Lethal aneurysm formation of pulmonary arteries in a woman with Behçet's disease

A. Filiz and Ö. Dikensoy1

Department of Chest Diseases, School of Medicine, University of Gaziantep, Gaziantep, Turkey

Sir, Behçet's disease is a chronic multi-system vasculitis of unknown aetiology. Pulmonary system involvement in Behçet's disease is rare, occurring in only 1–5% of patients. Aneurysm formation of pulmonary arteries is even less frequent, but when it occurs it is life threatening due to the increased risk of aneurysm rupture which often results in massive haemoptysis. Once haemoptysis occurs approximately 50% of patients die within 2 yr despite any therapeutic approach [1].

Our case concerns a 23-yr-old woman who was admitted to the hospital with massive haemoptysis. The admission chest X-ray showed bilateral, rounded opacities varying in size between 2 and 5 cm, four on the right and one on the left. This was her first episode of haemoptysis and occurred 1 month after the uneventful normal delivery of her third child. She had suffered recurring oral and genital ulcers for several years until 1 yr prior to admission.

Dynamic computed tomography (CT) scan of the chest revealed the simultaneous contrasting of those opacities with the pulmonary arterial system (Fig. 1Go). On the basis of her history of recurrent oral and genital ulcers and dynamic CT scan findings, the diagnosis of Behçet's disease presenting with pulmonary artery aneurysms was made. This diagnosis was supported by a positive pathergic skin test. She died from massive haemoptysis 18 months later, despite therapy, including prednisone and cyclophosphamide.



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FIG. 1. (A) Chest radiograph revealing multiple, bilateral opacities. (B) Dynamic CT scan revealing simultaneous contrasting opacities with pulmonary arteries.

 
The presentation of Behçet's disease with pulmonary artery aneurysms is very rare in females [1, 2]. To our knowledge, there are no more than 10 cases reported in the medical literature, whereas many more cases have been reported in males. The reason for this remarkable rarity in cases of Behçet's disease with pulmonary artery aneurysms is not known. Our case was a young female who had her first episode of haemoptysis 1 month after the normal delivery of her third child, suggesting a possible relationship between pregnancy and the formation of pulmonary artery aneurysms in Behçet's disease. There are few reports to indicate the influence of pregnancy on Behçet's disease or vice versa and they present conflicting results [3, 4]. Marsal et al. [3] investigated the possible effects of pregnancy and labour on Behçet's disease in a large study group. They suggested that there was no significant relationship between the course of Behçet's disease and pregnancy. Nevertheless, we know that pregnancy can increase the tendency for pulmonary thromboembolism and that pregnancy and labour both increase the pulmonary blood pressure. Therefore, we suggest that pregnancy and labour associated with vaginal delivery may have a possible effect on the development of pulmonary artery aneurysms and/or their rupture, especially in multigravida women with Behçet's disease.

Notes

1 Correspondence to: Ö. Dikensoy. Back

References

  1. DeRemee RA. Behçet's disease: Pulmonary vasculitis. In: Fishman AP, ed. Fishman's pulmonary disease and disorders, Vol 1. McGraw-Hill, 1998:1370–1.
  2. Celenk C, Celenk P, Akan H, Basoglu A. Pulmonary artery aneurysms due to Behçet's disease: MR imaging and digital subtraction angiography findings. Am J Roentgenol, 1999;172:844–5.[ISI][Medline]
  3. Marsal S, Falga C, Simeon CP, Vilardell M, Bolsch JA. Behçet's disease and pregnancy relationship study. Br J Rheumatol 1997; 36:234–8.[ISI][Medline]
  4. Bang D, Chun YS, Haam IB, Lee ES, Lee S. The influence of pregnancy on Behçet's disease. Yonsei Med J 1997;38:437–43.[Medline]
Accepted 8 September 1999





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