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Adult Still's disease patient developed thrombotic microangiopathy with diffuse digital gangrene

Adult onset Still’s disease (AOSD) is frequently manifested by the appearance of fever, skin rash, arthritis, sore throat, leucocytosis, hypertransaminasaemia, or hepatosplenomegaly (1). Severe lifethreatening complications, such as fulminant liver failure, acute respiratory distress syndrome, haemophagocytic syndrome, disseminated intravascular coagulation (DIC), or thrombotic microangiopathy (TMA) have been reported (2–4). We report here the case of an ASOD patient with pulmonary arterial hypertension (PAH) who developed TMA with diffuse digital gangrene in 2005 while the AOSD was still active. After immediate, aggressive treatment with pulse methylprednisolone and plasma exchange, progression of digital gangrene stopped, haemolytic anaemia and thrombocytopaenia gradually recovered. Our 42-year-old female was diagnosed with AOSD in October 1998 with the manifestations of arthritis, salmon-like skin rash, fever (up to 39.8 C̊), hyperferritinaemia (1134; normal 9–90 ng/mL), splenomegaly, and leucocytosis [white blood cell (WBC) count: 23.3610/L], fulfilling the diagnostic criteria proposed by Yamaguchi et al (1). At that time serum rheumatoid factor (RF) and anti-nuclear antibodies (ANA) were negative. Infection, malignancy, or other autoimmune diseases including Sjögren’s syndrome, systemic lupus erythematosus (SLE), and rheumatoid arthritis were also excluded after extensive studies. The patient then took prednisolone and nonsteroidal anti-inflammatory drugs (NSAIDs) irregularly but the disease activity of AOSD always remained active. Because of progressive exertional dyspnoea, pulmonary arterial hypertension (PAH) was diagnosed in April 2003, with right ventricle systolic pressure of 80 mmHg as determined by echocardiography. Other aetiologies that might contribute to the development of PAH were excluded, including anti-phospholipid syndrome (APS), in which serum anti-cardiolipin antibodies and lupus anticoagulant were negative. She was then treated with methotrexate (15 mg/week), prednisolone (10–15 mg/day), Bosentan (250 mg/day), and furosemide. On 28 April 2005, the digits of all four limbs suddenly became gangrenous (Figure 1) and the patient was drowsy the next day. At that time her vital signs were 35.5 C̊, 106/63 mmHg (blood pressure), 120/min (pulse rate), and 26/min (respiratory rate). WBC count was 10.6610/L and platelet count was 87610/L. Haemoglobin fell from 11.1 to 9.1 g/dL within 2 days after the digital gangrene developed. Urinalysis showed mild proteinuria. C-reactive protein was 12 mg/dL and erythrocyte sedimentation rate was 47 mm/h. A peripheral blood smear revealed many fragmented