Seyahi Emire, Melikoglu Melike, Akman Canan, Hamuryudan Vedat, Ozer Harun, Hatemi Gulen, Yurdakul Sebahattin, Tuzun Hasan, Oz Buge, Yazici Hasan
From Division of Rheumatology (ES, MM, VH, GH, SY, HY); Department of Radiology (CA, HO); Department of Cardiovascular Surgery (HT); and Department of Pathology (BO); Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey.
Medicine (Baltimore). 2012 Jan;91(1):35-48. doi: 10.1097/MD.0b013e318242ff37.
Pulmonary artery aneurysms (PAAs) are well known causes of mortality and morbidity in Behçet disease (BD). However, pulmonary artery involvement in BD is not limited to PAA; the other main type of pulmonary artery involvement is pulmonary artery thrombus (PAT), with or without associated PAA. In addition, other types of lung disease like nodules and cavities in the lung parenchyma are frequently associated with pulmonary artery involvement, and can be misinterpreted as being due to infection. We surveyed the clinical, radiologic, and laboratory characteristics and outcome of 47 BD patients with pulmonary artery involvement and the associated findings, all seen and followed at a single dedicated tertiary care center.We identified 47 (41 male, 6 female) patients in whom pulmonary artery involvement was diagnosed, who were registered in the multidisciplinary clinic at Cerrahpasa Medical Faculty between January 2000 and December 2007. Mean age at diagnosis was 29 ± 8 years, and mean disease duration to the onset of pulmonary artery involvement was 3.6 ± 4.8 years. Hemoptysis was the most common presenting symptom (79%) followed by cough, fever, dyspnea, and pleuritic chest pain. Thirty-four of 47 patients (72%) presented with PAA, including 8 with associated PAT. The remaining 13 patients (28%) had isolated PAT. Patients with isolated PAT in general have clinical features similar to patients with PAA. However, hemoptysis was less frequent and voluminous in patients with isolated PAT. Most (91%) of the patients had active disease outside the lungs when they presented with pulmonary artery involvement.Forty (85%) patients had nodules and 6 (13%) had cavities when first seen. Peripheral venous thrombosis was present in 36 of 47 (77%) patients, and intracardiac thrombi in 12 of the 36 (33%) patients. Nodules, cavities, and intracardiac thrombi were mainly present in the acute stages of pulmonary artery involvement.Pulmonary artery involvement is usually multiple, and involves mostly descending branches of the pulmonary artery. Pulmonary artery involvement may disappear, but arterial stenosis or occlusions usually develop at the same location. After a mean follow-up of 7 years, 12 of 47 (26%) patients were dead; patients with larger aneurysms were more likely to die. Sixteen of 47 (34%) patients were symptom free, and the remaining 40% had mild dyspnea (13/47) and/or small bouts of hemoptysis (8/47).Pulmonary artery pressure may be elevated, and may indicate a poor prognosis. Mediastinal lymphadenopathy and mild pleural and pericardial effusions may also be observed. Corticosteroids and immunosuppressive agents are the mainstays of treatment; however, refractory cases may require embolization, lobectomy, cavitectomy, and decortication.
肺动脉瘤（PAA）是白塞病（BD）中众所周知的致死和致残原因。然而，BD中肺动脉受累并不局限于PAA；肺动脉受累的另一种主要类型是肺动脉血栓（PAT），伴或不伴有相关的PAA。此外，其他类型的肺部疾病，如肺实质内的结节和空洞，常与肺动脉受累相关，且可能被误诊为感染所致。我们调查了47例有肺动脉受累及相关表现的BD患者的临床、放射学和实验室特征及预后情况，所有患者均在一家专门的三级医疗中心就诊并接受随访。我们确定了47例（41例男性，6例女性）被诊断为肺动脉受累的患者，他们于2000年1月至2007年12月在切拉比帕萨医学院的多学科诊所登记。诊断时的平均年龄为29±8岁，从疾病开始到出现肺动脉受累的平均病程为3.6±4.8年。咯血是最常见的首发症状（79%），其次是咳嗽、发热、呼吸困难和胸膜炎性胸痛。47例患者中有34例（72%）出现PAA，其中8例伴有相关的PAT。其余13例患者（28%）有孤立性PAT。孤立性PAT患者的一般临床特征与PAA患者相似。然而，孤立性PAT患者咯血的频率和量较少。大多数（91%）患者在出现肺动脉受累时肺部以外有活动性疾病。初诊时40例（85%）患者有结节，6例（13%）患者有空洞。47例患者中有36例（77%）存在外周静脉血栓，36例中有12例（33%）存在心内血栓。结节、空洞和心内血栓主要出现在肺动脉受累的急性期。肺动脉受累通常是多发性的，主要累及肺动脉的降支。肺动脉受累可能会消失，但动脉狭窄或闭塞通常会在同一部位出现。平均随访7年后，47例患者中有12例（26%）死亡；动脉瘤较大的患者更易死亡。47例患者中有16例（34%）无症状，其余40%有轻度呼吸困难（13/47）和/或少量咯血发作（8/47）。肺动脉压可能升高，这可能预示预后不良。也可能观察到纵隔淋巴结肿大以及轻度胸腔和心包积液。皮质类固醇和免疫抑制剂是主要的治疗方法；然而，难治性病例可能需要栓塞、肺叶切除术、空洞切除术和剥脱术。
