Hughes syndrome: a common problem in Kuwait hospitals

Abstract

The objective was to study antiphospholipid antibody syndrome (APS or Hughes syndrome) in two major teaching hospitals in Kuwait. patients with suspected Hughes syndrome were investigated with tests for anticardiolipin antibodies (aCL) and lupus anticoagulants (LAC) over 1 yr. Diagnosis was considered confirmed if significant levels of either or both antibodies with no obvious cause (primary), or with systemic lupus erythematosus (SLE) or SLE-like illness (including SLE serology) (secondary) were present. Twelve (37.5%; seven females, 58%) primary and 20 (62.5%; 18 females, 90%) secondary Hughes syndrome patients were seen during this period. patients were Kuwaiti, Middle-Eastern and North-African Arabs (29). Filipinos (2) and White (1). None were from the Indian subcontinent. The main presentation was thrombosis in 75% (arterial in 25% and venous in 50%), and recurrent abortions in 50% of married women. Haematological and dermatological manifestations were limited entirely to the secondary variety, seen in 25% and 19%, respectively. Clinical manifestations were severe, leading to death in one, intensive-care management in 31% and with partial or complete warfarin resistance or brittleness in 25%. Neurological/eye and cardiac manifestations were not seen, as these patients may be attending separate speciality hospitals for these diseases in Kuwait. The approximate prevalence of this syndrome was 2.66/1000 admissions in medical wards. Projected to the total referral areas of the two hospitals, an approximate figure of 52 patients/million population/year was obtained. Hughes syndrome was a common problem among Arabs, Filipinos and possibly Whites in Kuwait. Its manifestations were severe, often requiring intensive-care management, and in one case it was fatal. Patients from the Indian subcontinent were conspicuous by their absence, despite the fact that they were well represented in all other rheumatic disease groups. Ethnic and/or geographical factors could be important in this syndrome. To the best of our knowledge, this is the first report of Hughes syndrome from the Middle East.