An unusual evolution of the systemic capillary leak syndrome.

A 33-year-old black male, was referred to our department in 1981 for the following symptoms consistent with typical SCLS: several episodes of shock with generalized oedema; diffuse pain; and transient anuria. A monoclonal gammapathy of undetermined significance (MGUS) was discovered at that time. Three years later, he had minimal proteinuria (monoclonal component). Femoral artery superficialization was performed to obtain a permanent and easy vascular access, and the patient was treated during subsequent crises by colloid or gelatin fluids. Prevention of these crises was attempted unsuccessfully with corticosteroids, theophylline, cyclophosphamide, cyclosporin A, plasma exchanges, and naftazone. Beginning in 1986, transient diabetes mellitus occurred during the crises. Because of the presumably auto-immune mechanism common to both the SCLS and the episodes of diabetes mellitus, the patient began treatment with intravenous polyvalent immunoglobulins. These reduced the intensity of his crises, with only abdominal pain, moderate hypotension and weight gain. In 1991, the patient developed permanent diabetes mellitus and, beginning in 1999, diabetic retinopathy, progressive neuropathy and nephropathy. In May 2000, the patient had acute renal failure and persistent proteinuria, and a renal biopsy was performed. Optical examination showed osmotic nephrosis-like lesions in the podocytes and most tubules, but no amyloidosis. On immunofluorescence, some polyclonal depositions were seen in glomeruli as well as tubular basement membranes. Electronmicroscopy showed basal thickening, without fibrillar or granular deposition, and mesangial expansion, suggesting an early dia betic nephropathy. The patient recovered renal function after this episode. However, he presented more frequent crises, weight loss, thoracic pain and, finally, worsening cardiac function. A pericardial effusion was drained. Pericardial biopsy showed no amyloidosis despite evidence revealed by echocardiography. A bone marrow aspiration showed 80% abnormal plasmocytes, confirming the diagnosis of myeloma. M-component serum level was 18 gul. Humeral lacunae were seen on an X-ray. Cutaneous and salivary biopsies revealed AL amyloidosis. In April 2001, treatment was begun with melphalan and prednisone. However, the patient died after two treatment courses from cardiac and hepatic amyloidosis.