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Gastroenterology Department, Serviço de Gastrenterologia, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
Internal Medicine Department, Serviço de Medicina Interna, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
Intensive Care Medicine Department, Serviço de Medicina Intensiva, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
A 20-year-old male was admitted to the emergency department with a 1-week history of fever, right pleuritic chest pain and aggravating sporadic hemoptoic cough.
The patient mentioned oral aphthous ulcers more than 3 times per year and recurrent genital aphthous ulcers since puberty. He had a past medical history of multiple episodes of conjunctivitis, acne and skin lesions compatible with erythema nodosum. He denied pathergy reaction.
Physical examination was unremarkable.
Laboratory tests revealed anaemia [Haemoglobin: 10.8 g/dL (13.5-18.0 g/dL)], leukocytosis [Leukocytes: 20.8×103/µL (4.0-10.0×103/µL)] with neutrophilia [14.8×103/µL (2.0-8.0×103/µL)] and elevation of C-reactive protein [176.7 mg/L (0-5.0 mg/L)].
Chest X-Ray (CXR) (Fig. 1A) showed bilateral well-defined rounded hilar opacities of large dimensions. A chest Computed Tomography (CT) (Fig. 1B) was performed. What is the diagnosis?
Fig. 1Panel A. CXR showing rounded bilateral hilar opacities. Panel B. Chest CT showing multiple pulmonary artery aneurysms.
The chest CT revealed multiple pulmonary artery aneurysms (PAAs), the largest located in the right middle lobe and superior portion of the left inferior lobe, both with a diameter of approximately 6 centimetres and variable degrees of mural thrombosis (Fig. 1B). Clinical history, CXR and CT lead to the diagnosis of multiple PAAs secondary to Behçet's syndrome.
Behçet's syndrome is a multisystemic condition of unknown etiology, characterized by recurrent oral and genital ulcers and ocular involvement [
Pulmonary involvement has a reported prevalence of 1-7.7%. PAAs are the most common finding and are mainly located in the right lower lobar artery, even though they can be located anywhere in the pulmonary arterial tree [
PAAs usually manifest with hemoptysis, which can be massive and life threatening. Fever, pleuritic chest pain, dyspnea and/or cough, may also be present. Scarcely, they can also be asymptomatic [
The presence of PAAs in the main or lobar branches of a young man is almost unique to Behçet's syndrome.
According to EULAR recommendations, the medical management of PAAs consists of high dose glucocorticoids and cyclophosphamide. Surgical indications are not well established and surgery should not be undertaken except in life-threatening situations [
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