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Systemic Capillary Leak Syndrome with Monoclonal IgG and Complement Alterations

1979; Wiley; Volume: 206; Issue: 1-6 Linguagem: Inglês

10.1111/j.0954-6820.1979.tb13536.x

0001-6101

C.‐G. Löfdahl, L Sölvell, Anna‐Brita Laurell, Bengt R. Johansson,

Inflammatory Myopathies and Dermatomyositis

ABSTRACT A case of the rare systemic capillary leak syndrome (SCLS) is described. The patient suffered 9 attacks with muscle pain, weakness and profuse sweating. He showed increased Hct values up to 79% and a decreasing plasma volume to about 50% of normal. During the attacks the patient was in a state of shock and BP was unmeasurable. Studies with 131 I‐labelled albumin during attack showed an increased transcapillary escape rate to about 20%/ hour, compared to 6% when he was without symptoms. A monoclonal IgG with a constant concentration of about 5 g/l was found. Studies of the complement system during attack showed low C4 values, disproportions among the C1 subcomponents and C1r‐C1s‐C1IA complexes, suggesting a complement activation via the classic pathway. Hereditary angioedema was excluded by normal C1IA values. The complement activation might be part of the pathogenesis of the increased macromolecular permeability in this syndrome. A short review of cases described earlier is given.