Department of Pulmonary Medicine, AIIMS Bhopal, Madhya Pradesh, India.
Sultan Qaboos Univ Med J. 2024 Aug;24(3):399-401. doi: 10.18295/squmj.12.2023.095. Epub 2024 Aug 29.
Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease with multi-system involvement. It involves the upper respiratory tract, lungs and kidneys. A 36-year-old female patient presented to a tertiary care referral hospital in Central India in 2023 with complaints of low-grade fever, dry cough and loss of appetite initially followed by dyspnoea, purpuric skin lesions, right lower limb swelling with pain and redness. Her chest radiograph revealed right upper lobe cavitary lesion with consolidation in the right lower lobe. Mycobacterium tuberculosis was detected in sputum and broncho alveolar lavage via cartridge based nucleic acid amplification assay. Later, computed tomography pulmonary angiography revealed bilateral pulmonary artery thromboembolism. Furthermore, her cytoplasmic-antineutrophil cytoplasmic antibody test was positive, serum creatinine was rising, urine microscopy had red cell casts and lower limb venous doppler revealed deep venous thrombosis. Histopathological examination of the skin lesion revealed vasculitis. Based on these findings, the patient was diagnosed with GPA. The patient improved with pulse steroids, cyclophosphamide, anticoagulants and anti-tuberculous therapy.
肉芽肿性多血管炎（GPA）是一种罕见的多系统自身免疫性疾病。它涉及上呼吸道、肺部和肾脏。2023 年，一名 36 岁的女性患者因低热、干咳和食欲不振最初出现呼吸困难、紫癜性皮肤损伤、右下肢肿胀伴疼痛和发红，到印度中部的一家三级保健转诊医院就诊。她的胸部 X 光片显示右上叶有空腔病变，右下叶有实变。通过基于试剂盒的核酸扩增检测在痰和支气管肺泡灌洗液中检测到结核分枝杆菌。后来，计算机断层肺动脉造影显示双侧肺动脉血栓栓塞。此外，她的细胞质抗中性粒细胞胞质抗体检测呈阳性，血清肌酐升高，尿液显微镜检查有红细胞管型，下肢静脉多普勒显示深静脉血栓形成。皮肤损伤的组织病理学检查显示血管炎。根据这些发现，该患者被诊断为 GPA。患者在接受脉冲类固醇、环磷酰胺、抗凝剂和抗结核治疗后有所改善。
