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…
At the 2021 ACR State-of-the-Art Clinical Symposium, Saira Sheikh, MD, associate professor of Medicine and director of the Rheumatology Lupus Clinic, University of North Carolina, Chapel Hill, provided an update on the past, present and future of the management of systemic lupus erythematosus (SLE). This year, hydroxychloroquine received a great deal of attention, given early…
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…