Yoshifuji Hajime, Kagebayashi Sumika, Kinoshita Hideyuki, Fujii Takao, Okano Yoshiaki, Katsushima Masao, Mimori Tsuneyo
Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Department of Community Medicine Supporting System, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Immunol Med. 2021 Dec;44(4):263-269. doi: 10.1080/25785826.2021.1874137. Epub 2021 Jan 15.
Pulmonary arterial hypertension (PAH) is a rare complication of ANCA-associated vasculitis (AAV). We report a 37-year-old man with PAH complicated by both AAV and SSc who presented with dyspnea, cardiac enlargement, positive myeloperoxidase (MPO)-ANCA, anti-centromere antibodies, proteinuria, and urinary casts. Elevated pulmonary arterial pressure (58/22/34 mmHg) and low PAWP (2 mmHg) were confirmed by right heart catheterization. Treatment with glucocorticoids (GC) decreased urinary protein and serum MPO-ANCA; however, PAH did not respond to GC. Therefore, a combination of beraprost, bosentan, and tadalafil was needed. The differences in responses to GC suggest that the pathophysiology of nephropathy is different from that of PAH. We considered that nephropathy was associated with AAV but that PAH was associated with SSc in the present case. We discuss the pathophysiology and treatment response of PAH complicated by AAV, referring to nine past cases.
肺动脉高压（PAH）是抗中性粒细胞胞浆抗体相关性血管炎（AAV）的一种罕见并发症。我们报告一例37岁男性，患有PAH，合并AAV和系统性硬化症（SSc），表现为呼吸困难、心脏扩大、髓过氧化物酶（MPO）-ANCA阳性、抗着丝点抗体阳性、蛋白尿和尿沉渣。右心导管检查证实肺动脉压升高（58/22/34 mmHg）和肺动脉楔压降低（2 mmHg）。糖皮质激素（GC）治疗使尿蛋白和血清MPO-ANCA降低；然而，PAH对GC无反应。因此，需要联合使用贝拉前列腺素、波生坦和他达拉非。对GC反应的差异表明肾病的病理生理学与PAH不同。我们认为在本病例中，肾病与AAV有关，而PAH与SSc有关。我们参考9例既往病例，讨论了AAV合并PAH的病理生理学和治疗反应。
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