Primary hemophagocytic lymphohistiocytosis (HLH) is caused by genetic mutations and inherited syndromes; it therefore occurs in the pediatric age group. Secondary HLH, however, is more common in adults and is often triggered by other disease states, such as malignancies, chronic immunosuppression, infections and autoimmune disease.1,2 Macrophage activation syndrome (MAS) is a subset of secondary HLH…
A 59-year-old woman presented to our rheumatology clinic with a six-month history of a symmetric polyarthritis. She initially experienced pain in both knees. As time progressed, she began to notice pain in her ankles, hips, shoulders, hands and feet. She experienced joint stiffness lasting for more than 30 minutes every morning. She also described worsening…
Angioimmunoblastic T cell lymphoma (AITL) is an aggressive, peripheral T cell, non-Hodgkin’s lymphoma with an incidence of 0.05 cases per 100,000 person-years in the U.S., and it typically manifests in adults older than 60 years.1,2 AITL was previously known as angioimmunoblastic lymphadenopathy with dysproteinemia, immunoblastic lymphadenopathy or lymphogranulomatosis X, due to the hypothesis that the…
A 65-year-old woman was referred by an orthopedist to a rheumatologist for left knee pain. Previously, in 2014, she underwent left total knee arthroplasty (TKA) for severe osteoarthritis in a different institution. Following the procedure, she experienced severe chronic anterolateral knee pain at rest, exacerbated by walking. Because she was rendered wheelchair bound and required…
A 59-year-old woman with rheumatoid arthritis (RA) presented to our pulmonary clinic for progressively worsening dyspnea of five years’ duration. She described progressively worsening dyspnea after a few minutes of walking on level ground. In addition, she noted worsening pain and morning stiffness of the wrists, knees and metacarpophalangeal (MCP) joints, with subcutaneous nodules. She…
A 42-year-old man with a history of ulcerative colitis (UC), primary sclerosing cholangitis (PSC) and chronic sinusitis was referred to a rheumatologist to evaluate for a possible diagnosis of systemic vasculitis. This patient had developed new skin lesions, gingival hypertrophy and ulcerating tracheobronchitis, concerning for possible granulomatosis with polyangiitis (GPA). Since 1994, the patient had…
Hair dye products are commonly used by both men and women to enhance youth and beauty and to follow fashion trends. As reported in the medical literature, hair dyes and their ingredients are associated with allergic contact dermatitis. A possible association with joint inflammation has also been recognized. There is literature to support that para-phenylenediamine…
The patient medical history is far more prominent in clinical decisions for rheumatology than for many common chronic diseases in which a gold standard biomarker, such as blood pressure or serum glucose, is applicable to diagnosis and management of all individual patients.1 Components of a subjective patient history may be recorded as structured, quantitative, standard,…
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis. SRC occurs in 2–15% of patients with diffuse sclerosis and usually within the first five years from the time of diagnosis. Risk factors for SRC include, but are not limited to, early diagnosis, corticosteroid or cyclosporine use, and the presence of anti-RNA polymerase III…
Apremilast was first marketed in March 2014 for the treatment of adults with psoriatic arthritis (PsA). An immunomodulating drug, which is a small molecule inhibitor of phosphodiesterase 4 (PDE4) specific for cyclic adenosine monophosphate (cAMP), apremilast is administered orally. By inhibiting PDE4, intracellular cAMP levels are increased. Although the exact mechanism of action is not…