Clinical Characteristics, Diagnostic Findings and Therapeutic Outcome of Children Suffering Henoch-Schonlein Purpura: A Survey of Iranian Children

Despite the spread of Henoch-Schonlein purpura (HSP) in all societies, especially in Asian children, no comprehensive study on HSP has been conducted in Iranian children and most of these reports are limited to disease cases or exclusively to patients with HSP. Therefore, this study was conducted to describe the clinical, diagnostic, and therapeutic approaches in children with HSP in Iran. Methods: This historical cohort study was performed in all children suffering from HSPN hospitalized at Ali-Asghar Children’s Hospital, Tehran between April 2006 and March 2017. The patients' baseline characteristics including demographics, clinical symptoms and laboratory parameters were all collected from hospital files. The patients were followed up for at least six months of initiating treatment and also for 12 to 120 months after treatment. Results: Of 100 patients with HSP, 18 (11 boys and 7 girls) had indications for biopsy that were included in the study. The mean age of the participants was 7.72 ± 2.69 years. Nephrotic syndrome was found in 44.4% and nephritic syndrome in 61.1% of the patients. Hematuria was found in 66.7%, proteinuria in 66.7%, and hypertension in 38.9% of the patients. The mean serum creatinine was 1.0 ± 0.6 mg/dl with a mean GFR of 95 ± 5ml/min. Regarding pathological classification, 33.3% had class II and 66.7% had class III. With respect to therapeutic regimen, 61.1% were treated only with steroids while others were treated with a combination of steroids and immunosuppressant drugs. During the follow-up time, all patients were treated successfully with the mentioned regimens. In all treated subjects, proteinuria disappeared in all urine samples. Due to complete improvement in all patients, repeated renal biopsy was not indicated. Conclusion: Kidney involvement occurs as nephritic syndrome in about two thirds of patients and as nephrotic syndrome in the remaining cases. In the majority of patients, treatment with steroids alone is successful although combined therapy with immunosuppressant drugs is required in the remaining patients. In summary, the therapeutic protocols are associated with a significant long-term recovery (a five-year recovery of 87.5% in our study).