Perry Robert, Christidis Dimitrios, Nicholson Andrew G, Schomberg Lucy, Cheent Kuldeep
Department of Gastroenterology, Frimley Park Hospital, Surrey GU16 7UJ, UK.
Department of Rheumatology, Frimley Park Hospital, Surrey GU16 7UJ, UK.
JRSM Open. 2020 Apr 2;11(4):0954406220913584. doi: 10.1177/2054270419894834. eCollection 2020 Apr.
Adult-onset Still's disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37-year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still's disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still's disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still's disease with acute fibrinous organising pneumonia.
成人斯蒂尔病是一种罕见的炎症性疾病，其特征为发热、关节炎和皮疹。它可以有多种表现形式，5%的病例伴有肺实质受累。我们报告一例37岁男性患者，最初表现为发热、体重减轻和全血细胞减少。他的病情逐渐恶化，需要无创通气，胸部计算机断层扫描显示双侧结节状浸润。开放性肺活检显示为急性纤维蛋白性机化性肺炎，对皮质类固醇治疗反应良好。三年多后，他再次出现类似的全身性疾病，不过肺部受累较轻。经过广泛的进一步检查，他被诊断为符合山口标准的成人斯蒂尔病。我们认为这个病例很重要，因为成人斯蒂尔病与间质性肺病的关联罕见。更具体地说，我们未发现任何已发表的成人斯蒂尔病合并急性纤维蛋白性机化性肺炎的病例。
