Angiotensin-converting enzyme gene polymorphism in Kuwaiti patients with systemic lupus erythematosus.

OBJECTIVE: To investigate the frequency of angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism genotypes in patients with systemic lupus erythematosus (SLE), and to study the correlation between I/D polymorphism of the ACE gene and the clinical manifestations of SLE, especially vascular involvement, lupus nephritis and disease severity. METHODS: The frequency of ACE gene I/D polymorphism genotypes was determined in 92 patients with SLE from Kuwait, and compared to that in 100 ethnically matched healthy controls using the polymerase chain reaction. RESULTS: The distribution of ACE I/D polymorphism and allele frequencies in SLE patients was not significantly different from controls. Further analyses of SLE patients showed that there was a significant association between DD genotype and Raynaud's phenomenon (p=0.008, odd ratio=5.4, 95% confidence interval: 1.6-18.6). However, there was no significant association between the ACE genotype and lupus nephritis or disease severity. CONCLUSION: No difference was found between the distribution of the ACE genotype in SLE patients and the general pop-ulation in Kuwait. However, the presence of the DD genotype may confer susceptibility to the development of vascular morbidity.

Henoch-Schönlein's purpura in adults versus children/adolescents: A comparative study.

OBJECTIVE: To assess the possible differences in etiological and clinical factors between children/adolescents (< or = 20 years) and adults (> 20 years) with Henoch-Schonlein purpura (HSP). METHODS: A retrospective-cum-prospective study of consecutive patients with HSP who presented to our teaching hospital over 5 years. Patients were classified as having HSP according to the criteria proposed by Michel et al and the ACR criteria. RESULTS: 102 patients (43 of all patients being male and 59 female) were classified as having HSP; 20 of the patients were adults (mean age 32.1 +/- 11.7 years) and 82 were children/adolescents (mean age 6.2 +/- 2.6 years). We were unable to identify any precipitating event in 40% of the adults and 37% of the children/adolescents. The frequency of previous drug treatment and of previous upper respiratory tract infection was similar in both groups. At symptom onset, palpable purpura was the chief clinical manifestation in both groups. However, renal involvement, in all its aspects, was more frequent and severe in adults. Adults also had a higher frequency of diarrhoea (with or without occult blood) and leucocytosis, but a lower frequency of thrombocytosis. The frequency of joint manifestations, nausea, vomiting, malena/hematochezia and intussuseption was equal in both groups. Adults required more aggressive therapy, and had a longer hospital stay (10.2 vs. 4.3 days). The outcome was relatively worse in adults, with complete recovery in 18 adults (90%) compared to 81 children/adolescents (98.8%) after a mean +/- SD follow up of 2.8 +/- 1.7 and 2.4 +/- 1.3 years, respectively. CONCLUSION: In adulthood, HSP is a more severe clinical syndrome, with a higher frequency of diarrhoea and renal involvement. Adults also require aggressive treatment more frequently and have a longer hospital stay.

Clinicoepidemiological features and course of 43 cases of bullous pemphigoid in Kuwait.

BACKGROUND: The clinicoepidemiological characteristics and course of bullous pemphigoid (BP) have not been described in populations from the Arabian Gulf. Hypothesis. Ethnic and regional variations can influence the clinical behaviour and course of autoimmune diseases. METHODS: In this study, 43 patients with BP, registered in our autoimmune bullous diseases (ABD) clinic over a span of 14 years, were studied to determine the clinicoepidemiological features and course of the disease in our region. RESULTS: BP was observed to be the second commonest ABD in our clinic (27%), with a minimum estimated incidence of 2.6 cases/million/year among the referral population. The largest proportion (93%) of the patients was of Arab ethnicity and the female to male ratio was 5.1 :1. Mean +/- SD age at diagnosis was 65.20 +/- 18.80 years. Most of the patients (96%) had moderate to severe disease, and mucosal involvement was seen in 37% of the patients. Systemic steroids (prednisolone 20-60 mg daily) alone or in combination with azathioprine, intravenous immunoglobulins, tetracyclines, mycophenolate mofetil or dapsone were used to treat theses cases. At the last follow-up, 32% of patients were in complete remission and off treatment. The first-year mortality was 30%. Old age and poor general medical condition were the significant risk factors (P < 0.05) contributing to the mortality. CONCLUSION: The study highlights the regional variations of BP and thus a need to uncover the ethnic, genetic and geographical influences, if any, responsible for these variations.

Serological characteristics of systemic lupus erythematosus from a hospital-based rheumatology clinic in Kuwait.

Thirty-one consecutive patients with SLE were screened for antinuclear antibody (ANA), anti-DNA antibodies, extractable nuclear antigen antibodies (anti-ENAs) including anti-Sm, anti-RNP, anti-SSA (anti-Ro), anti-SSB; (anti-La), anti-Scl-70, rheumatoid factor (RF), C-reactive protein (CRP), C3 and C4 levels, anti-cardiolipin antibodies (aCL), biologically false positive serological test for syphilis (BF-STS) using VDRL test and Coombs' test. The age of the patients ranged from 11 to 52 year with a median of 29 year; female to male ratio of 5:1. There were 21 Kuwaitis, four Egyptians, three from the Indian subcontinent, two Filipinos and one Syrian. Main clinical categories of SLE were: mild cutaneous SLE in 12 (38.7%), clinical antiphospholipid syndrome (APS) secondary to SLE in 8 (25.8%), haematological manifestations of SLE in 5 (16.1%), renal lupus in four (12.9%), neuropsychiatric in three (9.7%), others (6.4%). Clinical features overlapped in several patients. ANA was positive in 96.8% (mean value 891.61 units/ml), anti-DNA in 35.5% (mean value 56.4 units) that was lower than expected and could be due to selection bias as the patients were from a rheumatology clinic, anti-ENA in 42%, anti-Sm 13% that was lower than other non-Caucasian populations, anti-RNP 13%, anti-SS-A in 35.5%, anti-SS-B in 19.4%, Scl-70 in 13%, CRP in 71% (moderate 58%, very high 13%); C3 mean 1.52 mg/ml (3.2% low levels), C4 mean 0.35 mg/ml (32% low levels), anticardiolipin mean GPL 35.35 units (high 58%), mean MPL 10.61 units (high 26%), BF-STS in 6%, Coombs' test in 6%, RF positive in 36%. The only significant positive clinical associations observed were those of renal involvement with anti-DNA antibodies (P = 0.042), and clinical antiphospholipid antibody syndrome with aCL antibodies (P = < 0.05).