| Abstract: | We report the clinical and laboratory findings of 88 patientswith renal manifestations of Schönlein-Henoch syndrome,all of whom were studied by renal biopsy. Sixty-six were referredto us from other centres because of diagnostic or therapeuticproblems. The patients' ages ranged from two to 19 years. Evidenceof recent streptococcal infection was obtained in 33 children,but their illness was indistinguishable from that of the remainder.Serum C3 levels were normal in all patients. Differential renalclearances of plasma proteins showed moderately or poorly selectiveproteinuria in most patients and appeared to have little prognosticvalue. The clinical manifestations of renal involvement were usuallyapparent early in the course of the illness, occurring withinone month of onset in 75 patients. They ranged in severity frommicroscopic haematuria alone to a nephrotic syndrome which oftenfollowed an acute nephritic onset. Children with severe renalinvolvement tended to be older and to have more prolonged systemicmanifestations, as well as troublesome alimentary symptoms. Light microscopy of renal biopsy specimens revealed minimalchanges in 15 patients, the remainder showing proliferativeglomerulonephritis ranging in severity from minor lesions offocal distribution to diffuse involvement, including crescents.The Electronmicroscopic appearance in specimens from 33 patientsis also described. The duration of follwup ranged from three months to 16 yearsfrom onset. Three children died within nine months of onset;of 59 survivors who were followed up for more than two yfiirs,34 were normal when last seen, 17 had minor urinary abnormalities,six had heavy proteinuria with or without hypertension, andtwo had deteriorating renal function. It was not possible todemonstrate any favourable effect of corticosteroids or cytotoxicdrugs, used singly or in combination. There was a fairly good correlation between the severity ofclinical presentation and the morphological appearances. Nochild with microscopic haematuria alone showed more than minorfocal lesions while, on the other hand, the occurrence of epithelialcrescents in more than 45 per cent of glomeruli was invariablyassociated with a nephrotic syndrome. A clinical presentationwhich included both nephritic and nephrotic features, and theinvolvement of more than 45 per cent of glomeruli with crescentswere identified as the two factors of greatest prognostic significance.
1Present address: Department of Paediatrics and Child Health,27 Blundell Street, Leeds LS1 3ET
2Present address: Derbyshire Children's Hospital, North Street,Derby |
