BETHESDA, MD—Rheumatologists from the National Institutes of Health (NIH) and from Jerusalem, Israel, have identified deficiency of adenosine deaminase 2 (DADA2) as an important cause of familial polyarteritis nodosa, which tends to present in childhood and can manifest with hematologic, immunologic and inflammatory signs, says Chip Chambers, MD, founder and president of the DADA2 Foundation….
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…
Varicella-zoster-virus (VZV) reactivation, which can cause patients to develop herpes zoster (i.e., shingles), occurs more frequently in patients with systemic vasculitis and systemic lupus erythematosus (SLE) who have received intravenous cyclophosphamide than in otherwise healthy adults, according to a retrospective study published in The Journal of Rheumatology by researchers in France.1 The study also shows…
A 39-year-old woman presented at the emergency department with three weeks of progressive, constant and pulsatile right-sided headache. She said her headache was worse in the morning and when she would bend forward. She reported associated nausea and vomiting. On initial assessment, she did not have any focal neurological deficits. Her medical history was significant for…
Researchers sought to identify predictors of patients with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) who took rituximab for maintenance had a better sustained remission rate through 60 months than those taking azathioprine, according to the latest results from the maintenance of remission using rituximab in systemic ANCA-associated vasculitis (MAINRITSAN) trial, a prospective, randomized trial…
Rheumatologists prescribe rituximab for induction and maintenance treatment for anti-neutrophil cytoplasm antibody (ANCA) associated vasculitides (AAV). Maintenance treatment typically employs fixed-schedule dosing, but in the recent maintenance of remission using rituximab in systemic ANCA-associated vasculitis II (MAINRITSAN2) trial, researchers from the French Vasculitis Study Group examined whether individually tailored maintenance dosing might work better. “The…
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by pain and morning stiffness at the neck, shoulders and hip girdle. It can be associated with giant cell arteritis (GCA); in fact, the two disorders may represent a continuum of the same disease process. This case describes a patient who initially refused treatment for PMR…
Severe infections occurred in 25% of patients with anti-neutrophil cytoplasm antibody (ANCA) associated vasculitis (AAV) who were treated with rituximab, according to results of an observational study conducted in the United Kingdom (U.K.) and Austria. Trimethoprim-sulfamethoxazole (TMP/SMX) prophylaxis, however, reduced the risk of severe infections, the investigators reported in Annals of the Rheumatic Diseases.1 Andreas…
Patients with polymyalgia rheumatica (PMR) or peripheral arthritis may require extra vigilance during treatment because of a suspected link to giant cell arteritis (GCA) and, potentially, permanent vision loss. “Development of giant cell arteritis after treating polymyalgia or peripheral arthritis: a retrospective case-control study,” a March 2018 study published in The Journal of Rheumatology, suggests…
A recent study in Arthritis & Rheumatology highlights new information about the epidemiology and disease course of the vasculitic disease granulomatosis with polyangiitis (GPA, formerly known as Wegener’s disease).1 GPA is a rare disease that’s generally specific to the lungs, kidneys and the upper airways. The study provides key new data about the incidence and…