Takayasu's arteritis in Kuwait.

The present study describes the epidemiological profile and clinical features of Takayasu's arteritis (TA) in Kuwait, as well as its association with other autoimmune diseases and primary hypercoagulable states. Thirteen patients were included from its start on 1 January 1989 till 30 June 1994. Diagnosis of TA was established by angiographic studies. Twelve patients were Arabs and 7 were Kuwaiti nationals. Five patients were males and renal disease secondary to isolated involvement of the abdominal aorta (TA, type II) was the main presentation in 4 patients. Coagulation tests were performed in 7 patients and included antiphospholipid antibody (aPL) assay as well as protein S, protein C and antithrombin III activity. Only one manifested recurrent thrombosis and laboratory tests confirmed the presence of a hypercoagulable state secondary to aPL and protein S deficiency. Serological tests of systemic lupus erythematosis (SLE) were positive in this patient. These data indicate that TA is not a rare disease in the Arabic population. In our study, female predominance was not a common feature of TA and renal disease secondary to TA type II disease was commonly encountered. The association of TA with SLE and primary hypercoagulable states was not a consistent finding in our patients with TA, and hence, the proposed role for thrombotic vasculopathy in the pathogenesis and progression of this disease was unfounded.

Progressive ischemic gangrene in dialysis patients: a clinicopathological correlation.

The syndrome of progressive ischemic gangrene (PIG) of the extremities was examined over 3.5 years in patients undergoing maintenance dialysis (MD) in Kuwait and was compared to that in a similar age group (> 40 years) in the general population. The incidence of PIG in MD patients was 15.4/1000 person years of observation (PYO) versus 0.086/1000 PYO in the general population. Patients with diabetes mellitus were found to be at particular risk. PIG developed in 41.4/1000 PYO of diabetic patients who received MD, compared to 7.1/1000 in nondiabetic patients on MD and 0.14/1000 in diabetics without renal disease. The clinical, biochemical, radiological, and histological findings in the 8 patients who developed PIG while on maintenance dialysis (MD) are presented. Two patients had severe hyperparathyroidism and their histological findings were consistent with systemic calciphylaxis. Histological examination, in the remaining patients, showed severe calcified atherosclerosis. Intimal hypertrophy was common especially in patients with long duration on dialysis. The three lesions produced a variable degree of luminal narrowing and were associated with arterial thrombosis. None of the patients showed evidence of iron deposition even in those with systemic calciphylaxis and excessive iron stores. Our study indicated a high incidence of PIG in patients undergoing MD, especially in those with diabetes mellitus. These findings constitute a cogent argument in favor of early parathyroidectomy in selected cases and concern with long-term consequences of atherosclerosis in this patient population.