The difference between Castleman disease and Castleman-like disease may be subtle, but it comes with significant ramifications. Case Presentation This case involves a pregnant 19-year-old woman who presents over multiple hospitalizations with concerns for systemic lupus erythematosus and macrophage activation syndrome. At 36 weeks’ gestation, the patient’s weight had dropped from 215 lbs. to 170…
Pulmonary manifestations in patients with systemic lupus erythematosus (SLE) include pleuritis, acute pneumonitis, interstitial lung disease, pulmonary arterial hypertension, shrinking lung syndrome and diffuse alveolar hemorrhage (DAH). DAH is a rare, but devastating, complication of SLE, with high mortality rates. The incidence of DAH in SLE ranges from 0.6% to 5.4%, but the mortality rate…
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 9 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…
Gout is the most common type of inflammatory arthritis in adults, and it typically occurs in men over the age of 50. When gout presents in younger patients or in women, this should warrant consideration of secondary causes. We describe an unusual genetic cause of tophaceous gout in a young, premenopausal woman. Case Report In…
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…