46 s 6e conference internationale sur la maladie de Behfet
C 45
Pulmonary arterial aneurysms in Beh~et's disease (12 case reports)
N. Trombati, A, Bahlaoui, M. Bartal, Z. Bouayad, A. El Meziane, A. Alaoui-Yazidi, A. Aichane
Twelve cases of Beh~et' s disease (BD) with multiple pul- monary aneurysms registered between January 1986 and De- cember 1992 were reported. The patients were all males aged between 17 and 41, the average age being 27. Hemoptysis was the prevailing clinical sign in 11 cases. Bipolar aphthosis was found in 11 cases and genital aphthosis solely in one patient. Sequels of sural phlebitis were reported in 8 cases.
In all cases, chest r a d i o g r a p h y revealed round and well limited pulmonary opacities. These latter were very often perihilar and were bilateral in 9 out of the 12 cases. Com- puted tomography with vascular opacification confirmed their aneurysmal origin. In most cases (11 out of the 12) the treatment included a corticosteroid therapy given in bolus in 3 cases and associated to an anticoagulant in 6 cases. During the course of the disease, lethal hemoptysis appeared in 3
patients. In one case, cataclysmic and life threatening ones required in emergency, surgical operation which ended in a right pneumonectomy. In one patient, where the aneurysm had clearly diminished, no surgical operation was advocated.
In another case, a left inferior lobectomy was carried out.
Whereas in the remaining cases and given the multiplicity and bilaterality of the aneurysms, surgical care was deemed impossible. On the basis of these 12 cases, the authors under- lined: 1) the relative prevalence of this form of BD; 2) its gravity, as it is one of the rare conditions which threatens life because of the risk of unpredictible massive hemoptysis; 3) the diagnosis problems it may ~aise; 4) and the therapeutic problems.
Department of Respiratory Diseases, CHU Ibn Rochd. Casablanca, Morocco
C 46
Prognosis of arterial
Le Thi. Huong Du, B. Wechsler, JC. Piette, T. Papo,
Among 300 patients with Beh~et's disease (BD), 23 had arterial involvement: 21 men and 2 women, with mean age of 31 + 8 years at diagnosis. The arterial lesions were isolated stenosis or thrombosis in 7 patients, aneurysms in 16 isolated or associated with stenosis.
Arterial stenosis involved: tibial arteries (n = 2), plantar arteries (n = 2), left anterior descending (n = 1), sub clavians (n = 1), and femoral artery (n = 1). One patient had ilio- femoral bypass which thrombosed one year later and needed reoperation. The arterial symptomatology was improved in all the patients with vasodilatators associated with antiaggre- g a n t s (n = 5) or a n t i c o a g u l a n t s (n = 2), corticosteroids (n = 3). One p a t i e n t was finally c o n s i d e r e d as having Crohn's disease. One patient died from septic shock due to Pseudomonas.
Arterial aneurysms involved: pulmonary arteries (n = 5), left anterior descending artery (n = 1 ), aorta (n = 4), femoral artery (n = 6). Four patients were previously operated. All relapsed without medical therapy. Among 5 patients treated post-operatively with corticosteroids alone: 1 was lost to fol- low-up, 2 died: the first patient suddenly 6 years after
a o r t o -lesions in Behget's disease
O. Bl~try, E. Kieffer, JM. Vitoux, C. Chapelon, P. Godeau
iliac bypass, the second patient from hemoptysis 1 year after embolization of pulmonary aneurysms. The 11 other patients were treated with corticosteroids and immunosuppressives (cyclophosphamide n = 5, azathioprine n = 7, chlorambucil n = 1). Aneurysms relapsed in only 1 patient despite cyclo- phosphamide. Three patients died: the first patient who had post-traumatic femoral aneurysm died after 3 operated aortic valvular prosthesis dehiscences from cardiac failure despite cyclophosphamide and azathioprine; the second patient died from hemoptysis due to pulmonary aneurysms despite cyclo- phosphamide, the third patient treated for coronary aneu- rysms died suddendly 2 years later while taking azathioprine.
In conclusion, if prognosis of arterial stenosis in BD is good, aneurysms may be lethal (5 deaths out of 16 patients) and anti-inflammatory treatment and surgery if possible are needed. However, this treatment is sometimes insufficient to prevent relapse. Immunosuppressive therapy with corticos- teroids seems to be more efficient than corticosteroids alone but their modalities of administration need to be improved.
Service Medecine Interne, CHU Piti6-Salp#triOre, 75013 Paris, France
La Revue de M#decine Interne
