Department of Respiratory Medicine, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Kure, Hiroshima, Japan.
Department of Dermatology, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Kure, Hiroshima, Japan.
Mod Rheumatol Case Rep. 2023 Jun 19;7(2):458-463. doi: 10.1093/mrcr/rxad006.
To the best of our knowledge, systemic sclerosis with overlapping characteristics of both microscopic polyangiitis and giant cell arteritis (i.e. microscopic polyangiitis involving the superficial temporal artery or giant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity) has not been reported previously. An 82-year-old woman with diffuse cutaneous systemic sclerosis experienced dyspnoea on exertion and fever. No signs of infection were observed on computed tomography. Her fever persisted despite antibiotic treatment for occult bacterial infection and secondary Clostridioides difficile-associated diarrhoea. Microscopic polyangiitis was suspected because of myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity, and giant cell arteritis was suspected as a differential diagnosis due to swelling of the superficial temporal artery. Arterial biopsy revealed inflammatory cell infiltration with granuloma formation. Based on the presence of granulomatous inflammation in the superficial temporal artery, we concluded that giant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity occurred as a complication. After glucocorticoid therapy, her fever and dyspnoea on exertion improved with a gradual decline in the serum myeloperoxidase anti-neutrophil cytoplasmic antibody levels. It is possible that vasculitis occurs as a complication in patients with systemic sclerosis in cases where the fever persists and cannot be explained by systemic sclerosis itself, infectious disease, or malignancy. Clinicians must be careful not to prematurely diagnose microscopic polyangiitis based on myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity or giant cell arteritis based on the swelling of the superficial temporal artery. Careful evaluation of the presence of granulomatous inflammation in an arterial biopsy specimen is essential to differentiate between microscopic polyangiitis and giant cell arteritis.
据我们所知，以前没有报道过同时具有显微镜下多血管炎和巨细胞动脉炎重叠特征的系统性硬化症（即累及颞浅动脉的显微镜下多血管炎或髓过氧化物酶抗中性粒细胞胞质抗体阳性的巨细胞动脉炎）。一位 82 岁的弥漫性皮肤系统性硬化症女性患者出现活动时呼吸困难和发热。计算机断层扫描未发现感染迹象。尽管对隐匿性细菌感染和继发艰难梭菌相关性腹泻进行了抗生素治疗，但她的发热仍持续存在。由于髓过氧化物酶抗中性粒细胞胞质抗体阳性，怀疑为显微镜下多血管炎，由于颞浅动脉肿胀，怀疑为巨细胞动脉炎作为鉴别诊断。动脉活检显示炎症细胞浸润伴肉芽肿形成。基于颞浅动脉存在肉芽肿性炎症，我们得出结论，发生了伴髓过氧化物酶抗中性粒细胞胞质抗体阳性的巨细胞动脉炎。在糖皮质激素治疗后，她的发热和活动时呼吸困难改善，血清髓过氧化物酶抗中性粒细胞胞质抗体水平逐渐下降。在发热持续且不能用系统性硬化症本身、传染病或恶性肿瘤解释的情况下，系统性硬化症患者可能会发生血管炎作为并发症。临床医生必须小心，不要仅凭髓过氧化物酶抗中性粒细胞胞质抗体阳性或颞浅动脉肿胀就过早诊断为显微镜下多血管炎或巨细胞动脉炎。仔细评估动脉活检标本中是否存在肉芽肿性炎症对于区分显微镜下多血管炎和巨细胞动脉炎至关重要。
