In September 2019, a previously healthy, 9-year-old white girl presented to the emergency department following two months of sinusitis and unexplained fever responsive to ibuprofen. She presented with anorexia; a 9Â lb. weight loss; intermittent, nonbilious, nonbloody emesis; and occasional epistaxis with digital manipulation of the nose. Six weeks prior to admission, she had presented to…
Immunoglobulin G4-related disease (IgG4-related disease) is relatively rare, although with greater recognition it is becoming more frequently diagnosed. Some of the more common presentations of the disease include aortitis, cholangitis, autoimmune pancreatitis, sclerosing sialadenitis, orbital disease with proptosis and subacute mass development in the affected organ.1 It has protean manifestations and is diagnosed by classic…
Patients with autoimmune diseases, such as spondyloarthritis, are at risk of developing inflammatory bowel disease. Here are considerations for its management and treatment.
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 proÂfessor 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…
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 immunoÂdeficiency (CVID) is a common primary immunoÂdeficiency disease, with an estimated incidence of one per 25,000–50,000 individuals.1 The classic presenÂtation includes frequent bacterial infections, secondary to dysfunctional B cell differÂentiation, impaired immunoglobulin production and diminished antibody response. The clinical presentation may be heterogenous and may include granulomatous disease as an uncommon manifestation. GranuloÂmatous…
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…
Since 1991, hydroxychloroquine (HCQ) has been a staple for the treatment of patients with systemic lupus erythematosus; it has been shown to improve survival, reduce cardiovascular risk, thrombosis and renal damage, delay or prevent lupus cerebritis and more. However, HCQ can potentially bind in the retinal pigment epithelium and cause degeneration of photoreceptors, leading to…