A case of pulmonary arterial hypertension complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis.

IF 2.7 Q3 IMMUNOLOGY Immunological Medicine Pub Date : 2021-12-01 Epub Date: 2021-01-15 DOI:10.1080/25785826.2021.1874137
Hajime Yoshifuji, Sumika Kagebayashi, Hideyuki Kinoshita, Takao Fujii, Yoshiaki Okano, Masao Katsushima, Tsuneyo Mimori
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引用次数: 1

Abstract

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.

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肺动脉高压合并抗中性粒细胞细胞质抗体相关血管炎及系统性硬化1例。
肺动脉高压(PAH)是anca相关性血管炎(AAV)的罕见并发症。我们报告一例37岁男性PAH合并AAV和SSc,表现为呼吸困难、心脏增大、髓过氧化物酶(MPO)-ANCA阳性、抗着丝粒抗体、蛋白尿和尿模。右心导管检查肺动脉压升高(58/22/34 mmHg), paap低(2 mmHg)。糖皮质激素(GC)治疗降低尿蛋白和血清MPO-ANCA;而PAH对GC无应答。因此,需要联合使用伯拉前列素、波生坦和他达拉非。对GC反应的差异表明肾病的病理生理与多环芳烃不同。我们认为肾病与AAV有关,但本病例中PAH与SSc有关。我们结合9例既往病例,讨论PAH合并AAV的病理生理及治疗反应。
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来源期刊
Immunological Medicine
Immunological Medicine Medicine-Immunology and Allergy
CiteScore
7.10
自引率
2.30%
发文量
19
审稿时长
19 weeks
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