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Background: Idiopathic capillary hyperpermeability syndrome is expressed by episodes of hypovolemic shock with normal consciousness., Case Report: A 61-year-old man had a history of recurrent shock. During the shock episodes, blood pressure was non-measurable and laboratory tests showed polycythemia, low serum protein and monoclonal immunoglobulinemia. The patient also developed pneumocystosis and had an infra-His block., Discussion: The final diagnosis was idiopathic capillary hyperpermeability syndrome. This rare syndrome associates recurrent shock with IgG monoclonal immunoglobulinemia and, at the time of the shock episodes, increased hematocrit with paradoxical fall in serum protein.

It is important to study coagulation during lupus disease, not only to detect circulating anticoagulants, which may be the cause of hemorrhage, but also in order to seek chronic hypercoagulability, which is more dangerous as it may favour the development of thrombosis on inflammatory arteritis or aggravate the development of glomerular lesions. We have thus explored in this way 25 patients with systemic lupus of which 17 had histologically confirmed renal disease. 25% had hypocoagulability, but 50% had hypercoagulability which justitied treatment with heparin.