Occurrence of pulmonary artery aneurysms and pulmonary artery thrombosis in a young man.
Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown aetiology. Arterial involvement is uncommon but associated with important morbidity and mortality. We describe the clinical course of BD with severe pulmonary artery involvement in a 19-year-old man. He presented with massive haemoptysis related to pulmonary artery aneurysms. Initial treatment consisted in urgent right inferior lobectomy, corticosteroids and monthly intravenous cyclophosphamide. Subsequently, he developed pulmonary artery thrombosis at non-aneurysmatic sites. Corticosteroid therapy was intensified, monthly intravenous cyclophosphamide was continued and an anticoagulant was added to the treatment with a favourable clinical and radiological response. Our case illustrates that timely initiation of highly potent immunosuppressive therapy is critical to obtain a favourable outcome. At present, a consensus regarding optimal management of vascular BD is lacking. ALthough anticoagulation is not generally recommended, our report encourages a patient-based decision after carefully tailoring potential risks and benefits.
Department Interne Geneeskunde, UZ Brussel, Vrije Universiteit Brussel, Brussel, België. iramon06@hotmail.com
I
I
Ramon
K
K
De Smet
S
S
Allard
B
B
Ilsen
G
G
Verfaillie
B
B
Velkeniers
D000783:Aneurysm / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008297:Male; D011651:Pulmonary Artery; D013927:Thrombosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D055815:Young Adult