A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

History of the present illness The patient was in his usual state of health until 2 months before admission, when he developed pain and stiffness in his right shoulder, neck, and back after playing softball. His symptoms progressively worsened despite manipulation by a chiropractor, and he developed new pain in his soles. This was diagnosed as plantar fasciitis and he was given a topical steroid injection and a short course of methylprednisolone. This treatment improved his pain, but at its completion, he developed disabling diffuse arthralgias in his shoulders, elbows, knees, and feet, along with fevers, anorexia, and weight loss. Worsening symptoms prompted hospitalization in another institution 3 weeks before admission, where he was febrile up to 103°F and hypertensive (144/100 mm Hg). An extensive evaluation revealed elevated inflammatory markers, including erythrocyte sedimentation rate (ESR; 102 mm/hour), C-reactive protein level (56 mg/liter), positive antinuclear antibodies (ANAs; 1:640), strongly positive anti–doublestranded DNA (anti-dsDNA) titers, low levels of C4 (9 mg/dl, normal range 16–38), prolonged prothrombin time (16.2 seconds, normal range 10–13), prolonged activated partial thromboplastin time (60 seconds, normal range 27– 38), and positive lupus anticoagulant (LAC) by dilute Russell’s viper venom time. His urine analysis showed proteinuria (0.440 gm protein/gm creatinine), hematuria (11–25 red blood cells [RBCs]/high-power field [hpf]), and pyuria (3–10 white blood cells [WBCs]/hpf). Antibodies to Sm/RNP, Ro/SSA, and La/SSB; anticardiolipin antibodies; antineutrophil cytoplasmic antibodies (ANCAs); and anti– cyclic citrullinated peptide antibodies were negative, and creatine phosphokinase was normal. An infectious disease evaluation was performed and was negative for all pathogens, including Lyme disease, human immunodeficiency virus, and viral hepatitis. Renal ultrasound and Doppler were performed and were negative for renal pathology and venous thrombosis. He was placed on prednisone 10 mg orally twice daily, losartan 50 mg daily, and aspirin 81 mg daily, and his fevers resolved with some improvement in joint pains. Two weeks before admission, he was evaluated by a rheumatologist who discontinued aspirin in anticipation of a kidney biopsy to rule out lupus nephritis. Laboratory evaluation again revealed persistent proteinuria (2 ), hematuria (10–20 RBCs/hpf), C4 hypocomplementemia (C3 111 mg/dl, C4 9 mg/dl), and strongly positive anti-dsDNA. The patient could not tolerate tapering of prednisone to 15 mg and he stayed at 17.5 mg daily. He presented to our clinic 3 days before admission and his scheduled kidney biopsy. Over the course of the previous week he had developed a few small tender erythematous skin lesions and some skin excoriation on his fingers and palms. He also experienced numbness of his fingertips and bilateral soles for 2 or 3 days. Weight loss of 30–33 pounds over the course of 3 months with decreased appetite, nausea, and mild diarrhea was noted. On examination, he appeared ill with a blood pressure (BP) of 140/100 mm Hg, and a heart rate of 96 beats per minute. There were a few erythematous lesions on his palate, as well as small tender erythematous nonblanching lesions in his palms, fingertips, and toe tips with rare splinter hemorrhages. He had dysesthesia/hypoesthesia in bilateral palms and finSupported by the Mary Kirkland Center for Lupus Research. Julie Cherian, MD: Stony Brook University Hospital, Stony Brook, New York; Roland Duculan, MD, Mary K. Crow, MD, Kyriakos A. Kirou, MD: Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, New York; Isabelle Amigues, MD: Teaching Hospital Lyon Sud, Pierre Benite, Rhone Alpes, France. Drs. Crow and Kirou have filed a patent application for an interferon assay. Address correspondence to Kyriakos A. Kirou, MD, Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021. E-mail: kirouk@hss.edu. Submitted for publication October 21, 2010; accepted in revised form January 18, 2011. Arthritis Care & Research Vol. 63, No. 5, May 2011, pp 766–774 DOI 10.1002/acr.20439 © 2011, American College of Rheumatology