Adult onset Still’s disease in a patient with scleroderma: case report

Abstract

Background: Scleroderma and adult onset Still’s disease (AOSD) are both uncommon autoimmune disorders. These two disorders have rarely been documented to occur simultaneously. In fact, after a thorough literature review, we discovered only one prior case report in a pregnant individual. Here, we describe the first documented case of scleroderma and AOSD in a postmenopausal patient. Case presentation: The patient is a 61-year-old Caucasian female with a past medical history significant for peptic ulcer disease, mitral valve prolapse, chronic idiopathic pancreatitis, and limited cutaneous scleroderma with sclerodactyly, Raynaud’s, and calcinosis. She was sent to the emergency room by her primary care physician due to one-week history of intermittent spiking fevers (Tmax 101°F), sore throat, myalgias, arthralgias, and non-pruritic bilateral lower extremity rash. Diagnostic evaluation in the hospital included complete blood count, comprehensive metabolic panel, respiratory viral panel, antinuclear antibody panel, bone marrow biopsy, and imaging with computerized tomography. Our patient fulfilled Yamaguchi Criteria for AOSD and all other possible etiologies were ruled out. She was treated with a steroid taper and methotrexate was initiated on post-discharge day number fourteen. Clinical and biochemical resolution was obtained at three months. Conclusions: In this report, we describe the first ever documented case of scleroderma and AOSD in a postmenopausal patient. The clinical presentation, diagnostic work up, and management discussed herein may serve as a framework for which rheumatologists and other physicians may draw upon in similar future encounters.