Granulomatosis with Polyangiitis and Diffuse Alveolar Hemorrhage with Difficult Differential Diagnosis from Adenocarcinoma

DOI
  • Yamamoto Yuji
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Urasaki Koji
    Department of Pathology, National Hospital Organization Osaka Toneyama Medical Center
  • Tsujino Kazuyuki
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Kuge Tomoki
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Matsuki Takanori
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Fukushima Kiyoharu
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Kagawa Hiroyuki
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Miki Mari
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Miki Keisuke
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center
  • Kida Hiroshi
    Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center

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<p>Background. Granulomatosis with polyangiitis (GPA) is a small vessel, systemic vasculitis that is classified as an anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis. The cytology of bronchoalveolar lavage fluid (BALF) in GPA has never been thoroughly investigated due to the small number of patients. Case. We report the case of a 62-year-old man who was admitted to our hospital with a one-week history of exertional dyspnea and hemosputum. He had a history of chronic sinusitis, acute otitis media, and bronchial asthma for three years. The patient's serology was positive for anti-proteinase 3 ANCA. On high-resolution computed tomography (HRCT), diffuse consolidation and ground-glass opacities were observed in the lung fields. The patient underwent bronchoscopy and was diagnosed with diffuse alveolar hemorrhage (DAH). Moreover, adenocarcinoma in situ (AIS) was strongly suspected because the BALF showed countless atypical glandular cells. The histological findings obtained from transbronchial lung biopsy also supported the diagnosis of AIS. After treatment with corticosteroid pulse therapy; however, the pulmonary shadows on HRCT significantly improved. Moreover, in the lung specimens taken at bronchoscopy, organizing pneumonia was observed, without malignancy. Hence, AIS was finally excluded from the diagnosis. The patient was diagnosed with GPA based on the history of chronic sinusitis and pulmonary symptoms. He was successfully treated with a combination of corticosteroids and immunosuppressants. Conclusion. This is a rare case of GPA and DAH that mimicked AIS due to cellular atypicality. Even when histology supports a diagnosis of malignancy, GPA should be included in the differential diagnosis to prevent diagnostic delay and treatment failure.</p>

収録刊行物

  • 気管支学

    気管支学 42 (6), 501-506, 2020-11-25

    特定非営利活動法人 日本呼吸器内視鏡学会

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