Systemic capillary leak syndrome with monoclonal IgG and complement alterations. A case report on an episodic syndrome.
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 percent and a decreasing plasma volume to about 50 percent of normal. During the attacks the patient was in a state of shock and BP was unmeasurable. Studies with 131I-labelled albumin during attack showed an increased transcapillary escape rate to about 20 percent/hour, compared to 6 percent when he was without symptoms. A monoclonal IgG with a constant concentration of about 5g/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 angio-edema 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.
D000328:Adult; D001772:Blood Cell Count; D001794:Blood Pressure; D001798:Blood Proteins / Q000032:analysis; D001810:Blood Volume; D002199:Capillary Permeability; D003165:Complement System Proteins / Q000032:analysis; D005440:Fluid Therapy; D006400:Hematocrit; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007213:Indomethacin / Q000627:therapeutic use; D008297:Male; D010507:Periodicity; D011239:Prednisolone / Q000627:therapeutic use; D012711:Serum Albumin, Radio-Iodinated; D013577:Syndrome; D013726:Terbutaline / Q000627:therapeutic use; D014148:Tranexamic Acid / Q000627:therapeutic use
D001798:Blood Proteins; D007074:Immunoglobulin G; D012711:Serum Albumin, Radio-Iodinated; D014148:Tranexamic Acid; D003165:Complement System Proteins; D011239:Prednisolone; D013726:Terbutaline; D007213:Indomethacin