Hayashi Keigo, Watanabe Haruki, Yamamura Yuriko, Asano Yosuke, Katayama Yu, Hiramatsu-Asano Sumie, Ohashi Keiji, Morishita Michiko, Narazaki Mariko, Matsumoto Yoshinori, Sada Ken-Ei, Wada Jun
Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Medicine (Baltimore). 2021 Jan 22;100(3):e24028. doi: 10.1097/MD.0000000000024028.
Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement.
A 65-year-old man complained of a 2-week cough and fever.
Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract.
He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide.
His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated.
GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.
单纯支气管受累是肉芽肿性多血管炎（GPA）罕见的初始表现。在此，我们报告一例因支气管受累导致阻塞性肺炎的难治性GPA病例。
一名65岁男性，主诉咳嗽发热2周。
考虑到双肺因支气管阻塞或狭窄出现混浊及多处实变，对抗生素治疗无反应，且蛋白酶3-抗中性粒细胞胞浆自身抗体呈阳性，故诊断为GPA。正电子发射断层扫描-计算机断层扫描显示上呼吸道无异常发现。
给予泼尼松龙（PSL，50mg/d）及静脉注射环磷酰胺治疗。
患者全身及呼吸道症状改善。然而，PSL减至20mg/d治疗8周后，出现血管炎复发并伴有鼻窦炎和肥厚性硬脑膜炎。因此，PSL恢复至50mg/d，并开始每周给予利妥昔单抗治疗。症状逐渐缓解。
支气管受累的GPA通常难以治疗，需要密切随访，包括对上呼吸道和肥厚性硬脑膜炎的评估。
