillai M et. al.

Jan 23rd, 2017 - Kveim-reagent (Kv) skin testing was a historical method of diagnosing sarcoidosis. Intradermal injection of treated sarcoidosis spleen tissue resulted in a granuloma response at injection site by 4-6 weeks. Previous work indicates proteins as the possible trigger of this reaction. We aimed to identify Kv-specific proteins and characterise the ex vivo response of Peripheral Blood Mononuclear Cel...

An Exome Sequencing Study to Assess the Role of Rare Genetic Variation in Pulmonary Fib...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5519963
American Journal of Respiratory and Critical Care Medicine; Petrovski S, Todd JL et. al.

Jan 18th, 2017 - Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung disease of unknown etiology. The aim of this study was to use whole-exome sequencing to improve understanding of the genetic architecture of pulmonary fibrosis. We performed a case-control exome-wide collapsing analysis including 262 unrelated individuals with pulmonary fibrosis clinically classified as IPF acco...

FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lu...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5167039
Clinical and Experimental Immunology; Jolles S, Carne E et. al.

Nov 29th, 2016 - Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative...

Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensiti...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6026221
Chest Morisset J, Johannson KA et. al.

Nov 6th, 2016 - The treatment of chronic hypersensitivity pneumonitis (cHP) often includes systemic oral corticosteroids, but the optimal pharmacologic management remains unclear. The morbidity associated with prednisone has motivated the search for alternative therapies. We aimed to determine the effect of treatment with mycophenolate mofetil (MMF) or azathioprine (AZA) on lung function in patients with cHP. ...

Mechanism of granuloma formation in sarcoidosis.
https://doi.org/10.1097/MOH.0000000000000301
Current Opinion in Hematology; Sakthivel P, Bruder D

Oct 18th, 2016 - The formation of noncaseating granuloma is a hallmark of pulmonary sarcoidosis. This review summarizes recent progress made to explain the cellular dynamics within the granuloma structure that may considerably differ between the two clinically distinct variants, that is, acute and chronic sarcoidosis. Compelling evidence exists that in acute but not chronic sarcoidosis CD4 T lymphocytes specifi...

Referral to Palliative Care Infrequent in Patients with Idiopathic Pulmonary Fibrosis A...
https://doi.org/10.1089/jpm.2016.0258
Journal of Palliative Medicine; Liang Z, Hoffman LA et. al.

Oct 18th, 2016 - Palliative care has been recommended as a means to assist patients with idiopathic pulmonary fibrosis (IPF) in managing symptom burden and advanced care planning. Timing of referral is important because although most patients display a gradually progressive course, a minority experience acute deterioration, an outcome associated with high mortality. To describe characteristics of IPF patients r...

Utility of Transbronchial vs Surgical Lung Biopsy in the Diagnosis of Suspected Fibroti...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5310128
Chest Sheth JS, Belperio JA et. al.

Oct 12th, 2016 - Surgical lung biopsy (SLB) is invasive and not possible in all patients with undiagnosed interstitial lung disease (ILD). We hypothesized that transbronchial biopsy (TBB) findings combined with clinical and high-resolution CT (HRCT) data leads to a confident diagnosis congruent to SLB and therefore avoids the need for SLB in some patients. We evaluated 33 patients being investigated for suspect...

Thin-Section CT Features of Idiopathic Pulmonary Fibrosis Correlated with Micro-CT and ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5375628
Radiology Mai C, Verleden SE et. al.

Oct 7th, 2016 - Purpose To elucidate the underlying lung changes responsible for the computed tomographic (CT) features of idiopathic pulmonary fibrosis (IPF) and to gain insight into the way IPF proceeds through the lungs and progresses over time. Materials and Methods Micro-CT studies of tissue cores obtained from explant lungs were examined and were correlated 1:1 with a CT study obtained immediately before...

Improved detection of myocardial damage in sarcoidosis using longitudinal strain in pat...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5523448
Echocardiography (Mount Kisco, N.Y.); Murtagh G, Laffin LJ et. al.

Sep 29th, 2016 - Cardiac infiltration is an important cause of death in sarcoidosis. Transthoracic echocardiography (TTE) has limited sensitivity for the detection of cardiac sarcoidosis (CS). Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is used to diagnose CS but has limitations of cost and availability. We sought to determine whether TTE-derived global longitudinal strain (GLS) ma...

Presence of Late Gadolinium Enhancement by Cardiac Magnetic Resonance Among Patients Wi...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5449111
Circulation. Cardiovascular Imaging; Hulten E, Agarwal V et. al.

Sep 13th, 2016 - Individuals with cardiac sarcoidosis have an increased risk of ventricular arrhythmia and death. Several small cohort studies have evaluated the ability of late gadolinium enhancement (LGE) by cardiac magnetic resonance imaging (MRI) to predict adverse cardiovascular events. However, studies have yielded inconsistent results, and some analyses were underpowered. Therefore, we sought to systemat...

Cardiac Sarcoidosis: The Impact of Age and Implanted Devices on Survival.
https://doi.org/10.1016/j.chest.2016.08.1457
Chest Zhou Y, Lower EE et. al.

Sep 10th, 2016 - To assess the clinical characteristics, diagnosis, and outcome of cardiac sarcoidosis in a single institution sarcoidosis clinic. Patients with cardiac sarcoidosis were identified using refined World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria of highly probable and probable. Patient demographics, local and systemic treatments, and clinical outcome were collecte...

Sonographic Features of Endobronchial Ultrasound in Differentiation of Benign Lymph Nodes.
https://doi.org/10.1016/j.ultrasmedbio.2016.07.019
Ultrasound in Medicine & Biology; Wang L, Wu W et. al.

Sep 10th, 2016 - Endobronchial ultrasound (EBUS) features have been found to be a useful tool in differentiating malignant from benign lymph nodes, but the use of these features to distinguish benign intrathoracic lymphadenopathies, including tuberculosis, sarcoidosis and reactive lymphadenitis, has not been established. The goal of this study was to evaluate the use of EBUS features in predicting tuberculosis,...

Single-specificity anti-Ku antibodies in an international cohort of 2140 systemic scler...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5008592
Medicine Hoa S, Hudson M et. al.

Sep 1st, 2016 - Autoantibodies directed against the Ku autoantigen are present in systemic sclerosis (SSc) and have been associated with myositis overlap and interstitial lung disease (ILD). However, there is a paucity of data on the clinical correlates of anti-Ku antibodies in the absence of other SSc-specific antibodies. The aim of this study was to assess the clinical correlates of single-specificity anti-K...

Hermansky-Pudlak Syndrome.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4987498
Clinics in Chest Medicine; El-Chemaly S, Young LR

Aug 15th, 2016 - Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diatheses, granulomatous colitis, and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis shows many of the clinical, radiologic, and histologic features found in idiopathic pulmonary fibrosis, but occurs at a yo...

Immunological Evidence for the Role of Mycobacteria in Sarcoidosis: A Meta-Analysis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4968811
PloS One; Fang C, Huang H et. al.

Aug 1st, 2016 - Sarcoidosis is a granulomatous disease, the etiology of which is currently unknown. The role of mycobacteria in the etiology of sarcoidosis has been extensively investigated. In this meta-analysis, we assessed the immunological evidence of the possible role of mycobacteria in the pathogenesis and development of sarcoidosis. We performed a systematic search of relevant articles from PubMed, Emba...

Hypersensitivity Pneumonitis Due to Metalworking Fluid Aerosols.
https://doi.org/10.1007/s11882-016-0639-0
Current Allergy and Asthma Reports; Burge PS

Jul 30th, 2016 - This review summarises the clinical knowledge of hypersensitivity pneumonitis in workers exposed to aerosols of metalworking fluid, reviewing published outbreaks and clinical cases. Metalworking fluid exposure has become the commonest recognised cause of occupational hypersensitivity pneumonitis, having been rare before 2000. There are many possible agents in the metalworking fluid which may be...

Diagnostic Yield and Complications of Transbronchial Lung Cryobiopsy for Interstitial L...
https://doi.org/10.1513/AnnalsATS.201606-461SR
Annals of the American Thoracic Society; Johannson KA, Marcoux VS et. al.

Jul 28th, 2016 - The diagnostic usefulness and safety of transbronchial lung cryobiopsy for the evaluation of interstitial lung disease remain unclear. This systematic review and metaanalysis aims to establish the diagnostic accuracy and yield of transbronchial cryobiopsy for interstitial lung diseases. We searched MedLine, EMBASE, Cochrane Central Register of Controlled Trials, and conference proceedings to id...

Prognostic Value of Myocardial Scarring on CMR in Patients With Cardiac Sarcoidosis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5237422
JACC. Cardiovascular Imaging; Coleman GC, Shaw PW et. al.

Jul 27th, 2016 - This study sought to perform a systematic review and meta-analysis to understand the prognostic value of myocardial scarring as evidenced by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging in patients with known or suspected cardiac sarcoidosis. Although CMR is increasingly used for the diagnosis of cardiac sarcoidosis, the prognostic value of CMR has been less wel...

Prevalence and Correlates of Early Right Ventricular Dysfunction in Sarcoidosis and Its...
https://doi.org/10.1016/j.echo.2016.06.001
Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography; Joyce E, Kamperidis V et. al.

Jul 27th, 2016 - Right ventricular (RV) function has not been systematically assessed in sarcoidosis. The aim of this study was to assess the prevalence and associates of RV dysfunction in sarcoidosis using global longitudinal peak systolic strain (GLS). Furthermore, whether RV dysfunction was associated with clinical outcomes was investigated. A total of 88 patients with sarcoidosis (mean age, 54 ± 13 years; 5...

T-cell immunology in sarcoidosis: Disruption of a delicate balance between helper and r...
https://doi.org/10.1097/MCP.0000000000000303
Current Opinion in Pulmonary Medicine; Broos CE, Hendriks RW et. al.

Jul 5th, 2016 - Although the aetiology of sarcoidosis is not yet completely understood, immunological changes within the T-cell compartment are characteristic for an exaggerated antigen-driven immune response. In this review, we describe the most recent findings on T-cell subset responses and regulation in sarcoidosis. We discuss how future immunological research can advance the field to unravel pathobiologica...

A novel surfactant protein C gene mutation associated with progressive respiratory fail...
https://doi.org/10.1002/ppul.23493
Pediatric Pulmonology; Litao MK, Hayes D et. al.

Jun 30th, 2016 - Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung disease, and outcomes. We report a novel mutation in SFTPC [c.435G->A, p.(Gln145)] that was associated with onset of symptoms in early infancy, progressive respiratory failure with need for prolonged mechanical ventilatory su...

Impact of idiopathic pulmonary fibrosis on advanced non-small cell lung cancer survival.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4954838
Journal of Cancer Research and Clinical Oncology; Kanaji N, Tadokoro A et. al.

Jun 28th, 2016 - The clinical features of patients with advanced non-small cell lung cancer (NSCLC) and interstitial lung disease (ILD) have not fully been elucidated. This study aimed to investigate the clinical features of these patients, particularly with idiopathic pulmonary fibrosis (IPF). Data on 218 patients with pathologically confirmed diagnoses of NSCLC who had been treated with chemotherapy and/or mo...

Orofacial granulomatosis.
https://doi.org/10.1016/j.clindermatol.2016.02.024
Clinics in Dermatology; Miest R, Bruce A et. al.

Jun 27th, 2016 - Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition with a multifactorial etiology and pathogenesis. Genetic, immunologic, allergic, and infectious mechanisms have been implicated. OFG is often used as a descriptor to encompass all entities with orofacial swelling and histologic evidence of noncaseating granulomas. The diagnosis of OFG should prompt evaluation for prov...

Risk of hypersensitivity pneumonitis and interstitial lung diseases among pigeon breeders.
https://doi.org/10.1183/13993003.00376-2016
The European Respiratory Journal; Cramer C, Schlünssen V et. al.

May 27th, 2016 - We studied the risk of hypersensitivity pneumonitis and other interstitial lung diseases (ILDs) among pigeon breeders.This is a retrospective follow-up study from 1980 to 2013 of 6920 pigeon breeders identified in the records of the Danish Racing Pigeon Association. They were compared with 276 800 individually matched referents randomly drawn from the Danish population. Hospital based diagnoses...

The clinical results of proton beam therapy in patients with idiopathic pulmonary fibro...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4835903
Radiation Oncology (London, England); Ono T, Hareyama M et. al.

Apr 19th, 2016 - The purpose of this study is to retrospectively evaluate the incidence of lung toxicities after proton beam therapy (PBT) in patients with idiopathic pulmonary fibrosis (IPF). Patients diagnosed with primary lung cancer or lung metastasis who were treated with PBT between January 2009 and May 2015 were recruited from our database retrospectively. Cases of pneumonitis (excluding infection-relate...

Severe radiation pneumonitis after lung stereotactic ablative radiation therapy in pati...
https://doi.org/10.1016/j.prro.2016.01.009
Practical Radiation Oncology; Bahig H, Filion E et. al.

Apr 13th, 2016 - To investigate the incidence and predictive factors of severe radiation pneumonitis (RP) after stereotactic ablative radiation therapy (SABR) in early-stage lung cancer patients with preexisting radiological interstitial lung disease (ILD). A retrospective analysis of patients with stage I lung cancer treated with SABR from 2009 to 2014 was conducted. Interstitial lung disease diagnosis and gra...

Clinical Predictors of a Diagnosis of Common Variable Immunodeficiency-related Granulom...
https://doi.org/10.1513/AnnalsATS.201511-728OC
Annals of the American Thoracic Society; Mannina A, Chung JH et. al.

Apr 11th, 2016 - Granulomatous-lymphocytic interstitial lung disease (GLILD) has emerged as a major cause of morbidity in patients with common variable immunodeficiency (CVID). While GLILD is among the most serious noninfectious pulmonary complications of CVID, risk factors for this condition have not been reported. To identify clinical, physiologic, and serologic risk factors for GLILD in adults with CVID. Of ...

Linear endobronchial and endoesophageal ultrasound: a practice change in thoracic medic...
https://doi.org/10.1097/MCP.0000000000000269
Current Opinion in Pulmonary Medicine; Bonta PI, Crombag L et. al.

Mar 18th, 2016 - Linear endosonography, including intrathoracic lymph nodal sampling by endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) and endoesophageal ultrasound fine-needle aspiration (EUS-FNA), has an important role in the diagnosing and staging of lung cancer. Furthermore, endosonography is applied in the mediastinal evaluation of sarcoidosis, lymphoma, cysts, and nodal metasta...

Occupational hypersensitivity pneumonitis: an EAACI position paper.
https://doi.org/10.1111/all.12866
Allergy Quirce S, Vandenplas O et. al.

Feb 25th, 2016 - The aim of this document was to provide a critical review of the current knowledge on hypersensitivity pneumonitis caused by the occupational environment and to propose practical guidance for the diagnosis and management of this condition. Occupational hypersensitivity pneumonitis (OHP) is an immunologic lung disease resulting from lymphocytic and frequently granulomatous inflammation of the pe...

Hypersensitivity pneumonitis and antigen identification--An alternate approach.
https://doi.org/10.1016/j.rmed.2015.09.001
Respiratory Medicine; Millerick-May ML, Mulks MH et. al.

Feb 2nd, 2016 - Identification of the causal antigen for patients with hypersensitivity pneumonitis (HP) is challenging in a standard clinical setting. The purpose of this pilot study was to determine whether it was possible to evaluate the home/workplace of patients, and identify the causal antigen. Using a case-control study design we compared the presence of antibody to antigen collected in the environment ...

Chronic Hypersensitivity Pneumonitis With a Usual Interstitial Pneumonia-Like Pattern: ...
https://doi.org/10.1016/j.chest.2015.12.030
Chest Chiba S, Tsuchiya K et. al.

Feb 2nd, 2016 - Hypersensitivity pneumonitis (HP) is an interstitial lung disease caused by the inhalation of environmental antigens. The relationship between clinical, radiologic, and histopathologic findings of chronic HP remains unclear. Sixteen patients with proven chronic bird-related HP with a usual interstitial pneumonia-like pattern were analyzed retrospectively. Histopathologic findings were semiquant...

TSLP Is a Potential Initiator of Collagen Synthesis and an Activator of CXCR4/SDF-1 Axi...
https://doi.org/10.1016/j.jid.2015.11.008
The Journal of Investigative Dermatology; Shin JU, Kim SH et. al.

Jan 29th, 2016 - Recently, thymic stromal lymphopoietin (TSLP), which is well studied in allergic diseases, has been reported in fibrotic diseases, including idiopathic pulmonary fibrosis and atopic dermatitis fibrosis. However, the role of TSLP in keloid is obscure. In this study, we assessed the expression of TSLP in keloid tissue and investigated the possible role of TSLP in keloid pathogenesis. We observed ...

Molecular Analysis of Sarcoidosis Granulomas Reveals Antimicrobial Targets.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942207
American Journal of Respiratory Cell and Molecular Biology; Rotsinger JE, Celada LJ et. al.

Jan 25th, 2016 - Sarcoidosis is a granulomatous disease of unknown cause. Prior molecular and immunologic studies have confirmed the presence of mycobacterial virulence factors, such as catalase peroxidase and superoxide dismutase A, within sarcoidosis granulomas. Molecular analysis of granulomas can identify targets of known antibiotics classes. Currently, major antibiotics are directed against DNA synthesis, ...

Germline SFTPA1 mutation in familial idiopathic interstitial pneumonia and lung cancer.
https://doi.org/10.1093/hmg/ddw014
Human Molecular Genetics; Nathan N, Giraud V et. al.

Jan 22nd, 2016 - Idiopathic interstitial pneumonias (IIPs) comprise a heterogeneous group of rare lung parenchyma disorders with high morbidity and mortality, which can occur at all ages. In adults, the most common form of IIPs, idiopathic pulmonary fibrosis (IPF), has been associated with an increased frequency of lung cancer. The molecular basis of IIPs remains unknown in most cases. This study investigates I...

Palliative Care for Patients Dying in the Intensive Care Unit with Chronic Lung Disease...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018894
Annals of the American Thoracic Society; Brown CE, Engelberg RA et. al.

Jan 19th, 2016 - Palliative care has been focused largely on patients with cancer, and yet patients with chronic lung diseases also have high morbidity and mortality. The majority of deaths in intensive care units (ICUs) follow decisions to withhold or withdraw life-sustaining treatments, suggesting that palliative care is critically important in this setting. We explored differences in receipt of elements of p...

Right Heart Involvement in Patients with Sarcoidosis.
https://doi.org/10.1111/echo.13163
Echocardiography (Mount Kisco, N.Y.); Patel MB, Mor-Avi V et. al.

Jan 16th, 2016 - The left ventricle (LV) is affected in 20-25% of patients with sarcoidosis and its involvement is associated with morbidity and mortality. However, effects of sarcoidosis on the right ventricle (RV) are not well documented. Our aims were to investigate the prevalence of RV dysfunction in patients with sarcoidosis and determine whether it is predominantly associated with direct cardiac involveme...

Prognosis of Myocardial Damage in Sarcoidosis Patients With Preserved Left Ventricular ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4718184
Circulation. Cardiovascular Imaging; Murtagh G, Laffin LJ et. al.

Jan 14th, 2016 - Cardiac sarcoidosis is associated with an increased risk of heart failure and sudden death, but its risk in patients with preserved left ventricular ejection fraction is unknown. Using cardiovascular magnetic resonance in patients with extracardiac sarcoidosis and preserved left ventricular ejection fraction, we sought to (1) determine the prevalence of cardiac sarcoidosis or associated myocard...

Epidemiology of Sarcoidosis 1946-2013: A Population-Based Study.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4744129
Mayo Clinic Proceedings; Ungprasert P, Carmona EM et. al.

Jan 4th, 2016 - To characterize the epidemiology of sarcoidosis from 1946 through 2013. An inception cohort of patients with incident sarcoidosis from January 1, 1976, through December 31, 2013, in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Inclusion required physician diagnosis supported by histopathologic confirmation, radiologic features of intrathorac...

Combined pulmonary fibrosis and emphysema in hypersensitivity pneumonitis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4691920
BMJ Case Reports; Soumagne T, Pana-Katatali H et. al.

Dec 22nd, 2015 - Combined pulmonary fibrosis and emphysema is a distinct syndrome reported in patients who smoke. A 72-year-old, never-smoking female dairy farmer was referred for progressive dyspnoea on exertion, basal crackles on auscultation, normal spirometry and normal lung volumes but decreased diffusing capacity of the lung for carbon monoxide, centrilobular emphysema in the upper zones of the lungs and ...

IFN-γ-Producing T-Helper 17.1 Cells Are Increased in Sarcoidosis and Are More Prevalent...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4910899
American Journal of Respiratory and Critical Care Medicine; Ramstein J, Broos CE et. al.

Dec 9th, 2015 - Pulmonary sarcoidosis is classically defined by T-helper (Th) cell type 1 inflammation (e.g., IFN-γ production by CD4(+) effector T cells). Recently, IL-17A-secreting cells have been found in lung lavage, invoking Th17 immunity in sarcoidosis. Studies also identified IL-17A-secreting cells that expressed IFN-γ, but their abundance as a percentage of total CD4(+) cells was either low or undeterm...

Pulmonary Sarcoidosis.
https://doi.org/10.1016/j.ccm.2015.08.006
Clinics in Chest Medicine; Valeyre D, Bernaudin JF et. al.

Nov 25th, 2015 - Sarcoidosis is a systemic disease, with lung involvement in almost all cases. Abnormal chest radiography is usually a key step for considering diagnosis. Lung impact is investigated through imaging; pulmonary function; and, when required, 6-minute walk test, cardiopulmonary exercise testing, or right heart catheterization. There is usually a reduction of lung volumes, and forced vital capacity ...

The Diagnosis of Sarcoidosis.
https://doi.org/10.1016/j.ccm.2015.08.003
Clinics in Chest Medicine; Govender P, Berman JS

Nov 25th, 2015 - Sarcoidosis is a diagnosis of exclusion; there exists neither a pathognomonic clinical feature nor a perfect diagnostic test. Missed diagnosis and overdiagnosis are common. A careful history and physical examination look for "footprints" of sarcoidosis or features suggesting alternative diagnoses. Some presentations are classic and do not require tissue confirmation. A tissue biopsy should be p...

T-cell receptor-HLA-DRB1 associations suggest specific antigens in pulmonary sarcoidosis.
https://doi.org/10.1183/13993003.01209-2015
The European Respiratory Journal; Grunewald J, Kaiser Y et. al.

Nov 20th, 2015 - In pulmonary sarcoidosis, CD4(+) T-cells expressing T-cell receptor Vα2.3 accumulate in the lungs of HLA-DRB1*03(+) patients. To investigate T-cell receptor-HLA-DRB1*03 interactions underlying recognition of hitherto unknown antigens, we performed detailed analyses of T-cell receptor expression on bronchoalveolar lavage fluid CD4(+) T-cells from sarcoidosis patients.Pulmonary sarcoidosis patien...

Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary ...
https://doi.org/10.1164/rccm.201504-0711OC
American Journal of Respiratory and Critical Care Medicine; Tomassetti S, Wells AU et. al.

Nov 12th, 2015 - Surgical lung biopsy is often required for a confident multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF). Alternative, less-invasive biopsy methods, such as bronchoscopic lung cryobiopsy (BLC), are highly desirable. To address the impact of BLC on diagnostic confidence in the multidisciplinary diagnosis of IPF. In this cross-sectional study we selected 117 patients with fibroti...

Prognostic value of skin lesions in sarcoidosis: clinical and histopathological clues.
https://doi.org/10.1684/ejd.2015.2666
European Journal of Dermatology : EJD; Esteves TC, Aparicio G et. al.

Nov 10th, 2015 - Although tremendous advances have been made, a significant gap exists between the vast knowledge accumulated concerning sarcoidosis in recent years and our understanding of this disease. Describe the main clinical and histopathological findings associated with cutaneous sarcoidosis and to investigate the relationship of these skin lesions with systemic involvement. A retrospective review of 41 ...

Epidemiology of Sarcoidosis in a Prospective Cohort Study of U.S. Women.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4722845
Annals of the American Thoracic Society; Dumas O, Abramovitz L et. al.

Oct 26th, 2015 - Sarcoidosis is a systemic granulomatous and inflammatory disorder that most often involves the lungs but also affects many other organs. Data on sarcoidosis from large epidemiological studies remain scarce. To evaluate the baseline prevalence and 22-year incidence of sarcoidosis and their associations with demographic and geographic characteristics in a large cohort of U.S. women. The Nurses' H...

Multidisciplinary Approach to Hypersensitivity Pneumonitis.
https://doi.org/10.1097/RTI.0000000000000186
Journal of Thoracic Imaging; Elicker BM, Jones KD et. al.

Oct 19th, 2015 - Hypersensitivity pneumonitis (HP) may be a challenging diagnosis given the wide variability of its clinical, radiographic, and pathologic manifestations. A multidisciplinary approach to diagnosis is critical in maintaining a high specificity for HP. An in-depth knowledge of all 3 arms of the multidisciplinary approach helps clinicians, radiologists, and pathologists interpret their own findings...

Prostacyclin and oral vasodilator therapy in sarcoidosis-associated pulmonary hypertens...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4594624
Chest Bonham CA, Oldham JM et. al.

Oct 6th, 2015 - It is unclear whether recent advances in pulmonary arterial hypertension therapy can be safely applied to sarcoidosis-associated pulmonary hypertension (SAPH). Evidence for prostacyclin (PG) therapy in SAPH is limited. We conducted a single-center, retrospective review of 46 patients with sarcoidosis, 26 of whom had SAPH. Thirteen received PG as monotherapy or in combination with oral vasodilat...

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review.
https://doi.org/10.1183/13993003.02316-2014
The European Respiratory Journal; Raghu G, Amatto VC et. al.

Oct 1st, 2015 - Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies p...

Mycobacterium gordonae-induced humidifier lung.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4589115
BMC Pulmonary Medicine; Utsugi H, Usui Y et. al.

Sep 30th, 2015 - Nontuberculous mycobacteria are well known to be a cause of hot tub lung, however, to our knowledge, there exists no case report of humidifier lung induced by mycobacteria. A case of a nonimmunocompromised female patient with Mycobacterium gordonae-induced humidifier lung is described. She spontaneously recovered after discontinuing ultrasonic humidifier use. When subjected to a provocation tes...

Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4864500
Arthritis Care & Research; Moghadam-Kia S, Oddis CV et. al.

Sep 28th, 2015 - Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) presenting with the characteristic rash of DM without objective muscle weakness. Asian studies report that anti-melanoma differentiation-associated gene 5 (anti-MDA-5) autoantibody in CADM is associated with interstitial lung disease (ILD), particularly rapidly progressive ILD (RPILD). These associations have not b...

Chemical determinants of occupational hypersensitivity pneumonitis.
https://doi.org/10.1093/occmed/kqv143
Occupational Medicine (Oxford, England); Seed MJ, Enoch SJ et. al.

Sep 26th, 2015 - Workplace inhalational exposures to low molecular weight (LMW) chemicals cause hypersensitivity pneumonitis (HP) as well as the more common manifestation of respiratory hypersensitivity, occupational asthma (OA). To explore whether chemical causation of HP is associated with different structural and physico-chemical determinants from OA. Chemical causes of human cases of HP and OA were identifi...

Fusariosis.
https://doi.org/10.1055/s-0035-1562897
Seminars in Respiratory and Critical Care Medicine; Nucci F, Nouér SA et. al.

Sep 23rd, 2015 - Fusarium species are frequent agents of onychomycosis and fungal keratitis, and occasional agents of invasive disease. The clinical spectrum of fusariosis in the lungs includes allergic disease (allergic bronchopulmonary fusariosis), hypersensitivity pneumonitis, colonization of a preexisting cavity, and pneumonia. Fusarial pneumonia occurs almost exclusively in severely immunocompromised patie...

The wide-ranging clinical implications of the short telomere syndromes.
https://doi.org/10.1111/imj.12868
Internal Medicine Journal; Barbaro PM, Ziegler DS et. al.

Aug 7th, 2015 - There is an increasing number of inherited disorders in which excessive telomere shortening underlies the molecular defect, with dyskeratosis congenita (DC) being the archetypal short telomere syndrome. DC is classically described as a mucocutaneous triad of oral leukoplakia, nail dystrophy and abnormal skin pigmentation. However, excessive telomere shortening can affect almost any organ system...

Management of Systemic-Sclerosis-Associated Interstitial Lung Disease.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4515778
Rheumatic Diseases Clinics of North America; Silver KC, Silver RM

Jul 26th, 2015 - Although scleroderma-associated interstitial lung disease (SSc-ILD) is a significant contributor to both morbidity and mortality, its pathogenesis is largely unclear. Pulmonary function tests and high-resolution computed tomographic scanning continue to be the most effective tools to screen for lung involvement and to monitor for disease progression. More research and better biomarkers are need...

Hepatopulmonary syndrome is a frequent cause of dyspnea in the short telomere disorders.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4594621
Chest Gorgy AI, Jonassaint NL et. al.

Jul 14th, 2015 - Telomere syndromes have their most common manifestation in idiopathic pulmonary fibrosis and emphysema. The short telomere defect in these patients may manifest systemically as bone marrow failure and liver disease. We sought to understand the causes of dyspnea in telomerase and telomere gene mutation carriers who have no parenchymal lung disease. Clinical and pathologic data were reviewed as p...

Granuloma genes in sarcoidosis: what is new?
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4569001
Current Opinion in Pulmonary Medicine; Fischer A, Rybicki BA

Jul 14th, 2015 - Nonnecrotizing granulomas in the affected organ are the hallmark of sarcoidosis. This review summarizes most recent genetic findings in sarcoidosis with a focus on genes that might influence granuloma formation or resolution. Specific results in multiple ethnic groups and certain clinical subphenotypes, such as extra-pulmonary organ involvement, are discussed. Associations of genetic variants i...

Sarcoidosis.
https://doi.org/10.1016/j.det.2015.03.006
Dermatologic Clinics; Sanchez M, Haimovic A et. al.

Jul 5th, 2015 - Sarcoidosis is a disease characterized by noncaseating granulomatous infiltration of 1 or more organs. In North America, after the lungs and thoracic lymph nodes, the skin is the next most commonly involved organ. Data from multiple studies indicate a coaction between genetic and environmental factors in immunologically susceptible hosts. The disease's many clinical manifestations and course va...

The amount of avian antigen in household dust predicts the prognosis of chronic bird-re...
https://doi.org/10.1513/AnnalsATS.201412-569OC
Annals of the American Thoracic Society; Tsutsui T, Miyazaki Y et. al.

May 26th, 2015 - Bird-related hypersensitivity pneumonitis is induced by inhalation of avian antigen. Evaluation to avoid repeated exposure to avian antigen is a key part of the treatment for bird-related hypersensitivity pneumonitis. It can be difficult, however, to reliably evaluate exposure to the antigen because bird-related hypersensitivity pneumonitis in its chronic form may be caused by unrecognized and ...

Immunology of IgG4-related disease.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4516435
Clinical and Experimental Immunology; Della-Torre E, Lanzillotta M et. al.

Apr 13th, 2015 - Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4(+) plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has rem...

Lung sarcoidosis in children: update on disease expression and management.
https://doi.org/10.1136/thoraxjnl-2015-206825
Thorax Nathan N, Marcelo P et. al.

Apr 9th, 2015 - Sarcoidosis is a rare lung disease in children. The aim of the present study was to provide update information on disease presentation and progression, patient management and prognosis factors in a cohort of children with lung sarcoidosis. With the network of the French Reference Centre for Rare Lung Diseases (RespiRare), we collected information on a large cohort of paediatric thoracic sarcoid...

Etiologies of Sarcoidosis.
https://doi.org/10.1007/s12016-015-8481-z
Clinical Reviews in Allergy & Immunology; Chen ES, Moller DR

Mar 16th, 2015 - Since sarcoidosis was first described more than a century ago, the etiologic determinants causing this disease remain uncertain. Studies suggest that genetic, host immunologic, and environmental factors interact together to cause sarcoidosis. Immunologic characteristics of sarcoidosis include non-caseating granulomas, enhanced local expression of T helper-1 (and often Th17) cytokines and chemok...

Pathology of Sarcoidosis.
https://doi.org/10.1007/s12016-015-8479-6
Clinical Reviews in Allergy & Immunology; Rossi G, Cavazza A et. al.

Mar 12th, 2015 - Pathologists are frequently involved in the diagnosis of sarcoidosis on conventional biopsies or examining bronchoalveolar lavage fluid and assisting bronchoscopists when performing bronchial or transbronchial biopsies or transbronchial needle aspiration (TBNA)/endobronchial ultrasound (EBUS)-guided biopsies of enlarged lymph nodes. Histology generally does not pose difficult tasks in the corre...