Immunoglobulin and complement studies in children with Schönlein-Henoch Syndrome and other vasculitic diseases.
In 35 children with Schönlein-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 IgG 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.
Petersen
S
S
Department of Paediatrics, Glostrup Hospital, Denmark.
Taaning
E
E
Söderström
T
T
Pilgaard
B
B
Tranebjaerg
L
L
Christensen
T
T
Nielsen
H E
HE
D000293:Adolescent; D001323:Autoantibodies / Q000032:analysis; D001792:Blood Platelets / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood / Q000276:immunology* / Q000473:pathology; D007070:Immunoglobulin A / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007136:Immunoglobulins / Q000032:analysis*; D007223:Infant; D010976:Platelet Count; D011414:Properdin / Q000032:analysis*; D012171:Retinal Vessels / Q000473:pathology; D014657:Vasculitis / Q000097:blood / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; D007070:Immunoglobulin A; D007075:Immunoglobulin M; D007136:Immunoglobulins; D011414:Properdin