Behçet's syndrome in pregnancy

Abstract

Summary: Behç et's syndrome (BS), a systemic inflammatory disease characterized by oral and genital ulceration, eye inflammation and arthritis, usually presents in the third and fourth decades of life, but is rare in pregnancy. BS is not usually associated with a detrimental effect on pregnancy outcome. In most women BS is reported to improve in pregnancy, although it may not always follow a similar course in successive pregnancies and it is not possible to predict the course of BS in a particular pregnancy. Many of the drug therapies used to treat BS are safe to use in pregnancy and in the breastfeeding mother. These include corticosteroids, azathioprine, calcineurin inhibitors and probably colchicine. Experience with use of biologics in pregnancy is increasing. Drugs used in the management of BS that should be avoided in women planning a pregnancy include methotrexate, mycophenolate mofetil, thalidomide, cyclophosphamide and chlorambucil.

Pregnancy Outcomes in Systemic Vasculitides

“…Vascular complications were also more common in a study of 63 pregnancies with BD [ 56 ]. Rare vascular complications in pregnancies with BD are reported, such as cerebral venous thrombosis, superior vena cava thrombosis, intracardiac thrombosis, Budd-Chiari syndrome, ovarian vein thrombosis, deep vein thrombosis, and pulmonary embolism [ 52 ••, 56 , 58 , 83 ]. Given this heightened risk, patients should be closely monitored for thromboembolic events, especially in the postpartum period.…”

“…A small number of case reports describe transient neonatal BD mostly consisting of oral or genital ulcerations and skin findings that resolve up to 8 weeks after birth [ 83 , 88 , 89 ]. One neonatal death occurred from respiratory distress and neurological involvement following delivery at 34 weeks of gestation and another neonate, born at 38 weeks, suffered life-threatening complications a few days postpartum treated with glucocorticoids [ 90 , 91 ].…”

“…Most cases were reported in mothers who suffered from BD before pregnancy and had orogenital ulcerations during pregnancy [ 89 ]. It has been hypothesized that the disease could be mediated by a transplacental transfer of maternal antibodies [ 83 ]. Necrotizing villitis and decidual vasculitis have been identified in two placentas of patients affected with BD, one pregnancy occurred with good maternal and fetal outcomes and the other one resulted in a therapeutic abortion because of colchicine exposure [ 92 ].…”

Behçet’s Disease: An Enigmatic Malady with Plethoric Expressions

“…Gastrointestinal involvement was observed in 3-26% of patients and varies among different populations [38,39]. It is much more frequent in Japan than in the Middle East and the Mediterranean region [1][2][3]. Mucosal inflammation and ulceration can occur anywhere in the gastrointestinal tract but typically in the ileocecal region.…”

“…This exemplary presentation would have been probably first identified by Hippocrates [1,2]. Even though Behçet's disease was first identified by the father of medicine in the fifth century BC, there were no literature representations for this entity till 1930, and then a Greek ophthalmologist Benediktos Adamantiades presented one of his case of relapsing eye lesions associated with genital ulceration and arthritis in a 20-year-old male; since then entity has been added with a synonym "Adamantiades-Behçet's disease" [3][4][5][6]. Further to its classic expression of orogenital ulceration and ocular lesions, the conditions seem to involve the musculoskeletal system, nervous system, gastrointestinal tract, vascular beds, urogenital tract and cardiopulmonary system, so Behçet's disease have a high morbidity and mortality particularly in males with early age onset [7,8].…”