History of the present illness The patient had been admitted 6 months earlier to an outside hospital with a high-grade fever, an erythematous rash on her lower extremities, and pain in her legs. She had a history of bipolar disorder and schizoaffective disorder, and had been treated with the antipsychotic medications ziprasidone and quetiapine fumarate. An initial evaluation for infections was negative, and broadspectrum antibiotics failed to relieve the fever. The patient’s fevers were attributed to the neuroleptic malignant syndrome (Table 1). Her fevers continued unabated to temperatures as high as 103°F, despite discontinuation of her antipsychotic medications and treatment with bromocriptine. She was transferred to another hospital for further evaluation and management. At the transfer hospital, the patient exhibited a transient increase in her creatine kinase level to 1,389 units/liter (normal value 240). Assays for antinuclear antibodies, antibodies to double-stranded DNA, and extractable nuclear antigens (Ro, La, Sm, and RNP) were negative, as were anti–Jo-1 antibodies. A magnetic resonance image of the lower leg showed muscle edema with localized infarction. A muscle biopsy showed perivascular inflammatory infiltrates consistent with an inflammatory myopathy. Prednisone 60 mg/day was started and azathioprine was considered, but the patient’s localized rash and leg pain resolved spontaneously before treatment with azathioprine was started. The patient was prescribed a tapering course of prednisone for presumed dermatomyositis, and did not experience recurrent episodes of fever, rash, or leg pain while receiving glucocorticoids (Table 1). No photographic record of the patient’s original rash exists. Within one week of discharge, the patient developed recurrent fevers and was readmitted to a third hospital. Her dose of prednisone at that time was unknown. She was later found to have a urinary tract infection caused by Escherichia coli and was treated with trimethoprim/sulfamethoxazole. Despite antibiotic treatment, the patient continued to have intermittent high-grade fevers and episodes of hypotension, with blood pressure as low as 70/40 mm Hg (Table 1). She was transferred to a fourth hospital. Upon transfer, the patient had a temperature of 104°F, sinus tachycardia (140–160 beats/minute), hypotension, and a hematocrit of 20%. There was no rash despite the development of intermittent high-grade fever. Multiple cultures were negative for infection. The patient underwent a bone marrow biopsy that showed a hypercellular marrow, and a peripheral smear showed some dysplastic myeloid forms and blasts. These were interpreted as being consistent with reactive changes associated with anemia of chronic disease. A second muscle biopsy finding showed mild perivascular inflammatory infiltrates in the endomysium, consistent with an inflammatory myopathy. However, further analysis by electron microscopy and immunohistochemical staining demonstrated no definitive evidence of such a condition. Antibodies against the membrane attack complex of complement, the major histocompatibility complex, CD20, CD3, laminin , Brown-Brenn stain, and NADH reductase were negative. The final interpretation of the muscle biopsy finding was nonspecific and possibly the result of trauma or ischemia at needle entry sites. The patient continued receiving prednisone without a Richard C. Chou, MD, PhD (current address: DartmouthHitchcock Medical Center, Lebanon, New Hampshire), Judith A. Ferry, MD: Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; Charles A. Dinarello, MD: University of Colorado School of Medicine in Denver, Aurora; Paola Dal Cin, PhD: Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts. Address correspondence to Richard C. Chou, MD, PhD, Section of Rheumatology, Department of Medicine, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756. E-mail: Richard.C.Chou@hitchcock. org. Submitted for publication January 29, 2009; accepted in revised form September 14, 2009. Arthritis Care & Research Vol. 62, No. 1, January 15, 2010, pp 128–136 DOI 10.1002/acr.20024 © 2010, American College of Rheumatology
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