A Clinical and Pathological Study on the Treatment and Course of Purpura Nephritis in Chidren

ABSTRACT

Purpura nephritis, one of the commonly known complications of allergic purpura has variable symptoms ranging from mild transient hematuria to severe nephrotic syndrome leading to renal failure and death. This paper reports on the treatment and course of purpura nephritis with special reference to serum immunoglobulins and immunopathology. These cases were selected among pediatric patients with purpura nephritis admitted to the pediatric department of Severance Hospital, Yonsei University College of Medicine from Jan., 1978 to Apr., 1980. The results are as follows 1. Among 18 patients with purpura nephritis whose serum immunoglobulin level had been checked at least once in the course of the disease, 14 cases were male and 4 female. The age distribution was from 3 years and 5 months to 15 years of age, and the peak incidence occurred at 7 years of age in 5 cases. 2. Clinical classifications were made according to clinical symptoms and laboratory results. These classifications were as follows nephrotic syndrome 5 cases, acute nephritis 5 cases, persistent hematuria and proteinuria 3 cases, and persistent proteinuria, recurrent hematuria, recurrent hematuria and proteinuria, transient hematuria and others, 1 case each. 3. Remission occurred in 5 cases which were all male and their clinical classifications were acute nephritis 3 cases, transient hematuria 1 case and recurrent hematuria 1 case. All of these showed mild renal involvement and none had nephrotic syndrome. 4. Remission occurred in 3 cases among 7 with prednisolone therapy, whereas only 1 case showed remission among 8 cases of combined therapy with immunosuppressant. Remission occurred in 1 case without any therapy. But no therapy was specifically "effective." 5. Nine out of 18 cases(50%) developed signs of renal involvement within 15 days of onset of purpura, and almost all cases(90%) within 2 months. Nephritis preceded purpura in 2 cases by 14 days and 4 months respectively and in another case, nephritis appeared 4 years after purpura. With such variation in duration, no correlation existed between the time interval from nephritis to purpura and the course of the disease. 6. Renal biopsies revealed 14 cages of focal proliferative glomerulonephritis and 1 case of diffuse proliferative glomerulonephritis, but none showed significant crescent formation in the glomeruli. Immunofluorescent microscopic examination revealed granular deposits of Ig(G, A, M), C3/C4, fibrinogen in 8 cases, IgM was absent in 6 cases and IgG absent in 1 case. There wag no correlation with the course of the disease. Sites of deposits were mainly in the mesangium. 7. Serum levels of immunoglobulin and complement were checked in all cases. IgG was elevated in 1 case and IgA was elevated at least once in the course of the disease in 9 cases among 18(50%). IgM mas within normal limits in all. Elevation of C3 was noticed in 2 cases but levels of C4 were normal in all. 8. Among 9 casas with elevated serum IgA in the acute phase, 6 cases showed a drop in the serum IgA level to normal, mostly within 6 months; but this factor was not related to the course and prognosis of the disease since 9 other cases which showed no elevation of serum IgA were not otherwise significantly different.