Pulmonary Manifestations in Behçet's Syndrome

doi:10.1378/chest.95.3.585

Chest

Volume 95, Issue 3, March 1989, Pages 585-589

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Among 72 patients with Behçet's syndrome, seven had pulmonary vascular involvement. Additional data from 42 cases in the literature are discussed. Recurrent episodes of dyspnea, cough, chest pain, and hemoptysis were the primary clinical signs, mainly in young men, appearing 3.6 years after the first manifestation of Behçet's syndrome. Fever, elevated ESR, and anemia were common, and chest x-ray films showed pulmonary infiltrates, pleural effusions, and prominent pulmonary arteries. Ventilation-perfusion scans showed perfusion defects even when chest x-ray films were normal. Pulmonary artery aneurysms were seen in 7/13 in whom angiography was done. Of 42 patients, 16 died, 15 from fatal pulmonary hemorrhage, 80 percent within two years from the development of pulmonary disease. Histopathologic study results showed vasculitis of pulmonary vessels of various sizes, leading to thrombosis, destruction of the elastic laminae, aneurysms, and arteriobronchial fistula. In addition, pulmonary emboli and the aphthous lesion of the tracheobronchial tree may aid the clinical picture. Anticoagulant therapy may be hazardous in patients with aneurysmal dilatation of the pulmonary vascular tree, and the beneficial effect of corticosteroid therapy is discussed. Pulmonary vasculitis in Behçet's syndrome is a unique clinical and pathologic picture, differing from other vasculitides affecting the lung, presents a major threat to the patient's life.

Section snippets

Patient Population

Of 72 patients who had diagnoses of Behçet's syndrome² from 1973 to 1987, seven developed pulmonary disease during the follow-up period. The patients were six males (four Arabs and two Moroccan Jews) and one Askenazi female. The first manifestation of the disease (oral ulcers) appeared at the mean age of 24.7 (range, 14 to 42) years. Pulmonary disease presented one to 13 years later (mean, seven years) at the mean age of 32 (range 22 to 47) years (Table 1). Five patients had recurrent episodes

Discussion

Pulmonary manifestations have been reported in more than 80 patients1, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35 suffering from Behçet's syndrome, most of whom (42 in addition to the seven reported here) have presented with characteristic clinical and pathologic manifestations in which the pulmonary vascular tree was affected.1, 5, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31,

References (39)

T Shimizu et al.
Behçet's disease
Semin Arthritis Rheum
(1979)
P Durieux et al.
Multiple pulmonary arterial aneurysms in Behçet's disease and Hughes-Stovin syndrome
Am J Med
(1981)
CN Gamble et al.
The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease
Am J Med
(1979)
WJ Cunliffe et al.
Treatment of Behçet's syndrome with phenformin and ethyloestrenol
Lancet
(1969)
T Chajek et al.
Behçet's disease: report of 41 cases and a review of the literature
Medicine (Baltimore)
(1975)
T Lehner et al.
Criteria for diagnosis and classification of Behçet's syndrome
A Decroix et al.
Syndrome de Behçet avec manifestation pulmonaires (a propos d'une observation)
Bull Soc Med Hop Paris
(1968)
N Dilsen et al.
Pleuropulmonary manifestation in Behçet's disease
Excerpta Medica International Congress Series
(1979)
R France et al.
Relapsing iritis with recurrent ulcers of the mouth and genitalia (Behçet's Syndrome)
Medicine (Baltimore)
(1951)
J Boe et al.
Behçet's Syndrome: report of case with complete autopsy performed
Acta Pathol Microbiol Scand
(1959)

VA Alonen et al.

Obstructive lung disease in Behçet's syndrome

Scand J Respir Dis

(1978)

T Rosenthal et al.

Systemic amyloidosis in Behçet's disease

Ann Intern Med

(1975)

C Berlin

Behçet's syndrome with involvement of the central nervous system

Arch Dermatol

(1944)

G Ohta et al.

An autopsy case of Behçet's syndrome associated with pulmonary arteritis and tuberculosis

Jpn Circ J

(1974)

TL Petty et al.

Recurrent pneumonia in Behçet's syndrome

JAMA

(1977)

J Efthimiou et al.

Pulmonary disease in Behçet's syndrome

Q J Med

(1986)

RN Gibson et al.

Pulmonary artery aneurysms in Behçet's disease

Br J Radiol

(1985)

C Mayaud et al.

Haemoptysis au cours d'une maladie de Behçet

Nouv Presse Med

(1982)

RE Slavin et al.

Pathology of the lung in Behçet's disease

Am J Surg Pathol

(1981)

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: Manuscript received February 29; revision accepted July 21

^†: Department of Pathology, Hadassah University Hospital, Jerusalem, Israel

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Chest

Clinical InvestigationsPulmonary Manifestations in Behçet's Syndrome

Section snippets

Patient Population

Discussion

Semin Arthritis Rheum

Am J Med

Am J Med

Lancet

Behçet's disease: report of 41 cases and a review of the literature

Medicine (Baltimore)

Criteria for diagnosis and classification of Behçet's syndrome

Syndrome de Behçet avec manifestation pulmonaires (a propos d'une observation)

Bull Soc Med Hop Paris

Pleuropulmonary manifestation in Behçet's disease

Excerpta Medica International Congress Series

Relapsing iritis with recurrent ulcers of the mouth and genitalia (Behçet's Syndrome)