DR.KAVYASHRI G M (DNB), DR.AMRITA RAO, CONSULTANT

A CASE REPORT - BEHCET’S SYNDROME IN PREGNANCY
DR.KAVYASHRI G M (DNB),
DR.AMRITA RAO, CONSULTANT, DEPT OF OBG , MANIPAL HOSPITAL, BANGALORE
INTRODUCTION
BEHCET’S SYNDROME
Behcet’s disease is a chronic relapsing
multisystemic disorder characterised by mucocutaneous,
ocular, vascular & CNS manifestations.
Incidence: More common & most severe along ancient silk road.
Most common in Turkey ( 80 -370 cases / 1 lakh ),
17.5 cases per 1 lakh in Asian ancestry.
Most clinical manifestations are believed to be due to
vasculitis. Involve blood vessels of all sizes on both the arterial &
venous sides of circulation.
It typically affects young adults 20 to 40 years of age.
Etiology & Pathogenesis
Underlying cause of Behcet’s disease is UNKNOWN. As with other
autoimmune diseases, the disorder may represent aberrant immune activity
triggered by exposure to an agent, perhaps infectious, in patients with a
genetic predisposition to develop the disease.
• Genetic predisposition
• Bacterial antigens with cross reactivity to human peptides
• Altered populations of haematopoitic cells & associated cytokines
• Presence of immune complexes & autoantibodies
• Vascular endothelial activation & hypercoagulability
PATHOLOGY
Classic Behcet’s lesion is necrotizing leucocytoclastic obliterative
perivasculitis & venous thrombosis with lymphocytic inﬁltration of capillaries ,
veins, & arteries of all sizes.
Panniculitis in erythema nodosum like lesions & neutrophilic &
mononuclear inﬁltrates in spontaneous acute lesions & following testing for
Pathergy (skin prick test).
CASE REPORT – BEHCET’S SYNDROME IN PREGNANCY
A 31 year old primigravida, IVF conception (Male factor
infertility), DCDA twin gestation with vanishing twin. She
presented at 15weeks of gestation with painful skin lesions all
over the body, mainly over face, upper and lower limb joints and
multiple oral ulcers and ulcers in the vagina, associated with
malaise, body ache & joint pains. There were no complaints of
neurological or eye problems.
O/E- Multiple erythematous lesions over face, thighs,
legs, knee joints & aphthous ulcers on buccal surface & in
vagina. No secondary infection and lymphadenopathy noted.
Investigations done( LFT, RFT, Absolute eosinophil count ,
ANA, APLA, Skin biopsy from nodular lesion). All parameters were
normal except for skin biopsy, which was suggestive of Erythema
nodosum with features of vasculitis. Collabarative diagnosis of
Behcet’s syndrome was reached on the basis of clinical features and
biopsy report.
During the course of pregnancy, she had 3 episodes of antepartum
haemorrhage, Scan showed retroplacental clot, which persisted till 32
weeks, which was managed conservatively. She developed DM secondary
to steroids, started on Insulin. Labour was induced with PGE2 at 37weeks
in view of decreased fetal movements. In view of non progress of labour,
LSCS done and she delivered a healthy male baby weighing 2.68kgs.
Treatment: Started on Prednisolone 20mg/day and slowly
tapered. Skin lesions and ulcers subsided after starting steroids. She
continued to receive maintenance dose throughout pregnancy
Post partum period was uneventful. She continued to receive
prednisolone in tapering dose and started on colchicine after 6weeks,
following which breast feeding was discontinued.
CLINICAL FEATURES International study group criteria (1990)
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Oral ulcers ( Most common)
Urogenital ulcers (75%)
Cutaneous lesions ( 75%)
Ocular disease (25-75%)
Neurological disease (<1/5th)
Vascular disease
Arthritis (50%)
GI ulcerations
Cardiac disease
Renal disease
Criteria
Required features
Recurrent oral
ulceration
Observed by physician or patient, atleast 3
episodes in a year
Plus any 2 of
following
Recurrent genital
ulceration
Ulceration or scarring observed by patient or
physician
Eye lesions
Anterior/ posterior uveitis in vitreous or retinal
vasculitis( ophtholmologist)
Skin lesions
Erythema nodosum like lesions (patient),
papulopustular skin lesions or pseudofollicultis
with acneiform nodules(physician)
Pathergy test
Interpreted at 24-48 hours by physician
PROGNOSIS
• Waxing & waning course
• More severe in young, male
• Greatest morbidity & mortality comes
from neurologic, ocular & large vessel
arterial or venous disease.
• Ocular & neurological lesions may
improve with immunosuppressive
therapy but are often not fully reversible.
• Pregnancy complications
REFERENCES
MANAGEMENT
Glucocorticoids are the mainstay of
Behcet’s disease treatment..
High dose – acute life/organ
threatening
disease.
Low dose – less acute or severe
disease.
Other immunosuppressing agents:
Colchicine, Azathioprine,
Cyclophosphamide, Cyclosporine, Anti
TNF alpha drugs (Iniximab, Etanercept),
Interferon alfa, Mycophenolate mofetil
• PREGNANCY : Disease activity is some what reduced,
• Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behcet’s disease in the US: a population however risk of complications is higher.
based study. Arthritis Rheum 2009; 61:600
Pregnancy complication rate - 16%
• Saenz A, Ausejo M, Shea B, et al. Pharmacotherapy for Behcet’s syndrome.Cochrane database Syst Rev 2000; :CD001084
Miscarriages, caesarean section, medical termination of
• Hatemi G, Silman, Bang D,et al.Management of Behcet’s disease; a systematic literature review for the European League
pregnancy, HELLP syndrome & immune thrombocytopenia
Against Rheumatism evidence-based recommandations for the management of Behcet’s disease. Ann Rheum Dis 2009;
• Others : Fever, malaise, ﬁbromyalgia, urinary & erectile function.
68:1528
• Sakane T, Takeno M, Suzuki N, et al. Behcet’s disease. N Engl J Med 1999; 341: 1284

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