Abstract

Behcet’s disease (BD) is a multisystem vasculitis and pulmonary involvement in BD is reported to indicate a poor prognosis and high mortality. We aimed to study the manifestations of pulmonary involvement in a group of patients with BD and to study the correlation of pulmonary involvement with other clinical manifestations. Our study included 15 patients with BD admitted to Cairo University Hospital, 14 men (93.3%) and one woman (6.7%), mean age 30.06 ± 9.8 years. All patients fulfilled the diagnostic criteria published by the International Study Group for Behcet’s Disease in 1990. All patients were subjected to both plain chest radiography and a helical computed tomography study of the chest. Pulmonary manifestations were present in 11 patients, 10 men (90.9%) and one woman (9.1%). The main pulmonary and constitutional symptoms were as follows: dyspnea (81.8%), cough (63.6%), expectoration (36.4%), chest pain (54.5%), hemoptysis (36.4%), massive hemoptysis (9.1%), fever (36.4%), and weight loss (63.6%). In the 11 patients with pulmonary manifestations, analyses of both vascular and parenchymal lung lesions in helical computed tomography scan indicated the following: pulmonary artery aneurysm occurred in five patients (45.4%), pulmonary nodules in three patients (27.2%), pleural effusion in three patients (27.2%), pulmonary infarction in one patient (9.1%), and pneumonitis in one patient (9.1%). Pulmonary involvement was associated significantly with a positive pathergy test, erythema nodosum, and gastrointestinal manifestations. A high frequency of pulmonary artery aneurysm was observed in our patients with pulmonary BD. Patients with pulmonary BD have higher frequencies of skin and gastrointestinal manifestations.

Highlights

  • Behçet’s disease (BD) is a multisystem and chronic inflammatory disease with unknown cause

  • The actual prevalence of the pulmonary manifestations in BD is unknown as no prospective study has evaluated all pulmonary symptoms in an unselected group of patients

  • Oral ulcers and genital ulcers occurred in 100% of cases, eye lesion in 40%, erythema nodosum in 13.3%, other skin lesions in the form of papulopustular and acneiform lesions in 20%, a positive pathergy test in 26.6%, musculoskeletal symptoms in 60%, neurological manifestations in 20%, gastrointestinal manifestations in 13.3%, vascular involvement in 80%, and pulmonary involvement in 73.3% of cases

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Summary

Introduction

Behçet’s disease (BD) is a multisystem and chronic inflammatory disease with unknown cause. The disease was first described by the Turkish dermatologist Hulusi Behçet in 1937 and consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. The diagnosis is made on the basis of the criteria proposed by the International Study Group for Behçet’s Disease in 1990 [2]. (a) Recurrent genital ulcerations, (b) Eye lesions, including uveitis, and retinal vasculitis,. The actual prevalence of the pulmonary manifestations in BD is unknown as no prospective study has evaluated all pulmonary symptoms in an unselected group of patients. Behçet’s disease (BD) is a multisystem vasculitis and pulmonary involvement in BD is reported to indicate a poor prognosis and high mortality. All patients fulfilled the diagnostic criteria published by the International Study Group for Behçet’s Disease in 1990. All patients were subjected to both plain chest radiography and a helical computed tomography study of the chest

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