Zhang Chen-Xing, Yin Lei, Mao You-Ying, Zhou Zheng-Yu, Zhou Wei
Department of Nephrology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, 1678 Dongfang Road, Shanghai, China.
CEN Case Rep. 2024 Dec 11. doi: 10.1007/s13730-024-00949-0.
Secondary vasculitis is encountered in about one-third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported a few times in previous literature. We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring, simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. The presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.
继发性血管炎在所有系统性红斑狼疮（SLE）病例中约占三分之一。皮肤是狼疮相关小血管炎最常累及的部位。尽管抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎（AAV）相对不常见，但它可能是最危险的表现，死亡率很高。SLE和AAV是具有不同病理生理学的独立疾病，先前文献中仅报道过几次重叠综合征。我们报告了一例独特的小儿肺肾综合征病例，表现为肺泡出血和快速进展性肾小球肾炎。血清学和活检结果提示同时发生SLE和AAV。肾活检显示坏死性和新月形肾小球肾炎，叠加在IV级弥漫性节段性增殖性狼疮性肾小球肾炎之上。自身免疫性疾病和血管炎的表现可能是多系统的。考虑重叠综合征，特别是在患有潜在结缔组织病或系统性血管炎的患者中，对于该人群的及时治疗和预防发病至关重要。
