Hereditary deficiency of the third component of complement in two sisters with systemic lupus erythematosus-like symptoms.

We observed two sisters with lupus-like syndrome with homozygous C3 deficiencies. A 19-year-old woman and her 15-year-old sister developed malar rash, arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The older sister experienced recurrent bronchitis in her childhood, but the younger sister had no recurrent infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and C3 hemolytic activity were extremely low.

[1]  A. P. Ball,et al.  Recurrent bacterial meningitis in patients with genetic defects of terminal complement components. , 1980, Clinical and experimental immunology.

[2]  B. Pussell,et al.  COMPLEMENT DEFICIENCY AND NEPHRITIS A Report of a Family , 1980, The Lancet.

[3]  B. H. Petersen,et al.  Neisseria meningitidis and Neisseria gonorrhoeae bacteremia associated with C6, C7, or C8 deficiency. , 1979, Annals of internal medicine.

[4]  V. Agnello.  Association of systemic lupus erythematosus and SLE-like syndromes with hereditary and acquired complement deficiency states. , 1978, Arthritis and rheumatism.

[5]  V. Agnello.  COMPLEMENT DEFICIENCY STATES , 1978, Medicine.

[6]  H. Colten,et al.  Homozygous C3 deficiency: detection of C3 by radioimmunoassay. , 1977, Clinical immunology and immunopathology.

[7]  T. Lint,et al.  Hereditary deficiency of the third component of complement in a child with fever, skin rash, and arthralgias: response to transfusion of whole blood. , 1977, The Journal of pediatrics.

[8]  J. Prahl,et al.  Third component of human complement: purification from plasma and physicochemical characterization. , 1976, Biochemistry.

[9]  S. Ebisu,et al.  Activation of the alternative complement pathway by water-insoluble glucans of streptococcus mutans: the relation between their chemical structures and activating potencies. , 1976, Journal of immunology.

[10]  S. Ahlstedt,et al.  A C1q Immunosorbent Assay Compared with Thin‐Layer Gel Filtration for Measuring IgG Aggregates , 1976, Scandinavian Journal of Immunology.

[11]  M. Schonland,et al.  A family with partial and total deficiency of complement C3. , 1976, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[12]  R. Simmons,et al.  Chemotaxis under agarose: a new and simple method for measuring chemotaxis and spontaneous migration of human polymorphonuclear leukocytes and monocytes. , 1975, Journal of immunology.

[13]  N. Day,et al.  Complete absence of the third component of complement in man. , 1975, The Journal of clinical investigation.

[14]  K. Inoue,et al.  Chemical studies on damages of Escherichea coli by the immune bactericidal reaction. II. Release of phosphatidylethanolamine from a phospholipase A-deficient mutant of E. coli during the immune bactericidal reaction. , 1974, Biken journal.

[15]  H. Kitamura,et al.  Molecular analysis of the reaction of C9 with EAC1-8: reaction of C9 with EAC1-8. , 1974, Journal of immunology.

[16]  O. Götze,et al.  A SERUM FACTOR IN CHRONIC HYPOCOMPLEMENTEMIC NEPHRITIS DISTINCT FROM IMMUNOGLOBULINS AND ACTIVATING THE ALTERNATE PATHWAY OF COMPLEMENT , 1974, The Journal of experimental medicine.

[17]  K J Bloch,et al.  Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3. , 1973, The New England journal of medicine.

[18]  H. Colten,et al.  Homozygous deficiency of C3 in a patient with repeated infections. , 1972, Lancet.

[19]  N. Cooper,et al.  THE REACTION MECHANISM OF HUMAN C5 IN IMMUNE HEMOLYSIS , 1970, The Journal of experimental medicine.

[20]  P. Teisberg.  High Voltage Agarose Gel Electrophoresis in the Study of C 3 Polymorphism , 1970, Vox sanguinis.

[21]  R. Good,et al.  Defective candidacidal capacity of polymorphonuclear leukocytes in chronic granulomatous disease of childhood. , 1969, The Journal of pediatrics.

[22]  C. West,et al.  Serum C'3 Lytic System in Patients with Glomerulonephritis , 1969, Science.

[23]  J. White,et al.  In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. , 1967, The Journal of clinical investigation.

[24]  C. Laurell,et al.  Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. , 1966, Analytical biochemistry.

[25]  H. Colten,et al.  Homozygous human C3 deficiency. The role of C3 in antibody production, C-1s-induced vasopermeability, and cobra venom-induced passive hemolysis. , 1976, The Journal of clinical investigation.