Division of Clinical Immunology & Pediatric Rheumatology, Advanced Centre for Immunology & Rheumatology, Kathmandu, 44600, Nepal.
Indian J Pediatr. 2024 Oct;91(10):1065-1071. doi: 10.1007/s12098-023-04877-2. Epub 2023 Oct 9.
Pediatric rheumatological diseases present with diverse manifestations affecting various tissues and organs. Though most childhood illnesses with immune dysregulation affect multiple organs or systems simultaneously or gradually, few inflammatory conditions affect certain organs as distinguished primary targets. Pediatric inflammatory diseases of the eye, bowel, and bone represent significant disorders with heterogeneous entities with varying pathophysiological basis like chronic inflammation, vasculitis, autoimmunity, and autoinflammation. Associations or complications of these diseases may remain obscure if their extensions are not explored specifically. Pediatric uveitis, non-uveitic inflammatory diseases, inflammatory bowel diseases, and autoinflammatory bone diseases are the important constituents of this cluster. The author concisely discusses these disorders in this review from pediatric rheumatological perspective. If untreated, these diseases may be organ-threatening. Outcomes are vastly different in undiagnosed patients and in timely diagnosed and treated patients. Lack of awareness and missed exploration of associated illnesses and complications may result in a missed diagnosis or inadequate symptomatic treatment. A multidisciplinary approach to diagnosis and treatment is desirable in these heterogeneous entities. Pediatricians must be aware of the immunological origin of organ-centered symptoms and signs. A proper understanding of molecular details of etiopathogenesis and significant advances in therapy have reduced the morbidity, disability, and mortality of such disorders.
儿科风湿性疾病表现出多种多样的表现,影响到各种组织和器官。虽然大多数免疫失调引起的儿童疾病同时或逐渐影响多个器官或系统,但很少有炎症性疾病以特定器官为明确的主要靶标。儿科眼部、肠道和骨骼的炎症性疾病是具有不同病理生理基础的异质实体的重要疾病,如慢性炎症、血管炎、自身免疫和自身炎症。如果不专门探讨这些疾病的扩展,其关联或并发症可能仍不清楚。儿科葡萄膜炎、非葡萄膜炎性炎症性疾病、炎症性肠病和自身炎症性骨病是该类疾病的重要组成部分。作者从儿科风湿病学的角度简要讨论了这些疾病。如果不治疗,这些疾病可能会危及器官。未经诊断的患者与及时诊断和治疗的患者的预后差异很大。对相关疾病和并发症认识不足或探索不足可能导致漏诊或症状治疗不足。这些异质实体的诊断和治疗需要多学科的方法。儿科医生必须了解以器官为中心的症状和体征的免疫学起源。对发病机制的分子细节有了更好的理解,并在治疗方面取得了重大进展,降低了这些疾病的发病率、残疾率和死亡率。