What happens when systemic sclerosis (SSc) overlaps with other systemic autoimmune rheumatic diseases? Patients with either diffuse cutaneous or limited cutaneous SSc sometimes develop systemic lupus erythematosus (SLE) as well. A new, large cohort study published in the Journal of Rheumatology reveals details on the epidemiology, clinical signs and survival data of SSc-SLE overlap syndrome.1…
Eosinophilic fasciitis generally presents with the acute onset of edema followed by progressive skin induration in the setting of hypergammaglobulinemia, an elevated erythrocyte sedimentation rate (ESR) and peripheral eosinophilia in 63–93% of patients.1,2 Skin involvement is typically limited to 20.1% of total body surface area and most commonly involves the extremities symmetrically.1,3 The condition was…
CHICAGO—At Hot Topics in Myositis, a session at the 2018 ACR/ARHP Annual Meeting, three experts discussed new classification criteria for idiopathic inflammatory myopathies (IIM) and offered practical primers on overlap myositis conditions and inclusion body myositis (IBM). New Myositis Classification Criteria After a 10-year development process, the new EULAR/ACR Classification Criteria for Adult and Juvenile…
Vasculitis is a group of chronic inflammatory diseases in which the blood vessel is the target of an immune reaction. They can be secondary to connective tissue disease, idiopathic or due to infection, neoplasm or drugs.1 Primary angiitis of the central nervous system (PACNS) is a rare syndrome characterized by inflammatory cell infiltration and necrosis…
Rheumatologists often rely on magnetic resonance imaging (MRI) in the evaluation of suspected muscular diseases. Here, we describe a case in which unexpected findings on MRI pointed to a diagnosis rarely considered as a mimicker of rheumatologic disease. The Case A 19-year-old man of Middle Eastern descent was admitted to our hospital for evaluation of…
Immunoglobin G4-related disease (IgG4-RD) is a rare fibro-inflammatory disease of unknown etiology that has been recently recognized. It can cause fibro-inflammatory masses in almost every organ of the body and is associated with dense lymphoplasmacytic infiltration of IgG4-postitive plasma cells, storiform fibrosis and elevated levels of serum IgG4.1 IgG4-RD is a systemic disease that may…
A recently published study gives new insight into an innovative approach to treating patients with severe scleroderma and internal organ involvement. During the six-year study, researchers demonstrated the durability of the beneficial effects of stem cell transplantation in this patient population. Their data also suggest the treatment has the potential to renormalize the autoreactive immune system…
The patient was a 48-year-old woman who saw her primary care physician for a flu-like illness three months prior to admission. Her symptoms initially improved, but recurred one month later; she was treated symptomatically, and again symptoms resolved. Two months later, she presented to an outside facility’s emergency department with fever to 103ºF, with associated…
WASHINGTON, D.C.—Experts speaking at the 2016 ACR/ARHP Annual Meeting session, Update on Small-Vessel Vasculitis, offered insight into the latest approaches to the diagnosis and treatment of diseases involving the inflammation of blood vessels. “Vasculitis is an immune-mediated process. White blood cells invade the vessel wall, causing inflammation throughout the vessel wall,” said Jason M. Springer,…
Erdheim-Chester disease (ECD) is a rare, non-Langerhan’s cell histiocytosis characterized by tissue infiltration of CD68-positive and CD1a-negative foamy histiocytes.1 ECD was discovered as a lipid granulomatosis in 1930 by Jakob Erdheim and his pupil, William Chester, and approximately 500 cases have been described to date.1 ECD has a heterogeneous course and prognosis ranging from an…