Chest
Volume 127, Issue 6, June 2005, Pages 2243-2253
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Reviews
Pulmonary Vasculitis in Behçet Disease

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Study objectives

The aims of this study were to investigate the frequency of pulmonary problems in Behçet disease (BD), and to discuss lesser-known features of pulmonary BD such as clinical characteristics, analysis of prognosis, and evaluation of treatment options with respect to the previously published cases.

Design

We conducted a comprehensive review of the literature to analyze cumulated data about pulmonary involvement in BD.

Setting

We found 159 articles regarding pulmonary disease associated with BD in May 2003.

Patients

The evaluation of these articles demonstrated 598 pulmonary problems in 585 cases.

Results

Pulmonary artery aneurysms (PAAs) are the most common pulmonary lesion in BD, and these are almost always associated with hemoptysis. Seventy-eight percent of patients with aneurysms have concomitant extrapulmonary venous thrombi or thrombophlebitis. Other pulmonary problems are reported in BD, and these are principally related to vascular lesions and radiologic abnormalities.

Conclusions

Pulmonary vascular problems, either PAA or involvement of small-sized vessels, are the main pulmonary disorders in BD. Immunopathologic findings indicate that the underlying pathogenesis is pulmonary vasculitis, which may result in thrombosis, infarction, hemorrhage, and PAA formation. Patients with small nonspecific radiologic abnormalities should be followed up closely since early diagnosis of vascular lesions may be life-saving. Immunosuppression is the main therapy for the treatment of a vasculitis. It is important that pulmonary angiitis is not mistaken for pulmonary thromboembolic disease since fatalities have occurred in BD shortly after initiation of anticoagulation/thrombolytic treatment.

Section snippets

Materials and Methods

We conducted a comprehensive review of the literature to analyze cumulated data about pulmonary involvement in BD. The PubMed database was searched for articles using the term BD combined with one of the following terms: pulmonary artery, lung, pulmonary involvement, and pleura. Pertinent articles cited as references in the identified articles also were reviewed. These articles were in English, French, Spanish, Japanese, German, Portuguese, Polish, Italian, Romanian, and Turkish; only articles

Results

Our findings are presented in two parts: PAA and pulmonary parenchymal changes/other problems. The distinction between pulmonary parenchymal changes and other problems is difficult in most of the cases. Since the specificity of pulmonary parenchymal changes/other problems is not clear and in order to avoid repetition, the data are presented in the “Discussion.”

Discussion

The first case with BD-associated pulmonary problems was published in 1959.109 Thereafter, many cases with different pulmonary diseases/problems have been reported. Many of these studies cite case reports published before 1937, the description year of BD. The frequency of pulmonary problems shows a wide variation, from < 1 to 18% (Table 5).123126135136 Pulmonary disorders in BD are one of the most common direct causes of death.145

Although many patients with BD and pulmonary problems have been

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