Abstract

Scleroderma or Systemic sclerosis is a vascular and fibrotic disease with organ-based complications and high mortality and morbidity. Renal involvement can be quite varied and scleroderma renal crisis is the most severe manifestation. We report our experience. In February 2016 a 52-year-old male was admitted to hospital with pulmonary oedema and severe arterial hypertension (240/120 mmHg). From 2015 he was suffering from Scleroderma and renal failure due to biopsy proven thrombotic microangiopaty. We treated the scleroderma renal crisis with haemodialysis, plasma exchange, nitroglycerin I.V., ramipril, irbesartan and amlodipine p.o. with significant improvement. Then the patient started Extracorporeal Photopheresis, scheduled as 2 treatments every month for 6 months. After 2 months he was able to stop haemodialysis (creatinine 5 mg/dl, Glomerular filtration rate 16 ml/min). The patient completed the scheduled Extracorporeal Photopheresis cycle. After 10 months the creatinine was 4.6 mg/dl and cardiovascular and respiratory functions were stable. Scleroderma renal crisis is a life-threatening complication of Systemic Sclerosis. With the advent of ACE- inhibitors, mortality associated with SRC decreased from 76% to < 10% but up to now there is no cure for SSc. In our experience, after pharmacological therapy and haemodialysis, we performed a cycle of ECP. Further studies are needed to confirm this treatment option.

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