Pediatrics, Om Hospital and Research Centre, Kathmandu, Bagmati, Nepal
Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
BMJ Case Rep. 2022 Feb 9;15(2):e248477. doi: 10.1136/bcr-2021-248477.
Polyarteritis nodosa (PAN) is a medium vessel vasculitis with necrotising vascular changes along with multisystemic involvement. Due to variable initial presentations, diagnosis of systemic PAN in children requires a comprehensive work up. In addition, systemic PAN needs an aggressive therapy. Mycophenolate mofetil is an emerging newer alternative for the treatment of PAN. We report a case of childhood systemic PAN who initially presented with subtle signs like reduced sensation over lateral foot, non-deforming arthritis and multiform rashes. After comprehensive aetiological work up, nerve biopsy and supporting evidence clinched the diagnosis. Vasculitis in children presenting with benign subtle signs is sometimes a diagnostic challenge to clinicians. Our case highlights the importance of lateral thinking while dealing with non-specific multisystemic signs. Evidence of successful treatment of PAN with mycophenolate mofetil is gradually being built up. It is also described to result lower relapse and increased treatment free survival rate.
结节性多动脉炎（PAN）是一种中等大小血管炎，伴有坏死性血管改变和多系统受累。由于初始表现多样，儿童系统性 PAN 的诊断需要全面的检查。此外，系统性 PAN 需要积极的治疗。霉酚酸酯是治疗 PAN 的一种新兴替代药物。我们报告了一例儿童系统性 PAN 病例，其最初表现为轻微症状，如足外侧感觉减退、非变形性关节炎和多形性皮疹。经过全面的病因学检查、神经活检和支持性证据，确诊了该病例。儿童血管炎表现为良性轻微症状，有时对临床医生来说是一个诊断挑战。我们的病例强调了在处理非特异性多系统症状时进行发散思维的重要性。霉酚酸酯治疗 PAN 的成功证据正在逐渐建立。它还被描述为降低复发率和提高无治疗生存率。
