Santos Cláudia, Oliveira Rui Caetano, Serra Paula, Baptista João Pedro, Sousa Eduardo, Casanova Paula, Pimentel Jorge, Carvalho Lina
Pulmonology Department, Coimbra University Hospital, Portugal.
Pathology Department, Coimbra University Hospital, Portugal.
Pathophysiology. 2019 Sep-Dec;26(3-4):213-217. doi: 10.1016/j.pathophys.2019.04.001. Epub 2019 May 2.
Acute Fibrinous and Organizing Pneumonitis (AFOP) is a disease with histopathological pattern characterized by the presence of intra-alveolar fibrin in the form of fibrin "balls" and organizing pneumonia represented by inflammatory myofibroblastic polyps. Symptoms of this rare interstitial pulmonary disease can be either acute or sub-acute and it can rapidly progress to death. Diagnosis should be considered in the Intensive Care Unit (ICU) if patients' symptomatology and radiology correlates with non-responding or progressive pneumonia and when morphology, on biopsies, encompasses criteria of diffuse alveolar damage (DAD) and organizing pneumonia (OP) balancing in between. Three clinical cases of patients presenting severe lung disease requiring mechanical ventilation and prolonged intensive care fitted on the variable spectra of AFOP histopathology and had poor outcome: a 23 year-old women had AFOP in the context of antiphospholipid syndrome pulmonary compromise; a 35 year-old man developed a letal intensive care pneumonia with AFOP pattern registered in post-mortem biopsy; and a 79 year-old man died 21 days after intensive care unit treatment of a sub-pleural organizing pneumonia with intra-alveolar fibrin, seen in post-mortem biopsy. The predominance of acute fibrin alveolar deposition pattern is helpful in raising AFOP differential diagnosis while organizing pneumonia pattern establishes a wider range of diagnosis that can go till solitary pulmonary nodule, remaining indefinite to suggest diagnosis. The performance time of biopsy in a larger number of clinical cases may be helpful in establishing the evolutionary morphological pattern, taking in mind the poor outcome of the disease, deserving rapid diagnosis to define treatment.
急性纤维素性机化性肺炎（AFOP）是一种具有组织病理学特征的疾病，其特征为肺泡内存在纤维蛋白“球”形式的纤维素以及以炎性肌成纤维细胞息肉为代表的机化性肺炎。这种罕见的间质性肺疾病的症状可以是急性或亚急性的，并且可能迅速进展至死亡。如果患者的症状和影像学表现与无反应或进行性肺炎相关，且活检时形态学符合弥漫性肺泡损伤（DAD）和机化性肺炎（OP）之间的平衡标准，则应在重症监护病房（ICU）考虑诊断。三例患有严重肺部疾病需要机械通气和长期重症监护的患者符合AFOP组织病理学的不同谱型，且预后不良：一名23岁女性在抗磷脂综合征肺部受累的情况下患有AFOP；一名35岁男性发生致命性重症监护肺炎，尸检活检显示为AFOP型；一名79岁男性在重症监护病房治疗亚胸膜机化性肺炎伴肺泡内纤维蛋白21天后死亡，尸检活检可见该病变。急性纤维素肺泡沉积模式占主导有助于提高AFOP的鉴别诊断，而机化性肺炎模式则建立了更广泛的诊断范围，可直至孤立性肺结节，诊断仍不明确。考虑到该疾病预后不良，活检在大量临床病例中的执行时间可能有助于确立演变的形态学模式，因此需要快速诊断以确定治疗方案。
