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…
Juvenile dermatomyositis (JDM) is a childhood form of inflammatory myopathy affecting children younger than 18 years. The diagnosis of JDM is based on characteristic clinical features of proximal muscle weakness, skin rash, elevated muscle enzymes, magnetic resonance imaging and muscle biopsy.1 Autoantibodies seen in patients with inflammatory myopathy are not only associated with particular phenotypes…
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…
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,…
Dermatomyositis (DM) is an idiopathic inflammatory myopathy involving proximal muscle weakness and skin rash. An associated increased risk of malignancy is well established.1 The most frequent malignancies are related to the ovary, endometrium, lung, gastrointestinal tract, prostate, breast and lymphatics.2 On rare occasions, DM has been reported with certain types of non-Hodgkin’s lymphoma, specifically cutaneous…
We report on a 77-year-old patient who was diagnosed with giant cell arteritis (GCA) on histopathology of the submandibular gland and eventually went on to develop classic signs and symptoms of GCA. This observation highlights neck swelling as a rare initial presentation of GCA. Case Presentation A 77-year-old previously healthy man presented to his primary…
A 24-year-old woman presented to our rheumatology office in 2017 with a blotchy purple rash on her arms and legs. She reported no history of miscarriage or blood clots. The rash pattern was concerning for livedo reticularis or livedo racemosa, and she was noted to have an anti-nuclear antibody (ANA) titer of 1:160 with a…