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…
Scleroderma, or systemic sclerosis (SSc), is an autoimmune disease characterized by vasculopathy and fibrosis. Although relatively rare, with a prevalence in North America of approximately 300 per 1 million people, SSc is associated with significant morbidity and high rates of mortality.1 Patients with scleroderma have four times greater mortality than age- and sex-matched controls, with…
We report a case of a 27-year-old woman who was initially diagnosed with systemic lupus erythematosus (SLE), had features of scleroderma and was subsequently found to have lymph node biopsy consistent with multicentric Castleman disease (MCD). She also had serologic evidence of acute Epstein-Barr virus (EBV) infection (vs. reactivation of EBV). The occurrence of MCD…
Rheumatoid arthritis (RA) is rarely associated with renal manifestations, but secondary amyloidosis due to chronic inflammation is reported to be the etiology of renal dysfunction in many cases of RA.1,2 The discovery of biologic therapy, with TNF-alpha inhibitors in particular, made a huge difference in the disease course and prognosis of RA patients. However, TNF-alpha…
A 47-year-old Caucasian woman presented to the rheumatology clinic with a one-year history of pain and swelling involving the wrists, hands, ankles and feet that progressively worsened. Her symptoms included generalized fatigue, morning stiffness in the hands and lower back lasting more than an hour, Raynaud’s phenomena (triphasic), photosensitivity, tightening of the skin of the…
Despite a generation of advances in molecular biology, a huge gap exists between the Sjögren’s syndrome (SS) patient’s description of their symptoms and the objective findings. Current issues include: Many SS patients are misclassified as either rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE), even within rheumatology clinics. Frequently, the sickest SS patients with extraglandular…