Immunoglobulin G4-related disease (IgG4-related disease) is relatively rare, although with greater recognition it is becoming more frequently diagnosed. Some of the more common presentations of the disease include aortitis, cholangitis, autoimmune pancreatitis, sclerosing sialadenitis, orbital disease with proptosis and subacute mass development in the affected organ.1 It has protean manifestations and is diagnosed by classic…
In 1888, Dr. Jan Mikulicz-Radecki reported a case of chronic, bilateral, painless enlargement of the salivary and lacrimal glands that appeared to be idiopathic.1 In subsequent years, other patients with these findings were reported, and the term Mikulicz syndrome was used to describe these cases. Although Mikulicz syndrome is now known to be associated with…
ACR CONVERGENCE 2020—Sjögren’s syndrome requires care from several specialists, and presenters at the Sjögren’s Syndrome: Dental and Ocular Perspectives session shared diagnostic and treatment pearls from their respective specialties. Rebecca Manno, MD, MHS, of the Comprehensive Arthritis and Rheumatology Center of the U.S. Virgin Islands moderated the session. Oral Health Dry mouth is famously associated…
Tumor necrosis factor-α inhibitors (TNFi’s) have emerged as an integral part of therapeutic strategies for several rheumatic diseases. TNF-α is a pro-inflammatory cytokine implicated in the pathogenesis of rheumatoid arthritis (RA), seronegative spondyloarthropathies and inflammatory bowel disease (IBD). It also plays a central role in the immune response to mycobacterial infection. Many biologic agents, particularly…
A large, international team of rheumatologists, geneticists, hematologists and other researchers has discovered a severe inflammatory syndrome linked to an acquired genetic mutation in the bone marrow of older men. The X-linked syndrome, they found, is caused by a somatic mutation in myeloid stem cells that hobbles the master regulator of a pathway tasked with…
Three experts discuss recent developments in the diagnosis & treatment of APS & the future direction of research.
ACR CONVERGENCE 2020—This has been a busy year for research publications covering a number of pediatric rheumatic diseases, including the emerging multi-system inflammatory syndrome (MIS-C) associated with SARS CoV-2. Despite the many challenges brought about by the COVID-19 pandemic, a healthy collection of publications covering a wide range of pediatric rheumatology research topics were published…
Early results in a phase 2 trial are finding that adding a TNF blocker to other treatment could greatly lower the risk of serious complications during pregnancy for women who have antiphospholipid syndrome with lupus anticoagulant.
ACR CONVERGENCE 2020—New evidence, though derived from small numbers, suggest that treatment with the TNF-inhibitor certolizumab could help improve high-risk pregnancy outcomes in women with antiphospholipid syndrome (with or without systemic lupus erythematosus) and lupus anticoagulants, an expert said. In the Phase 2 IMPACT trial, which has already enrolled 27 women and is continuing to…
A recent paper in Arthritis & Rheumatology opens up the possibility of a new research avenue to treat systemic sclerosis: dipeptidyl peptidase 4 (DPP4) inhibitors, a previously approved therapy for type 2 diabetes.1 Work in mouse models and on skin samples from systemic sclerosis patients suggests these drugs pose a promising area of future translational…