[A history of diseases: apropos of idiopathic capillary hyperpermeability syndrome]. / Une histoire de malaises: à propos du syndrome d'hyperperméabilité capillaire idiopathique.

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.

[Systemic signs of primary Gougerot-Sjögren syndrome. 48 cases]. / Signes systémiques du syndrome de Goujerot-Sjögren primitif. 48 observations.

Forty-eight patients with primary Sjögren syndrome are documented together with the results of their baseline investigations. The majority of patients were female (44 out of 48) and mean age was 63.2 years. Common clinical features included 20 parenchymal lung disease, among which 4 had interstitial pulmonary fibrosis and 2 lymphocytic interstitial pneumonitis, 12 neurologic manifestations, 16 Raynaud's phenomenon, 12 arthritis, and 3 gastrointestinal involvement. Haematological features occurred in 15 patients and another autoimmune disease was encountered in 13 cases. These extraglandular manifestations were the dominating reasons for hospital referral in 43.5% of cases, the sicca syndrome were most often only mentioned by the patients after special questioning which explain considerable delay before the diagnosis.