A case of hereditary angioneurotic edema associated with systemic lupus erythematosus

Japanese Journal of Medicine
Y SuzukiM Hara

Abstract

A pedigree of C1 inhibitor (C1 INH) deficiency associated with positive LE cell and an elevated titer of DNA antibodies and antinuclear factor (ANF) and nephropathy was presented. The proband of this family was diagnosed as having definite systemic lupus erythematosus (SLE) after a clinical course of several year since her first visit to our hospital and because of the lack of hemolytic activity of complement (CH50), in spite of the absence of idiopathic edema, C1 INH levels were 1.2 mg/dl (3.8% NHS) determined as antigen and 65 site forming unit (SFU) (5.8% NHS) determined by hemolytic assay in her blood. Her mother and brother had characteristic idiopathic edema of her face, larynx, hand and bowel and they had low levels of C1 INH of 1.8 mg/dl (5.8% NHS) and 4.8 mg/dl (15.5% NHS) respectively in their blood. On the basis of these findings, this family was diagnosed as having a pedigree of hereditary angioneurotic edema (HANE) which is supposedly an inherited autosomal positive trait. Actually, however, the proband's serological and hematological indices became positive and progressed year by year, which implies that SLE was absent for the first several years. It might be said that this interesting clinical course indicates th...Continue Reading

Citations

Sep 14, 2002·The Journal of Dermatology·Masayo KoideFukumi Furukawa
Jul 1, 1997·Clinical Immunology and Immunopathology·Y SuzukiA Yamada
Jun 4, 2011·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Henriette FarkasPéter Gergely
Feb 11, 2015·Autoimmunity Reviews·Irène Gallais SérézalOlivier Fain
Jul 1, 1991·The British Journal of Dermatology·N H CoxA Fay
Mar 21, 2000·Rheumatology·M C Pickering, M J Walport
Mar 1, 1994·The British Journal of Dermatology·W PerkinsR S Lever

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