Thrombotic microangiopathy in adult onset Still's disease: a case report

Thrombotic microangiopathy (TMA) is a rare but life-threating disorder. Coexistence of thrombotic microangiopathy and adult onset Still's disease (AOSD) is extremely rare. A 34-year-old man was admitted to the hospital for a 4-week history of fever, arthralgias, diffuse muscle pain and skin rash. Physical examination revealed a temperature of 39o C, bilateral inflammation of the metacarpophalangeal joints, peripheral lymphadenopathy and a nonpruritic, macular rash. Initial laboratory findings revealed elevated erythrocyte sedimentation rate (ESR) 89mm/h (normal values [n.v.] 3-23 mm/h), C-reactive protein (CRP) 148 mg/l (n.v. <5.0 mg/l), white blood cell count (WBC) 17.3x109/l (n.v. 4-10), platelets 561x109/l (n.v. 140-440) and a normocytic anemia with a hemoglobin of 11.8 g/dl (n.v. 14-18 g/dl). Serum ferritin level was higly elevated 7999 µ g/l (n.v. 10-300 µ g/l). Other biochemical values revealed elevated aspartate aminotransferase, alanine aminotransferase, lactic acid dehydrogenase, gamma-glutamyl transferase, alcal phosphatase and a normal total bilirubin level. Extensive diagnostic workout excluded infectious, malignant and hematological disease. The patient was diagnosed with AOSD and successfully treated with high dose intravenous glucocorticoids. Five months after presentation, patient was doing well on oral glucocorticoids and we initiated chloroquine phosphate. Nine months after the onset of adult Still’ s disease, patient presented with asymptomatic thrombocytopenia during chloroquine therapy. Physical status was unremarkable except for the pallor skin and mucosa. Laboratory evaluation revealed: platelets 27 x109/l, RBC 2.89x1012/l, Hct 0.279 L/L (n.v. 0.41-0.55 L/L), Hb 94 g/l (9.4 g/dL), Rtc 4.20 % (normal 0.5-2.15 %), WBC 6.5 x109/l, ESR 23 mm/h, CRP 4.1 mg/l. LDH, total bilirubine and indirect bilirubine levels were elevated. Haptoglobin level was decreased to 0.1 g/l (n.v. 0.3-2 g/l). The renal function tests were all normal. Peripheral blood smear showed frequent schistocytes. Based on the presence of thrombocytopenia and microangiopathic hemolytic anemia, with exclusion of other known causes, patient was diagnosed with thrombotic microangiopathy and successfully treated with plasma exchange and intravenous glucocorticoids. Awareness of the possible development of thrombotic microangiopathy in patients with adult onset Still's disease is critical, so as to initiate early treatment and to prevent complications and recurrence of thrombotic microangiopathy.