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AB0100 Systemic manifestation of behcet adamantiades syndrome
  1. DV Mitrovic,
  2. LJ Pavlica,
  3. BC Glisic,
  4. MR Petronijevic,
  5. G Pavlovic
  1. Clinics of Rheumatology, Military Medical Academy, Belgrade, Yugoslavia

Abstract

Background After the disease was firstly described it was beleived that this syndrome consisted of three symptoms ? orogenital ulcerations and inflammatory disease of eyes. In last several decades numerous facts indicated that we have deall systemic vasculitis, that induced multiorgan manifestations.

Objectives The aim of this study is: a) to analyse 64 patients and find the spectrum of systemic manifestations of the disease, b) to show therapeutic approach in acute phase of the disease.

Methods All patients fulfilled diagnostic criteria for the diagnosis of Behcet-Adamantiades syndrome.

Results Out of systemic manifestations the most common was the involvement of locomotoric system ? arthritis 38 (59%), arthralgiae 25 (39%), tendinitis 3 (4.7%), sacroiliitis 1 (1.6%) and afterwards the involvement of cardiovascular system ? thrombophlebitis 11 (17%), thrombosis v. cavae inferior 2 (3.1%), v. cavae superior 1 (1.6%), involvement of cardiac structures 4 (6.2%). Central nervous system was involved in ten patients: meningoencephalitis in 2 ? 3.1%, pyramidal and extrapyramidal syndrome in 1 ? 1.6%, thrombosis of venous sinuses 1 ? 1.6%. Involvement of cranial nerves was found in 3 (4.7%) patients and peripheral neuropathy of sensorial type in 4 (6.2%). Pulmonary vasculitis was confirmed in one patient ? 16 year old girl in the acute phase of the disease. Enterocolitis was developed in 5 (7.8%) and orchiepididimytis in 2 (3.1%).

In the acute phase of the disease all patients were treated with glucocorticoids and in case of multisystemic manifestations firstly with ?pulse? dose of methylprednisolone three days and afterwards cyclophosphamide in ?pulse? doses one monthly during 6 months and it was administered every 3 months until one year. A total of 19 patients were treated with cyclosporin A. Colchicin was administered in acute phase of the disease in patients with ocular involvement. Antieggregation treatment (Aspirin) was also administered.

Conclusion This paper confirms that Behcet-Adamantiades syndrome is not only three-symptom disease but also systemic generalised vasculitis. Multisystemic manifestations developed due to systemic vasculitis.

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