剖検は患者ケアの進歩に欠かせないものである。剖検で診断された進行性特発性間質性肺炎の異常な呈示と致死的転帰の報告

Autopsies are indispensable in the advancement of patient care: Report of an unusual presentation and fatal outcome of an autopsy-diagnosed case of advanced idiopathic interstitial pneumonia.

• Evi Abada
• Kunil Raval

抄録

特発性間質性肺炎（IIP）は、線維化性肺疾患の一群であり、患者に重大な罹患率と死亡率をもたらす。特発性間質性肺炎の患者の多くは、まず呼吸困難および／または咳嗽を呈する。ここでは53歳の男性の症例を報告する。症状は軽度の呼吸困難と咳嗽であった．身体検査と胸部CT検査で肺炎が示唆され，複数の抗生物質を投与されたが，呼吸が悪化して高気圧酸素を必要とし，10日後に死亡した．剖検した肺標本の病理組織学的検査の結果，びまん性肺胞損傷の混合型IIPパターンに続発した重度の肺損傷が認められ，広範な間質性線維症の上にハニカムの特徴が重なっており，進行した間質性/末期肺疾患と一致した．この症例はIIPの珍しい、致命的なプレゼンテーションを代表するものであり、臨床現場で役立つかもしれません。

Idiopathic interstitial pneumonias (IIPs) are a group of fibrosing lung disorders conferring significant morbidity and mortality to patients. Most patients with IIP first present with dyspnea and/or cough. Here, we report the case of a 53-year-old male who presented with severe abdominal pain and weight loss of approximately 100 pounds of 3-month duration. Symptoms of mild dyspnea and cough were obtained during additional history taking. Physical examination and computed tomography of the chest were suggestive of pneumonia, and he was placed on multiple antibiotics but developed worsening respiration that necessitated hyperbaric oxygen and died after 10 days. Histopathological examination of autopsy lung specimen, revealed severe lung damage secondary to a mixed IIP pattern of diffuse alveolar damage, superimposed on extensive interstitial fibrosis, with features of honeycombing, consistent with advanced interstitial/end-stage lung disease. This case typifies an unusual and fatal presentation of IIP, which may be useful in clinical practice.