Cannabis Use Among Patients in a Large US Rheumatic Disease Registry
Kristin Wipfler, Teresa Simon, Patricia Katz et. al.

Nov 13th, 2019 - Background/Purpose: Legalization of cannabis use is rapidly increasing worldwide, raising the need to evaluate trends and medical implications. A systematic literature review showed inconsistent results on tolerability and safety of cannabis-based medicines for any chronic pain [1]. Additionally, little is known about the use and effectiveness of cannabis in rheumatic diseases [2]. We sought to...

Machine-learning Classification Identifies a Subset of Patients That Improve on Abatacept via Modulation of a CD28-Related Pathway
Bhaven Mehta, Jennifer Franks, Yiwei Yuan et. al.

Nov 13th, 2019 - Background/Purpose: We analyzed a phase 2 study designed to assess the efficacy of abatacept in patients with diffuse Systemic Sclerosis (SSc). In this work, we analyze data from ASSET and seek to confirm the hypothesis that patients in the inflammatory subset on abatacept show a significant decline in modified Rodnan Skin Score (mRSS), which is correlated to modulation of pathways related to t...

Metabolic Signatures of Pathogenic T Cells in Medium and Large Vessel Vasculitis
Mitsuhiro Akiyama, Hui Zhang, Ryu Watanabe et. al.

Nov 13th, 2019 - Background/Purpose: Giant cell arteritis (GCA) is an autoimmune vasculitis that causes aortic arch syndrome, blindnesss, and stroke. Embedded in granulomatous infiltrates, CD4 T cells persist over long periods in the inflamed vessel wall where they drive wall remodeling, intramural neoangiogenesis, intimal hyperplasia and luminal occulsion. To survive and function in a nonlymphoid tissue enviro...

Ancestry-Specific Classical HLA Alleles Define Phenotypic Subsets in the African American Scleroderma Population
Pravitt Gourh, Sarah Safran, Steven Boyden et. al.

Nov 13th, 2019 - Background/Purpose: Systemic sclerosis (SSc), or scleroderma, is a heterogeneous disease that is divided into limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) forms based on the extent of skin involvement, and internal organ involvement also adds to phenotypic variability. The three main internal organ complications are interstitial lung disease (ILD), pulmonary arterial hypertension (PA...

Geographic Disparities in Systemic Sclerosis Mortality in the United States: 1999 to 2017
Alicia Rodriguez-Pla1 and Robert W Simms, University of Arizona/Banner Health Medical Center, Tucson et. al.

Nov 13th, 2019 - Background/Purpose: Population mortality studies in the United States have previously reported a progressive increase in the scleroderma (SSc) mortality rates from 1959 to 2002. Identification of areas with clusters of higher mortality rates is important to implement targeted interventions and may provide clues about possible etiology factors, especially environmental. In this study, we aimed t...

Increased Expression of Extracellular Matrix Proteins in Human Fibroblast Synoviocytes and Lung Epithelial Cells Following Malondialdehyde-Acetaldehyde Adduct (MAA)/Citrullinated Protein Stimulation
Matthew O'Dell, Logan Duryee, Lynell Klassen et. al.

Nov 13th, 2019 - Background/Purpose: Recently we have shown that both malondialdehyde-acetaldehyde adducts (MAA) and citrullinated proteins (CIT) are co-localized in joint and lung tissues of patients with rheumatoid arthritis (RA). Therefore, to investigate the possibility that these post-translational modifications pose a synergistic pathogenic role in RA, human synovial fibroblasts and lung epithelial cells ...

Serum Interferon Score Predicts Clinical Outcome at 12 Months in Diffuse Cutaneous Systemic Sclerosis as Measured by Global Ranked Composite Score (GRCS) and Composite Response Index in SSc (CRISS)
Antonio Carriero, Giuseppina Abignano, MIchelle Hutchinson et. al.

Nov 13th, 2019 - Background/Purpose: Systemic sclerosis (SSc) is a highly heterogeneous disease orphan of effective disease modifying agents. The diffuse cutaneous clinical subset (dcSSc) is currently targeted in most clinical trials. Nevertheless, there is still high variability in clinical outcome within dcSSc, which is limiting effective clinical trial design and interpretation. Global Ranked Composite Score...

Cryopyrin-Associated Periodic Syndrome Treated with Canakinumab – Long-Term Follow-up Data Documents Sustained Safety and Remission
Jasmin Kuemmerle-Deschner, Norbert Blank, Michael Borte et. al.

Nov 13th, 2019 - Background/Purpose: Targeting the interleukin(IL)-1 pathway with anti-IL-1 drugs is a treatment option in patients with autoinflammatory diseases like monogenic periodic fever syndromes. The study aims to gain further insights into long-term effectiveness and safety of canakinumab (CAN), an anti-IL1 inhibitor, under routine clinical practice conditions in pediatric and adult patients with CAPS ...

Clinical Subsets in Giant Cell Arteritis
K Bates Gribbons, Cristina Ponte, Anthea Craven et. al.

Nov 13th, 2019 - Background/Purpose: Giant cell arteritis (GCA) is a clinically heterogeneous disease.  Disease subsets based upon cranial versus extracranial artery involvement have been proposed.  The study objective was to determine if GCA subsets are associated with distinct clinical profiles or merely reflective of differential diagnostic assessment. Methods: Patients were included from a large internation...

Comparison of Arterial Patterns of Disease in Takayasu’s Arteritis and Giant Cell Arteritis
K Bates Gribbons, Cristina Ponte, Anthea Craven et. al.

Nov 13th, 2019 - Background/Purpose: Current classification criteria differentiate between Takayasu’s arteritis (TAK) and giant cell arteritis (GCA), the two most common forms of large-vessel vasculitis, based primarily on clinical assessment, yet patients with TAK and GCA may differ in patterns of arterial disease.  This study aimed to use computer-based algorithms to determine if patterns of arterial disease ...

Deriving Accurate Prednisone Dosing from Electronic Health Records: Analysis of a Natural Language Processing Tool for Complex Prescription Instructions
Francine Castillo, Adrienne Strait, Michael Evans et. al.

Nov 13th, 2019 - Background/Purpose: Prednisone is commonly used to treat rheumatic diseases, yet few comparative effectiveness studies on different dosing regimens are available. Electronic health records (EHR) and prescriptions are potential data sources for such research. However, a barrier to this work is organizing non-standardized prednisone “sigs”, or free-text instructions within a prescription, into a ...

Application of Bayesian Statistics to Support Approval of Intravenous Belimumab in Children with Systemic Lupus Erythematosus in the United States
Ginto Pottackal, James Travis, Rosemarie Neuner et. al.

Nov 13th, 2019 - Background/Purpose: In April 2019, FDA approved the first treatment, intravenous (IV) belimumab (BEL), specifically for children 5 to 17 years of age with active, seropositive SLE receiving standard care (SOC). The approval was supported by a single post-marketing required randomized, controlled trial (NCT01649765) that evaluated the efficacy, safety and pharmacokinetics (PK) of 10 mg/kg IV BEL...

Access to Care and Diagnostic Delays in Juvenile Dermatomyositis
Jessica Neely, Susan Kim, Hugh Sturrock 2 and Julia Shalen et. al.

Nov 13th, 2019 - Background/Purpose: Factors associated with diagnostic delay in Juvenile Dermatomyositis (JDM) are not known.  We hypothesized that limited access to pediatric rheumatologists can lead to delay in the diagnosis of JDM and worse outcomes. We investigated demographic factors, including access to care, associated with diagnostic delays and clinical outcomes of subjects with JDM in the Childhood Ar...

An Integrated Gut Microbiomic and Plasma Metabolomic Analysis in Patients with Four Systemic Autoimmune Diseases
Chiara Bellocchi, Álvaro Fernández-Ochoa, Gaia Montanelli et. al.

Nov 13th, 2019 - Background/Purpose: Gut microbiota may be relevant in the regulation of immune processes and in the development of systemic autoimmune diseases (SADs). A reduction of microbiota diversity and a selective decrease of commensal pro-regolatory bacteria have been observed in systemic lupus erythematosus (SLE), Sjogren syndrome (SJS) and primary anti-phosholipid syndrome (PAPS). None studies have be...

Rilonacept in Recurrent Pericarditis: Efficacy and Safety Data from an Ongoing Phase 2 Pilot Clinical Trial
Allan Klein, David Lin, Paul Cremer et. al.

Nov 13th, 2019 - Background/Purpose: Recurrent pericarditis (RP) is characterized by the recurrence of pericarditis signs and symptoms after a symptom-free period of ≥4 to 6 weeks and affects 20-30 % of acute pericarditis patients. IL-1α/β, mediators of the inflammatory process, predominate in recurrent pericarditis (RP). Methods: Participants with symptomatic or corticosteroid (CS)-dependent idiopathic or post...

Rituximab as Rescue Therapy in Treatment-Refractory CTD-ILD
Julia Sun, Charles Oshinsky, Nicole Garcia et. al.

Nov 13th, 2019 - Background/Purpose: Interstitial lung disease (ILD) is the leading cause of mortality and carries significant morbidity in connective tissue disease (CTD). Corticosteroids are often used as first line therapy, yet the use of additional immunosuppressive (IS) agents remains largely empiric. In this study we evaluated the effect of Rituximab (RTX) as rescue therapy on lung function in a cohort of...

Imaging Acquisition Technique Influences Interpretation of Positron Emission Tomography Vascular Activity in Large-Vessel Vasculitis
Kaitlin Quinn, Joel S. Rosenblum, Casey A. Rimland et. al.

Nov 13th, 2019 - Background/Purpose: 18F-flurodeoxyglucose (FDG) positron emission tomography (PET) is one of several imaging modalities used in the assessment of patients with large-vessel vasculitis (LVV).  Conventionally PET images are obtained 1 hour after radiotracer injection; however, delayed imaging may be more sensitive to detect vascular inflammation.    The objectives of this study were to: 1) determ...

Interferon Signature Predicts Response to Tofacitinib in Haploinsufficiency of A20
Sarah Blackstone, Daniella Schwartz, Natalia Sampaio Moura et. al.

Nov 13th, 2019 - Background/Purpose: The protein A20, encoded by TNFAIP3, represses signaling upstream of the inflammatory transcription factor nuclear factor (NF)-kB by regulating ubiquitination. Heterozygous loss-of-function mutations in TNFAIP3 cause the systemic autoinflammatory disease Haploinsufficiency of A20 (HA20) [1]. Because no FDA-approved medications directly target NF-kB signaling, current HA20 th...

Anakinra Treatment in Patients with Familial Mediterranean Fever: A Single-center Experience
Serdal Ugurlu, Bugra Han Egeli, Bilgesu Ergezen et. al.

Nov 13th, 2019 - Background/Purpose: The main goal of Familial Mediterranean Fever (FMF) treatment is to prevent attacks and subclinical inflammation. Mainstay of treatment is colchicine; however, 5-10% of patients are unresponsive to colchicine even in maximum tolerated dosage. Anakinra has been proven to be effective in controlled trials in terms of attack frequency and subclinical inflammation in colchicine ...

The Use and Safety of Rituximab in Connective Tissue Disease Associated Interstitial Lung Disease
Christopher Mesa, Snehaja Yadlapati 1 and Myriam Guevara, Louisiana State University Health Science Center et. al.

Nov 13th, 2019 - Background/Purpose: Interstitial Lung Disease (ILD) is the most common lung manifestation in Connective Tissue Diseases (CTD). It is present in most types of CTD, such as: Rheumatoid Arthritis (RA), Systemic Sclerosis (SSc), Systemic Lupus Erythematous (SLE), Inflammatory Myositis (IM), Mixed Connective Tissue Disease (MCTD), Sjogren Syndrome and Sarcoid. Despite it being so common, treatment h...