Gomes Rita, Padrão Eva, Dabó Hans, Soares Pires Filipa, Mota Patrícia, Melo Natália, Jesus José Miguel, Cunha Rui, Guimarães Susana, Souto Moura Conceição, Morais António
Pulmonology Department, Hospital Sousa Martins, ULS-Guarda, Guarda, Portugal Faculty of Health Sciences, University of Beira Interior, Covilhã, Portugal Pulmonology Department, Centro Hospitalar de São João, Porto, Portugal Faculty of Medicine, University of Porto, Portugal Radiology Department, Centro Hospitalar de São João, Porto, Portugal Pathology Department, Centro Hospitalar de São João, Porto, Portugal.
Medicine (Baltimore). 2016 Jul;95(27):e4073. doi: 10.1097/MD.0000000000004073.
Acute fibrinous and organizing pneumonia (AFOP) is a rare diffuse pulmonary disease, but it is not yet known whether it is a distinct form of interstitial pneumonia or simply a reflection of a tissue sampling issue.
Cross-sectional evaluation of clinical and radiological findings, treatments, and outcomes for patients with histologically confirmed AFOP at a tertiary university hospital between 2002 and 2015.
Thirteen patients (7 women, 53.8%) with a mean ± SD age of 53.5 ± 16.1 years were included. The main symptoms were fever (69.2%), cough (46.2%), and chest pain (30.8%). All patients presented a radiological pattern of consolidation and 5 (38.5%) had simultaneous ground-glass areas. Histology was obtained by computed tomography-guided transthoracic biopsy in 61.5% of cases and by surgical lung biopsy in the remaining cases. Several potential etiologic factors were identified. Eight patients (61.5%) had hematologic disorders and 3 had undergone an autologous hematopoietic cell transplant. Two (15.4%) had microbiologic isolates, 5 (38.4%) had drug-induced lung toxicity, and 2 (15.4%) were classified as having idiopathic AFOP. In addition to antibiotics and diuretics used to treat the underlying disease, the main treatment was corticosteroids, combined in some cases with immunosuppressants. Median survival was 78 months and 6 patients (46.2%) were still alive at the time of analysis.
Our findings for this series of patients confirm that AFOP is a nonspecific reaction to various agents with a heterogeneous clinical presentation and clinical course that seems to be influenced mainly by the severity of the underlying disorder.
急性纤维素性机化性肺炎（AFOP）是一种罕见的弥漫性肺部疾病，但它究竟是一种独特的间质性肺炎形式，还是仅仅是组织采样问题的一种反映，目前尚不清楚。
对2002年至2015年在一所三级大学医院组织学确诊为AFOP的患者的临床和影像学检查结果、治疗方法及预后进行横断面评估。
纳入13例患者（7例女性，占53.8%），平均年龄±标准差为53.5±16.1岁。主要症状为发热（69.2%）、咳嗽（46.2%）和胸痛（30.8%）。所有患者均表现为实变的影像学模式，5例（38.5%）同时伴有磨玻璃影区域。61.5%的病例通过计算机断层扫描引导下经胸壁活检获取组织学样本，其余病例通过外科肺活检获取。确定了几种潜在的病因。8例患者（61.5%）有血液系统疾病，3例接受了自体造血细胞移植。2例（15.4%）有微生物分离株，5例（38.4%）有药物性肺毒性，2例（15.4%）被归类为特发性AFOP。除了用于治疗基础疾病的抗生素和利尿剂外，主要治疗方法是使用皮质类固醇，部分病例联合使用免疫抑制剂。中位生存期为78个月，分析时6例患者（46.2%）仍存活。
我们对这一系列患者的研究结果证实，AFOP是对各种因素的非特异性反应，临床表现和临床过程具有异质性，似乎主要受基础疾病严重程度的影响。
