Schönlein—Henoch Nephritis

Abstract

We report the clinical and laboratory findings of 88 patients with renal manifestations of Schönlein-Henoch syndrome, all of whom were studied by renal biopsy. Sixty-six were referred to us from other centres because of diagnostic or therapeutic problems. The patients' ages ranged from two to 19 years. Evidence of 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 renal clearances of plasma proteins showed moderately or poorly selective proteinuria in most patients and appeared to have little prognostic value. The clinical manifestations of renal involvement were usually apparent early in the course of the illness, occurring within one month of onset in 75 patients. They ranged in severity from microscopic haematuria alone to a nephrotic syndrome which often followed an acute nephritic onset. Children with severe renal involvement tended to be older and to have more prolonged systemic manifestations, as well as troublesome alimentary symptoms. Light microscopy of renal biopsy specimens revealed minimal changes in 15 patients, the remainder showing proliferative glomerulonephritis ranging in severity from minor lesions of focal distribution to diffuse involvement, including crescents. The Electronmicroscopic appearance in specimens from 33 patients is also described. The duration of follwup ranged from three months to 16 years from 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, and two had deteriorating renal function. It was not possible to demonstrate any favourable effect of corticosteroids or cytotoxic drugs, used singly or in combination. There was a fairly good correlation between the severity of clinical presentation and the morphological appearances. No child with microscopic haematuria alone showed more than minor focal lesions while, on the other hand, the occurrence of epithelial crescents in more than 45 per cent of glomeruli was invariably associated with a nephrotic syndrome. A clinical presentation which included both nephritic and nephrotic features, and the involvement of more than 45 per cent of glomeruli with crescents were identified as the two factors of greatest prognostic significance.