February 2020 Scleroderma News

Modeling Progressive Fibrosis with Pluripotent Stem Cells Identifies an Anti-fibrotic Small Molecule. We describe the generation and characterization of an in vitro progressive fibrosis model that uses cell types derived from induced pluripotent stem cells. PubMed, Cell Rep, 2019 Dec 10;29(11):3488-3505.e9. (Also see Stem Cells)

Human Leukocyte Antigen (HLA) and Autoantibodies Define Scleroderma Subtypes and Risk in African and European Americans and Suggest a Role for Molecular Mimicry. These data suggest a possible link between HLA alleles, autoantibodies, and environmental triggers in the pathogenesis of systemic scleroderma. PubMed, Proc Natl Acad Sci U S A, 2020 Jan 7;117(1):552-562. (Also see Ethnicity, Race and Geographical Regions)

Corticosteroid-sparing (CS) benefit of intravenous immunoglobulin (IVIg) in systemic sclerosis-associated myopathy (SScAM): A comparative study in 52 patients. This study suggests the benefit of IVIg as adjunctive therapy, with an acceptable tolerance profile, and supports its use as a CS-sparing agent, in SScAM. PubMed, Autoimmun Rev, 2020 Jan;19(1):102431. (Also see Treatments for Dermatomyositis and Polymyositis)

Characterizing Disease Manifestations and Treatment Patterns Among Adults with Systemic Sclerosis (SSc): A Retrospective Analysis of a US Healthcare Claims Population. One-third of patients with SSc in the healthcare claims population received immunomodulating therapy during the first year after diagnosis. PubMed, Rheumatol Ther, 11/16/2019. (Also see Immunosuppressants)

Advances in the Treatment of Scleroderma (SSc): Autologous Hematopoietic Stem Cell Transplant (HSCT). HSCT can be considered as an option in carefully selected patients with rapidly progressive SSc who have a risk of organ failure. Medscape, 1/2/2020. (Also see Stem Cell Transplantation)

Defining genetic risk factors for scleroderma-associated interstitial lung disease (SSc-ILD) : IRF5 and STAT4 gene variants are associated with scleroderma while STAT4 is protective against scleroderma-associated interstitial lung disease. Further work is required to understand the genetic basis of lung fibrosis in association with scleroderma. PubMed, Clin Rheumatol, 01/08/2020. (Also see Pulmonary Fibrosis Diagnosis and Genetics)

Case Report: Spontaneous bowel perforation in the setting of colonic involvement with scleroderma (SSc). It is essential that clinicians faced with abdominal symptoms and signs in patients affected by SSc are able to quickly differentiate acute visceral perforation from benign causes. PubMed, BMJ Case Rep. (Also see Bowel Dysfunction)

Case Report: Autoimmune Hepatitis and Systemic Sclerosis: a Rare Association. Autoimmune hepatitis and systemic sclerosis may be associated, therefore patients with these diseases should be monitored closely. PubMed, Eur J Case Rep Intern Med. (Also see Liver and Spleen Involvement)

Dipeptidylpeptidase 4 as a Marker of Activated Fibroblasts and a Potential Target for the Treatment of Fibrosis in Systemic Sclerosis (SSc). DPP-4 characterizes a population of activated fibroblasts and shows that DPP-4 regulates TGFβ-induced fibroblast activation in the fibrotic skin of SSc patients. PubMed, Arthritis Rheumatol, 2020 Jan;72(1):137-149. (Also see Fibroblasts)

Increased Risk of Ischemic Stroke in Systemic Sclerosis (SSc): A National Cohort Study of US Veterans. Patients with SSc represent a population likely to benefit from targeted stroke screening or prevention therapies. PubMed, J Rheumatol, 2020 Jan;47(1):82-88. (Also see Cardiac (Heart) Involvement)

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