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Factor VIII inhibitor associated with nephrotic syndrome

We encountered a 39-year-old male diagnosed with nephrotic syndrome due to minimal change disease in November 2003. The haemogram and coagulation profile before biopsy were normal. He received methylprednisolone 0.8 mg kg day. His proteinuria remitted and recurred. Twelve months later, he had easy bruising and ecchymoses on the calves, feet and arms. The complete blood count was normal. He had no proteinuria by the dipstick and the serum albumin was 3.5 g dL. The coagulation profile revealed PT 12.0 s (control 13.1 s) and activated partial thromboplastin time (APTT) 58.0 s (control 25.5 s). The prolonged APTT was not correctable with 1:1 mixture of patient and normal plasma (mixing APTT 46.4 s, control 30.0 s). The factor VIII assay showed his FVIII activity was 4.7%. A FVIII inhibitor assay found the titre of inhibitor was 15.2 Bathesda units. He had a negative reaction to the antinuclear antibody and lupus anticoagulant test. We started treatment of prednisolone 1 mg kd day along with cyclophosphamide 2 mg kg day. A bone marrow biopsy was done, which was complicated by haematoma in the pelvis and right thigh and subsequent infection by Salmonella chloraesuis. Two months following treatment, he had a normal coagulation profile, with APTT 26 s (control 28.5 s). We tapered steroid and cyclophosphamide. He had fluctuation of APTT so steroid dose was reduced but not withdrawn. Throughout the treatment course of acquired haemophilia, his proteinuria never recurred. Acquired haemophilia due to FVIII inhibitor is associated with a variety of conditions including malignancies, postpartum state or autoimmune diseases [1]. While rheumatoid arthritis and systemic lupus erythematosus account for most autoimmune diseases, other entities of autoimmune aetiology are also implicated. Although both acquired haemophilia and nephrotic syndrome are autoimmune in aetiology, the association of these two entities was reported only once in literature. Sakai et al. described a 2-year-old paediatric patient with acquired haemophilia occurring 2 months following the diagnosis of nephrotic syndrome, which was treated successfully with steroid pulse treatment [2]. Although our case shared certain features with the previous one, the clinical pictures differed. Sakai’s had a paediatric case with FVIII:C <1% while our adult case had FVIII:C activity 4.7%. Sakai et al. used 3-day pulse therapy with methylprednisolone 20 mg kg day therapy while prednisolone 1 mg kg day along with cyclophosphamide 2 mg kg day were given to our patient. In Sakai’s report, the titre of FVIII inhibitor never increased after steroid pulse therapy but the patient had frequent recurrences of nephrotic syndrome. Our patient demonstrated the opposite, with nephrotic syndrome in durable remission and chronic fluctuation of prolonged APTT. As the incidence of acquired haemophilia is low, the standard treatment remains to be an unsettled question. There is great variation among reports of treatment modalities and outcomes [3]. Our case and the previously reported one demonstrated that the different clinical pictures and therapeutic options.