Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report.
Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report.
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DOI：
10.1155/2012/549298
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发表时间：
2012
影响因子：
--
通讯作者：
Serrano EV
中科院分区：
文献类型：
--
作者：
Valim V;Rocha RH;Couto RB;Paixão TS;Serrano EV
急性化脓性和机化性肺炎（AFOP）是最近描述的一种组织学类型，其特征是肺泡腔内存在纤维蛋白球。术语未分化结缔组织病（UCTD）用于识别不符合分类为确定性结缔组织病标准的自身免疫性系统性疾病。AFOP从未被报道与UCTD相关。目前报告的病例是一名39岁白人女性，有干咳和进行性呼吸困难。8个月后，根据临床病史和放射学影像，她被诊断为“机化性肺炎”。她表现出雷诺现象，干燥综合征，靴子和手套神经性疼痛，和以前的甲状腺功能减退。抗核抗体、类风湿因子和特异性自身抗体均为阴性。唾液腺活检和神经肌电图正常。毛细血管镜检查显示“硬皮病”模式，毛细血管缺失和扩张。她经历了临床和放射学恶化。尽管接受环磷酰胺脉冲治疗，她仍发生出血，然后死亡。开胸肺标本显示AFOP的组织学类型。这篇文章显示了AFOP与UCTD的罕见联系。
Acute fibrinous and organizing pneumonia (AFOP), recently described, is a histologic pattern characterized by the presence of fibrin “balls” within alveolar spaces. The term undifferentiated connective tissue disease (UCTD) is used to identify autoimmune systemic diseases that do not fulfill the criteria to be classified as a definitive connective tissue disease. The AFOP has never been reported in association with UCTD. The present reported case is a 39-year-old Caucasian, female with dry cough and progressive dyspnea. Eight months later, she was diagnosed with “organizing pneumonia” based on clinical history and radiologic images. She manifested Raynaud's Phenomenon, sicca syndrome, boot and gloves neuropathic pain, and previous hypothyroidism. Antinuclear antibody, rheumatoid factor, and specific autoantibodies were negative. Salivary gland biopsy and electroneuromyiography were normal. The capillaroscopy showed a “scleroderma” pattern with capillary deletion and ectasia. She experienced clinical and radiologic worsening. Despite being submitted to cyclophosphamide pulse, she developed hemorrhage and then died. Thoracotomy pulmonary specimen showed histological pattern of AFOP. This paper shows a rare association of AFOP with UCTD.