Under Pressure: An Update on Pulmonary Hypertension
The Rheumatologist; Liebowitz J.

Nov 8th, 2020 - In patients with connective tissue diseases, such as systemic sclerosis (SSc), pulmonary hypertension (PH) remains one of the leading causes of morbidity and mortality. In her lecture on Friday, Nov. 6, Pulmonary Hypertension: An Update, Mardi Gomberg-Maitland, MD, MSc, professor of medicine and medical director of the Pulmonary Hypertension Program at The George Washington School of Medicine &...

Characterizing Morphea Subsets Using a Multi-center, Prospective, Cross-sectional Analysis
Jane Zhu, Smriti Prasad, Kaila Schollaert-Fitch et. al.

Nov 9th, 2020 - Background/Purpose: Morphea, or localized scleroderma, is an inflammatory condition of the skin and soft tissue that results in excessive collagen deposition, often producing permanent functional and cosmetic impairment. Morphea affects both adults and children, with approximately 50% of patients having childhood onset disease1,2. Numerous classification schemes for morphea exist, but none have...

Curdlan-induced Villous Permeability, Luminal Pathobiont Translocation to the Ileal Crypts, Ileitis and Arthritis Are Mitigated by Clostridia in Colonised Germ-free SKG Mice
Anne-Sophie Bergot, Rabina Giri, Amy Cameron et. al.

Nov 9th, 2020 - Background/Purpose: IL-23 dependent spondylitis, arthritis and inflammatory bowel disease (IBD) recapitulate human spondyloarthropathy (SpA) in 1,3 beta glucan (curdlan)-treated SKG mice. Human SpA and SKG mice have fecal dysbiosis, with increased Bacteroidaceae and Porphyromonadaceae. In SKG mice, anti-IL-23 supports homeostatic bacteria, including Clostridiaceae, which produce the short chain...

Comparison of Two Rituximab Regimens for Induction of Remission in Antineutrophil Cytoplasm Antibody-associated Vasculitis: Systematic Review and Meta-analysis
Valerie Benard, Cynthia Farhat, Melissa Zarandi-Nowroozi et. al.

Nov 9th, 2020 - Background/Purpose: Organ or life-threatening granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two of the antineutrophil cytoplasm antibody-associated vasculitis (AAV), are treated with cyclophosphamide or rituximab (RTX) in combination with glucocorticoids. Different RTX regimens for induction of remission are available: the 4-dose AAV regimen at doses of 375 mg/m2 I....

Cutaneous Side Effects of Hydroxychloroquine in Rheumatic Diseases –Combination of “Traditional” Multivariate Analysis for Risk Factors AndClassification Model Development Using Supervised Machine ...
Sandeep Surendran, Mithun CB, Arun Tiwari et. al.

Nov 9th, 2020 - Background/Purpose: Hydroxychloroquine (HCQ) is associated with varied cutaneous side effects but only few studies in literature characterizing the risk factors for this. Recently machine learning tools have emerged as a promising option for predicting various outcomes. The aim of our study was to use “traditional” multivariate analysis along with novel machine learning (ML) methodology to anal...

Characterization of Cytokine/chemokine Profile in Patient-derived M1/ M2 Macrophages to Identify Biomarkers for Genetically-defined Systemic Autoinflammatory Diseases
Farzana Bhuyan, Adriana Almeida de Jesus, Kim Johnson et. al.

Nov 9th, 2020 - Background/Purpose: Genetic mutations in key regulatory molecules of the innate immune system cause autoinflammatory diseases through propagation of hyperinflammatory responses. Monocytes/ macrophages regulate inflammatory processes during infection and homeostasis by secreting a wide range of cytokines and chemokines that control recruitment and migration of immune cells to inflamed tissue, an...

Comparison of Nation-wide Epidemical Study on 2009 and That on 2019 Revealed That Improvement of Disease Severity and Mortality Rate May Come from Progress of Proficient Management in Patients with...
Jun Shimizu, Yoshihisa Yamano, Kimito Kawahata2 and Noboru Suzuki et. al.

Nov 9th, 2020 - Background/Purpose: We conducted a retrospective survey study in 2009 and collected clinical data of 239 patients with relapsing polychondritis (RP). Using the survey data, we found a strong inverse relationship between ear and airway involvement, suggesting that the two major clinical symptoms of RP occurred in a mutually exclusive manner (Arthritis Rheumatol. 2018). When we divided the patien...

Assessing Patient Self-Reported Transition Readiness in a Large Pediatric Rheumatology Center
Alexander Alexander, Mary Robichaux, Priyanka Moolchandani et. al.

Nov 9th, 2020 - Background/Purpose: Pediatric rheumatology patients need effective transition from pediatric to adult providers. Texas Children’s Hospital (TCH) rheumatology clinic providers have developed a transition pathway, the BRIDGE (Baylor Rheumatology Initiative: Developing and Guiding Engagement), based on defined elements of transition and utilizing an electronic medical record-based transition plann...

Activated Memory T Cells Produce Ligands That Cause NF-κB-dependent Inflammatory Activation of the Endothelium: Identification of Novel Therapeutic Targets
Kim Jeucken, Jan Piet van Hamburg, Lotte Kocken1 and Sander Tas et. al.

Nov 9th, 2020 - Background/Purpose: Endothelial cells (EC) are important contributors to inflammation via expression of inflammatory mediators, including cytokines, chemokines and adhesion molecules. Production of these inflammatory mediators can be induced via canonical and NF-κB-inducing kinase (NIK)-dependent noncanonical NF-κB signalling. The ligands activating these pathways are well studied, but less is ...

Anti-Melanoma Differentiation Associated Protein 5 (MDA5) Positive Juvenile Dermatomyositis: Focus on the Lung
David Moreno McNeill, Miriah Gillispie-Taylor, Kevin Baszis et. al.

Nov 9th, 2020 - Background/Purpose: A subset of juvenile dermatomyositis (JDM), clinically amyopathic dermatomyositis (CADM), is uncommon and mainly described in adults. CADM is classically characterized by skin ulcerations, minimal muscle involvement, and interstitial lung disease (ILD). Anti-MDA5 antibodies are associated with CADM and ILD. A subset of anti-MDA5 patients have rapidly progressive ILD (RP-ILD)...

Assessing Preparation for Care Transition Among Adolescents with Rheumatologic Disease: A Quality Assessment with Patient Survey
Jordan Roberts, Olha Halyabar, Carter Petty3 and Mary Beth Son et. al.

Nov 9th, 2020 - Background/Purpose: Despite the risk for poor outcomes and gaps in care in the transition from pediatric to adult care, most pediatric rheumatology centers lack formal pathways for transition. We evaluated preparation for transition with a validated quality measure in a single pediatric rheumatology center. Methods: Patients 16 and older were sent the ADolescent Assessment of Preparation for Tr...

An Artificial Intelligence (AI) Assistant Identifying Spinal Diffuse Idiopathic Skeletal Hyperostosis on Plain X-rays: A Pilot Deep Learning Study
Sarah Ringsted, Nishad Sathe, Atul Deodhar2 and Dongseok Choi et. al.

Nov 9th, 2020 - Background/Purpose: Diffuse idiopathic skeletal hyperostosis (DISH) is a non-inflammatory condition most classically seen in the spine, and is characterized by ossification of the spinal ligaments and entheses. DISH may be asymptomatic, but in some cases is associated with back pain and stiffness. Risk factors include male sex, increasing age, obesity and metabolic syndrome. Important radiograp...

Autoantibodies Targeting Complement Receptors C3aR and C5aR1 Are Decreased in ANCA-associated Vasculitis and Correlate with a Higher Relapse Rate
Sebastian Klapa, Antje Müller, Andreas Koch et. al.

Nov 9th, 2020 - Background/Purpose: Activation of the alternative and common terminal complement pathways has been shown in ANCA-associated vasculitis (AAV). Circulating titers of the anaphylatoxin C5a are increased and correlate with disease activity in AAV. Binding to the corresponding G protein-coupled receptor (C5aR1/CD88) enhances the influx of neutrophils and their activation, leading to ROS generation a...

Advanced Integrated Science Courses: A Novel Approach to Medical Student Science Education
Eli Miloslavsky, Henrike Besche2 and John Flanagan, Massachusetts General Hospital et. al.

Nov 9th, 2020 - Background/Purpose: Scientific research is at the core of evidence-based medicine, and is transforming medical care at an increasingly rapid pace. The ability of students to engage with science as life-long learners is an important goal of undergraduate medical education and critical for practicing rheumatologists. However, there has been a trend towards shortening  pre-clinical science curricu...

Average Follow-up Time After Telemedicine Visit Is Longer Than Conventional Face-to-Face Visit in Outpatient Rheumatology Practice During COVID-19 Pandemic
Deepan Dalal, Tzu-Chun Chu, Brad Crough et. al.

Nov 9th, 2020 - Background/Purpose: In the era of recent COVID-19 crisis, outpatient rheumatology practices resorted to telemedicine to deliver longitudinal care, a fundamental component of chronic disease management.  However, little is known regarding time to follow up after telemedicine visits as compared to conventional face-to-face encounters as a marker of longitudinal care. Methods: We used electronic m...

Adverse Effects of Low Dose Methotrexate: Adjudicated Hematologic Outcomes in a Large Randomized Double-blind Placebo-controlled Trial
Kathleen Vanni, Nancy Berliner, Nina Paynter et. al.

Nov 9th, 2020 - Background/Purpose: Low dose methotrexate (LD-MTX), a cornerstone in the treatment of rheumatoid arthritis, is associated with a moderately increased risk of anemia and leukopenia, but the risk of myelosuppression during LD-MTX use of up to 20mg/week remains incompletely described. We examined the hematologic outcomes among patients taking LD-MTX versus placebo in a large randomized controlled ...

Geographic Distribution and Environmental Triggers of Systemic Sclerosis in Massachusetts
Anastasiya Muntyanu, Bina Kassamali, Ruth Ann Vleugels3 and Avery LaChance et. al.

Nov 9th, 2020 - Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune sclerosing disease with a 10-year survival rate of less than 65%. This rate has remained unchanged for over 40 years. Although the exact pathogenesis remains unclear, it has been hypothesized that an environmental trigger leads to disease development in a genetically predisposed host. Assessment of high incidence geographic di...

Gap in Contraceptive Education to Females with Rheumatic Disease on Teratogenic Medications
Andras Perl, Sundus Mian1 and Jihad Ben Gabr, SUNY Upstate Medical University et. al.

Nov 9th, 2020 - Background/Purpose: Teratogenic disease-modifying anti-rheumatic disease modifying drugs (DMARDs) are often prescribed to women of childbearing age. Contraception represents an important area of reproductive health for female patients with rheumatic diseases. Counseling prior to starting DMARDs can help prevent the potential pregnancy risks associated with such medications.  Methotrexate (MTX),...

CB2 Receptor Distribution and Effects of LenabasumTM in Dermatomyositis In Vitro
Spandana Maddukuri, Jay Patel, Christina Bax et. al.

Nov 9th, 2020 - Background/Purpose: Dermatomyositis (DM) patients report poor quality of life due to disease activity and persistent itch. Lenabasum is an oral non-immunosuppressive, non-psychoactive cannabinoid type 2 receptor (CB2R) reverse agonist being investigated for use in DM. Our previous studies showed increased IFNγ in DM compared to healthy controls (HC) and a decrease in IFNγ as a result of Lenabas...

Continued Treatment with Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Interim Analysis of SENSCIS-ON
Yannick Allanore, Madelon Vonk, Arata Azuma et. al.

Nov 9th, 2020 - Background/Purpose: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the rate of decline in forced vital capacity (FVC) (mL/year) over 52 weeks by 44% compared with placebo, with adverse events that were manageable for most patients. The SENSCIS trial (NCT02597933) was followed by an open-label extension trial, SENSCIS-ON (NCT03313180), to provide safety data, including data on...