HUGHES SYNDROME: A COMMON PROBLEM IN KUWAIT HOSPITALS

* Mubarak Al-Kabeer Hospital, ^Rheumatic Diseases Unit, Amiri Hospital, Ministry of Health and \Faculty ofMedicine, Kuwait University, KuwaitSUMMARYThe objective was to study antiphospholipid antibody syndrome (APS or Hughes syndrome) in two major teaching hospitalsin Kuwait. Patients with suspected Hughes syndrome were investigated with tests for anticardiolipin antibodies (aCL) and lupusanticoagulants (LAC) over 1 yr. Diagnosis was considered confirmed if significant levels of either or both antibodies with noobvious cause (primary), or with systemic lupus erythematosus (SLE) or SLE-lilce illness (including SLE serology) (secondary)were present. Twelve (37.5%; seven females, 58%) primary and 20 (62.5%; 18 females, 90%) secondary Hughes syndromepatients were seen during this period. Patients were Kuwaiti, Middle-Eastern and North-African Arabs (29), Filipinos (2) andWhite (1). None were from the Indian subcontinent. The main presentation was thrombosis in 75% (arterial in 25% and venousin 50%), and recurrent abortions in 50% of married women. Haematological and dermatological manifestations were limitedentirely to the secondary variety, seen in 25% and 19%, respectively. Clinical manifestations were severe, leading to death inone, intensive-care management in 31% and with partial or complete warfarin resistance or brittleness in 25%. Neurological/eyeand cardiac manifestations were not seen, as these patients may be attending separate speciality hospitals for these diseases inKuwait. The approximate prevalence of this syndrome was 2.66/1000 admissions in medical wards. Projected to the total referralareas of the two hospitals, an approximate figure of 52 patients/million population/year was obtained. Hughes syndrome wasa common problem among Arabs, Filipinos and possibly Whites in Kuwait. Its manifestations were severe, often requiringintensive-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 couldbe important in this syndrome. To the best of our knowledge, this is the first report of Hughes syndrome from the Middle East.KEY WORDS: Hughes syndrome, Middle East, Kuwait, Anti phospholipid syndrome.MORE