Kusaka Yu, Oba Takehiko
Department of Respiratory Medicine, Ome Municipal General Hospital, Ome, JPN.
Cureus. 2024 Apr 25;16(4):e59037. doi: 10.7759/cureus.59037. eCollection 2024 Apr.
This case study presents a rare occurrence of acute respiratory failure in a 17-year-old male diagnosed with common variable immunodeficiency (CVID) and granulomatous lymphocytic interstitial lung disease (GLILD), which typically have a gradual onset. The patient initially exhibited nonspecific symptoms such as dry cough and fever but quickly progressed to severe respiratory failure despite conventional treatments. Imaging showed extensive lung abnormalities, and blood tests revealed significantly low immunoglobulin levels, indicating an underlying immunodeficiency. Treatment with high-dose steroids and immunoglobulin replacement therapy resulted in a rapid and remarkable recovery of lung function. Lung biopsies confirmed the dual diagnoses of CVID and GLILD, emphasizing the challenge of diagnosing and managing GLILD in CVID patients. This case underscores the importance of early and aggressive intervention in improving outcomes for GLILD patients with acute respiratory distress.
本病例研究呈现了一名17岁男性罕见地发生急性呼吸衰竭的情况,该男性被诊断患有常见变异型免疫缺陷(CVID)和肉芽肿性淋巴细胞间质性肺病(GLILD),这两种疾病通常起病较为隐匿。患者最初表现出干咳和发热等非特异性症状,但尽管接受了常规治疗,病情仍迅速进展为严重呼吸衰竭。影像学检查显示肺部有广泛异常,血液检查显示免疫球蛋白水平显著降低,提示存在潜在的免疫缺陷。高剂量类固醇和免疫球蛋白替代疗法治疗后,肺功能迅速且显著恢复。肺活检证实了CVID和GLILD的双重诊断,凸显了在CVID患者中诊断和管理GLILD的挑战。本病例强调了早期积极干预对于改善急性呼吸窘迫的GLILD患者预后的重要性。