Kliniğimizde takip edilen Henoch-Schönlein purpurası tanılı çocuk hastaların klinik ve laboratuvar özelliklerinin değerlendirilmesi

PURPOSE: We aimed to determine the region specific characteristics of pediatric patients diagnosed with Henoch-Schönlein purpura and also to evaluate the follow-up, treatment and responses of patients for whom genetic analyses was obtained for familial Mediterranean fever. MATERIALS AND METHODS: We retrospectively analyzed clinical and laboratory characteristics of 85 pediatric patients aged 0 to 18 who were diagnosed with Henoch-Schönlein purpura and who admitted to the outpatient clinics, pediatric emergency clinics or followed up for treatment at Bolu Abant İzzet Baysal University Faculty of Medicine, İzzet Baysal Training and Research Hospital Pediatrics Department between January 2011 and December 2022. RESULTS: Eighty-five patients (44 girls, 41 boys) followed up with the diagnosis of HSP were included in the study. The male/female ratio was 0.93. The mean age of the patients at the time of diagnosis was 8.54 ± 3.8 years. Consistent with the literature, most of the patients were under the age of 10 years. The disease was found to be highest in autumn (35.3%) and October in accordance with the season. Among these patients, 54 (63.6%) had a history of recent infection, and 27 (31.8%) had a history of antibacterial use in the last 15 days. xxii In terms of clinical features, rash was present in all 85 patients (100%), arthritis and arthralgia in 52 (61.2%), edema accompanying arthralgia in the extremity in 41 (48.2%), and abdominal pain in 32 (37.6%). Ten (11.8%) of the patients had vomiting, 2 (2.4%) had epididymoorchitis, 1 (1.2%) had central nervous system findings. Headache was present as a central nervous system symptom. Leukocytosis was detected in 48.2% of the patients, and elevated C-reactive protein was found in 54.1%. In terms of Familial Mediterranean Fever (FMF) and HSP association, Serum Amyloid A (SAA) was high in 11 (45.8%) of 24 patients (28%). Renal involvement was present in 52.9% of the patients; 44% had both proteinuria and hematuria, 31.1% had isolated hematuria and 24.4% had isolated proteinuria. Macroscopic hematuria was observed in 1 patient (1.2%). Eight point two % of the patients developed kidney involvement in the long-term (3 months-12 months). Nephrotic syndrome was seen in 4 patients, nephritic syndrome in 2 patients, and nephrotic and nephritic syndromes together in 1 patient. Fecal occult blood test (FOBT) was negative in 55 of 77 patients (64.7%) and positive in 22 (25.9%) patients. Hematochezia was seen in 5 patients (5.9%). There were no patients describing Melena. Abdominal ultrasonography (USG) performed due to abdominal pain was normal in 50% of the patients. In the ultrasonography of the patients, 21.9% had intra-abdominal free fluid, 3.1% had splenomegaly, 21.9% had bladder echogenicity and internal echo, 6.3% had urinary stones with crystal and echogenicity. İn 15.6% of patients lymphadenitis, and in 3.1% of them ileus had been reported. No patient had undergone surgery. xxiii While 16.5% of the patients were followed without any drug treatment, 52.9% used non steroidal anti-inflammatory drugs, 40% used antihistamines, 7.6% colchicine and 17.6% steroids. In addition to these treatments, 22.4% of the patients also used antibacterials. While steroid was used in the treatment of severe abdominal pain with positive FOB and scrotal involvement, colchicine was used due to recurrent abdominal pain and resistant-recurrent skin rashes. Rashes recurred in 9.4% of the patients who were followed up and controlled. No statistically significant difference was found between age and gender in terms of rash recurrence. A statistically significant difference was found between the appearance of the rash on the hip at the first admission (p=0.017), and the elevation of CRP and recurrence of the rash (p=0.017). There was no statistically significant relationship between the use of colchicine in the treatment and the distribution of the recurrence of the rash (p=0.146). Eight off all patients (9.4%) had a diagnosis of FMF. Although one (12.5%) of these 8 patients had a diagnosis of FMF before the diagnosis of HSP, the other 7 patients (87.5%) were diagnosed with FMF after being diagnosed with HSP, and colchicine treatment was started. In terms of familial Mediterranean fever and HSP association, genetic testing was performed in 20 patients (23.5%), genetic analyses were reported as normal in 8 patients (40%). In 60% of the genetic analyses, FMF mutations were detected. CONCLUSION: Henoch-Schönlein purpura is a vasculitis that has a very good prognosis in children, can heal without treatment and without sequelae, and that meticulous follow-up most often is sufficient. However, there are literature findings that suggesting HSP may be one of the infrequent presentation forms of FMF disease. They propose that patients with HSP, especially with recurrent and resistant rashes, should be queried in terms of FMF xxiv clinical findings and family history, and MEFV genetic analysis should be performed for FMF if they are supportive. With early diagnosis, it is possible to prevent amyloidosis, which is a complication of FMF, with colchicine treatment.