A 61-year-old white woman presented to our rheumatology clinic in New England to establish care in early June 2018, following a move from Texas. She reported a medical history of inflammatory bowel disease, uveitis and seronegative inflammatory arthritis, which was difficult to control and required the use of multiple medications. At her initial visit, she…
Many a rheumatology consult has centered on a perplexing question: Does this patient have central nervous system (CNS) vasculitis? At the 2021 ACR State-of-the-Art Clinical Symposium, Rula Hajj-Ali, MD, FACP, professor of medicine and associate director of vasculitis care and research, Cleveland Clinic Lerner College of Medicine, discussed this topic in detail, providing a series…
Common variable immunodeficiency (CVID) is a common primary immunodeficiency disease, with an estimated incidence of one per 25,000–50,000 individuals.1 The classic presentation includes frequent bacterial infections, secondary to dysfunctional B cell differentiation, impaired immunoglobulin production and diminished antibody response. The clinical presentation may be heterogenous and may include granulomatous disease as an uncommon manifestation. Granulomatous…
A 26-year-old Peruvian woman presented to the emergency department of a large teaching hospital in Lima, Peru, with epistaxis and hematomas that had occurred over the preceding few days; she was found to have severe thrombocytopenia and a normocytic, normochromic anemia. She was treated with pulse doses of methylprednisolone; however, within two days, she presented…
Calciphylaxis, or calcific uremic arteriolopathy, is a rare disease characterized by calcification of the arterioles and capillaries in the dermis and subcutaneous tissue, resulting in thrombus formation and subsequent skin ischemia and necrosis.1 This serious condition most commonly occurs in patients with end-stage renal disease (ESRD) requiring dialysis or in kidney transplant recipients. In rare…
Dermatomyositis is an uncommon autoimmune condition involving skeletal muscle characterized by subacute onset of progressive weakness, intramuscular inflammatory infiltrates and the presence of myositis-specific autoantibodies.1 Immune-mediated myopathies may exert some pathogenic effects on the muscle tissue by targeting the microvasculature.1 Capillary inflammation, fragility and loss may contribute to heightened bleeding events in these patients. Here,…
A phase 3 trial described in The New England Journal of Medicine (NEJM) highlights the potential of a C5a receptor inhibitor, avacopan, for anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis.1 Avacopan may potentially offer a steroid-sparing option for the treatment of this serious disease. Current Treatment of ANCA-Associated Vasculitis Morbidity and mortality from ANCA-associated vasculitis have…
It is well known that hip fractures are associated with significant morbidity and mortality: Mortality increases 15–25% in the year following a hip fracture.1–5 We know that treating osteoporosis prevents fractures and improves patient survival. But is there a relationship beyond this? Several studies have found that bisphosphonate therapy is associated with a reduction in…
The ACR, in concert with the Vasculitis Foundation, released three new vasculitis guidelines online first in July, with a fourth slated for publication by the end of the year. These are the first ever to be produced and endorsed by the ACR and the Vasculitis Foundation. Although most of the recommendations are conditional, due to…
NEW YORK (Reuters Health)—Results of a randomized controlled trial do not support routine use of proactive therapeutic drug monitoring (TDM) during infliximab induction for improving disease remission rates in patients rheumatoid arthritis (RA) and other chronic immune-mediated inflammatory diseases. Proactive therapeutic drug monitoring tailors biologic therapy to individual patients by measuring serum drug levels and…