Immunoglobulin and Complement Studies in Children with Schönlein‐Henoch Syndrome and Other Vasculitic Diseases

ABSTRACT. In 35 children with Schonlein‐Henoch Syndrome (SHS) serum IgG, IgM, and IgA concentrations were increased in 15%, 21%, and 44% of cases, respectively. Seven children with other vasculitic syndromes (VS) had normal serum Ig concentrations. Serum concentration of IgG subclass IgG1 was increased in 72% of children with SHS and 57% with VS. In SHS this was related to the presence of arthritis, but inversely related to nephritic symptoms. Only a few children had IgC subclasses IgG2, IgG3, or IgG4 concentrations outside the normal ranges. Platelet associated Ig (PAIg) was found in 75% of children with SHS or VS. In SHS the identification of increased amounts of PAIgG was related to the presence of nephritis. The serum concentration of properdin, a component of the alternative complement system, was reduced in 21% of children with SHS. This was related to the presence of abdominal symptoms or nephritis. No cases of retinal vasculitis was observed, but 4 of 22 children with SHS had punctuate retinal haemorrhages. SHS and VS may be clinical variations of the same syndrome. The immunological aspects indicate a close relationship with autoimmune diseases.

[1]  S. Del Prato,et al.  Serum IgG subclasses and IgM imbalances in adult IgA mesangial glomerulonephritis and idiopathic Henoch-Schoenlein purpura. , 1989, Clinical and experimental immunology.

[2]  F. B. Waldos,et al.  Is Henoch-Schönlein Purpura the Systemic Form of IgA Nephropathy? , 1988 .

[3]  S. Petersen,et al.  Pattern of platelet‐associated immunoglobulin (classes and IgG subclasses) in childhood immune thrombocytopenic purpura , 1988, European journal of haematology.

[4]  H. Nielsen Epidemiology of Schönlein‐Henoch Purpura , 1988, Acta paediatrica Scandinavica.

[5]  H. Ohsawa,et al.  IgA nephropathy and Henoch-Schönlein purpura nephritis with anterior uveitis. , 1988, Nephron.

[6]  F. B. Waldo Is Henoch-Schönlein purpura the systemic form of IgA nephropathy? , 1988, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[7]  N. Blumberg,et al.  Clinical correlates in patients with elevated platelet-associated immunoglobulins. , 1988, Annals of clinical and laboratory science.

[8]  C. Koch,et al.  Congenital properdin deficiency and meningococcal infection. , 1987, Clinical immunology and immunopathology.

[9]  Howe Se,et al.  Platelet antibody binding in systemic lupus erythematosus. , 1987 .

[10]  D. Lynch,et al.  Platelet antibody binding in systemic lupus erythematosus. , 1987, The Journal of rheumatology.

[11]  M. Daha,et al.  IgA antibodies directed against cytoplasmic antigens of polymorphonuclear leukocytes in patients with Henoch-Schoenlein purpura. , 1987, Advances in experimental medicine and biology.

[12]  F. Saulsbury IgA rheumatoid factor in Henoch-Schönlein purpura. , 1986, The Journal of pediatrics.

[13]  J. Davin,et al.  Sequential Measurements of the Reticulo‐Endothelial System Function in Henoch‐Schönlein Disease of Childhood: Correlations with Various Immunological Parameters , 1985, Acta paediatrica Scandinavica.

[14]  F. Saulsbury,et al.  Cold reacting antinuclear antibody in Henoch-Schönlein Purpura. , 1983, Journal of clinical & laboratory immunology.

[15]  G. Mazzucco,et al.  Circulating immune complexes containing IgA, IgG and IgM in patients with primary IgA nephropathy and with Henoch-Schoenlein nephritis. Correlation with clinical and histologic signs of activity. , 1982, Clinical nephrology.

[16]  R. Macdermott,et al.  Similar disturbances in B cell activity and regulatory T cell function in Henoch-Schönlein purpura and systemic lupus erythematosus. , 1982, Journal of immunology.

[17]  M. Daha,et al.  Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA. , 1980, The American journal of medicine.

[18]  S. Musumeci,et al.  Lymphocyte subpopulations in anaphylactoid purpura. , 1980, Archives of disease in childhood.

[19]  R. Levinsky,et al.  IgA IMMUNE COMPLEXES IN HENOCH-SCHÖNLEIN PURPURA , 1979, The Lancet.

[20]  V. Oxelius IgG SUBCLASS LEVELS IN INFANCY AND CHILDHOOD , 1979, Acta paediatrica Scandinavica.

[21]  J. Urmson,et al.  Alteration of the complement system in children with Henoch Schönlein purpura. , 1978, Clinical immunology and immunopathology.

[22]  C. Chantler,et al.  Serum complement components in Henoch-Schönlein purpura. , 1978, Archives of disease in childhood.

[23]  Stiehm Er,et al.  Elevated serum IgA globulin in anaphylactoid purpura. , 1971 .

[24]  E. Stiehm,et al.  Elevated serum IgA globulin in anaphylactoid purpura. , 1971, Pediatrics.

[25]  D. Gairdner The Schönlein-Henoch syndrome (anaphylactoid purpura). , 1948, The Quarterly journal of medicine.