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Q1: What investigation would you perform next?
Massive haemoptysis is a life threatening event since flooding of the airways and alveoli may lead to respiratory failure. It requires rapid evaluation, therefore, bronchoscopy under general anaesthesia should be performed first for the visualisation of the bronchial tree in this patient.
Q2: What possible diagnoses are compatible with the patient's clinical presentation?
Other possible diagnoses include neoplasm (primary or metastatic carcinoma of the lung), a fungus ball, hydatid cyst, granulamatous diseases (nodular sarcoidosis), connective tissue diseases (rheumatoid arthritis), and vasculitis (Wegener's granulamatosis).
Q3: What is the most likely diagnosis?
The patient's history of recurrent orogenital ulcers and erythema nodosum, and radiological features of two bilateral and rounded opacities with an average size of 3 × 5 cm, suggested Behçet's disease and lung involvement with pulmonary artery aneurysms in this case.
Q4: What investigations would you perform to confirm your diagnosis?
Demonstration of the pulmonary artery aneurysms can be made by non-invasive techniques such as dynamic computed tomography, helical computed tomography, or magnetic resonance imaging (MRI) of the thorax, as well as by invasive techniques such as pulmonary angiography.
Q5: How would you manage this patient?
Resection of the aneurysms is indicated and lifesaving in such cases with massive haemoptysis. Medical treatment for Behçet's diseases includes glucocorticoids, and a cytotoxic agent such as chlorambucil, azathioprine, or cyclophosphamide.
The patient refused to have surgery, and even though a combined regimen including prednisone (1 mg/kg/day) and cyclophosphamide (2 mg/kg/day) was given she died from massive haemoptysis.
Behçet's disease is a chronic multisystem vasculitis of unknown aetiology affecting all sizes of arteries and veins. The sine qua non is aphthous ulcers in addition to two or more of the following: genital ulceration; eye lesions such as anterior/posterior uveitis, hypopyon, or retinal vasculitis; and skin lesions such as pustules, nodules, erythema nodosum, or dermatographism. The pathergic skin test is present in 85% of patients. Lungs, kidneys, joints, central nervous system, gastrointestinal tract, cardiovascular system, the epididymis, and muscles can be involved.1
Lung involvement in Behçet's disease is rare, occurring in only 1%–5% of patients. Aneurysm formation of pulmonary arteries is one of the most significant lesions with potential erosion into the bronchial tree with consequent exsanguination. These aneurysms may be either bilateral or unilateral and tend to affect predominantly young males. Hughes-Stovin syndrome (deep venous thrombosis associated with multiple pulmonary artery aneurysms) may be a form of Behçet's disease.1
Haemoptysis, sometimes massive, is the most common clinical manifestation of pulmonary involvement, and it is a poor prognostic sign, leading to death in 30% of patients, usually within two years. Haemoptysis may be caused by the rupture of a pulmonary artery aneurysm with erosion into a bronchus (pulmonary artery bronchial fistula) or it may be the result of the development of in situ thrombosis related to the active vasculitis.3
Lung involvement in Behçet's disease is rare, occurring in only 1%–5% of patients. Pulmonary artery aneurysm is the most important type of lung involvement and is responsible for the increased risk for fatal massive haemoptysis.
Although pulmonary artery aneurysms almost always occur in male patients with Behçet's disease, it should also be considered in females, especially when they present with massive haemoptysis and have either single or multiple sharply demarcated round opacities on the chest radiograph.
Demonstration of pulmonary artery aneurysms can be done safely by dynamic computed tomography of the thorax instead of invasive procedures.
When haemoptysis occurs, it is a sign for a poor prognosis; however, urgent surgical resection may be lifesaving in cases with massive haemoptysis.
Pulmonary angiography is regarded as the gold standard for the detection of pulmonary artery aneurysms in general, but this procedure may carry some risks for patients with Behçet's disease. Venous puncture or rapid injection of a large quantity of contrast medium may initiate a thrombus or aggravate an existing one in a patient with Behçet's disease. Therefore non-invasive procedures such as helical computed tomography, dynamic computed tomography, or MRI angiography are suggested as the more appropriate techniques in such patients.4 Demonstration of the pulmonary artery aneurysms in the presented case was made by dynamic computed tomography of the thorax (fig 1).
Numerous therapeutic regimens including prednisone, cyclophosphamide, chlorambucil, and colchicine have been suggested for Behçet's disease. Although a radiographic regression in response to medical treatment has been demonstrated, the development of vascular aneurysms is considered a poor prognostic sign. In cases of massive haemoptysis related to ruptured aneurysm, urgent surgical resection may be necessary.1
This case illustrates that a pulmonary artery aneurysm can develop in association with Behçet's disease even in young females and it is a fatal complication of the disease and does not respond to any treatment. Therefore, Behçet's disease should be considered in the differential diagnosis of such cases with massive haemoptysis and chest radiography showing multiple round consolidations, especially in regions where Behçet's disease is common.
Lung involvement of Behçet's disease with multiple pulmonary artery aneurysms.