Robert E. Morrison, MD

Catastrophic antiphospholipid syndrome (CAPS), first described by Asherson and colleagues1 in 1992, refers to a clinical scenario in which multiple vascular occlusive events involving small vessels that supply blood to organs occur over a short period.

A 41-year-old woman with a 4-yearhistory of polymyositis with lupus featureshas had constant rectal pain for4 months. She has not noticed any factorsthat either aggravate or relievethe pain. The patient complains of intermittentconstipation (but no dischargeor rectal bleeding), generalizedweakness and malaise for the past 2months, a low-grade fever for the pastmonth, and a 4.1-kg (9-lb) weight lossover the past 6 weeks. She denies nightsweats or chills, anorexia, vision problems,drug allergies, and tobacco oralcohol use.

A methodical approach to diagnosis usually reveals the cause of fever. In patients with simple fever, a careful history taking and physical examination combined with basic laboratory and imaging studies (complete blood cell count with differential, urinalysis, and possibly a chest film and blood cultures) usually yield the diagnosis. In patients with prolonged fever whose cause remains undiagnosed after extensive examination (fever of unknown origin), repeat the history taking and physical examination; also order routine laboratory studies, an HIV test, a tuberculin skin test, 3 sets of blood cultures, and chest films. In addition, abdominal CT scanning is often useful. Further testing at this point may include fluorodeoxyglucose positron emission tomography, technetium-tagged white blood cell scanning, transesophageal echocardiography, liver biopsy, bone marrow examination, and/or temporal artery biopsy. Exploratory laparotomy is rarely indicated.