Behçet's disease in Saudi Arabia.

OBJECTIVE: To study the presentation of Behçet's disease (BD) in the Saudi population and to determine any differences in clinical manifestation and prognosis between men and women. METHODS: One hundred nineteen patients with BD attending the King Faisal Specialist Hospital and Research Centre (KFSH & RC) and the King Khalid University Hospital (KKUH) between 1979 and 1992 were studied. RESULTS: The male to female ratio was 3.4:1. The mean age was 29.3; oral ulcer was present in all the patients (100%), genital ulcer 87%, ocular involvement 65%, skin manifestations 57%, and arthritis 37%. Central nervous system involvement was present in 52 patients (44%) of which 12 had benign intracranial hypertension (BICH), pleuropulmonary manifestation 16%, deep venous thrombosis 25%, arterial thrombosis and aneurysm 18%, gastrointestinal manifestations 4%, and epididymitis 4%. Significant proteinuria was present in 9 patients 7.5%, of whom 3 had renal biopsy. The pathergy test was positive in 15 of 85 (17.5%) patients. HLA-typing was performed for 85 patients and HLA B5(51) was positive in 61 (72%) patients compared with 26% general population. CONCLUSION: Using the Fisher exact test, there was no significant difference in clinical manifestation or prognosis comparing men to women.

Tuberculosis of peripheral joints: a dilemma in diagnosis.

15 cases of peripheral joint tuberculosis presenting to a tertiary referral hospital are reviewed. The presentation is characterized by an insidious onset with a slowly progressive, painful, ankylosed or swollen monarthropathy with a mean length of history of 6.5 +/- 7 years. The knee joint was involved in 7 patients, hip in 6, elbow and shoulder 1 each. Significant systemic toxicity was absent, the white count was normal (mean 7.3 +/- 2.4 x 10(9)/1) and the erythrocyte sedimentation rate (ESR) was only slightly elevated (mean 31 +/- 23 mm/first h). Features of extra-articular pulmonary tuberculosis were present in 7 patients and periarticular findings were present (abscesses in 7 and sinuses in 4 patients). Despite a characteristic presentation, the diagnosis was initially missed in 10, leading to delay in instituting correct treatment. One patient presented with osteoarthritis and a Baker's cyst. Definitive joint arthropathy was present in all patients. The highest diagnostic yield was with a combination of synovial histology, synovial fluid culture and direct smear examination for acid fast bacilli (14 patients) and lowest if direct smear examination was used alone (2 patients). Chemotherapy with rifampicin and isoniazid alone (3 patients) or with at least 1 other drug was given for a mean of 15 +/- 5 months. Apart from debridement/drainage surgery, fusion/excision arthroplasty was performed in 6 cases and one had a total knee replacement. A heightened diagnostic acumen is needed in such cases.

Pattern of adult onset of polymyositis and dermatomyositis and association with malignancy.

A retrospective study of 22 adult patients with dermatomyositis (DM) or polymyositis (PM) was performed. Male to female ratio was 1:2.7. Mean age of onset was 37.3 +/- (16.3) and symptoms were present for a mean of 11.2 +/- 14.6 months before diagnosis. Primary polymyositis was diagnosed in 11 (50%), primary dermatomyositis in three (13.6%). PM/DM was associated with connective tissue disease in three (13.6%) and malignancy in five patients (22.7%). Muscle disease followed the diagnosis of malignancy by a mean of 12.2 months (one to 36 months). All were female. Diffuse erythema was observed in all three patients with DM and malignancy. Arthritis was seen more frequency in our patients (55%). Sixty-eight percent of patients showed substantial improvement of muscle disease with steroids alone or in combination with other immunosuppressive agents, 18% did not improve or their disease progressed in spite of the treatment. Three patients died (14%), two from respiratory failure and one from underlying malignancy.

Association of anti-cardiolipin antibodies with vascular thrombosis and neurological manifestation of Behçets disease.

We have studied 44 patients with Behçet's Disease (BD) to look for any correlation of arterial and venous thrombosis or central nervous system (CNS) manifestations with anti-cardiolipin antibodies (ACLA). Twenty patients were positive for ACLA by MELISA method. Ten patients had IgG antibody, four had IgM and six had both IgG and IgM. Of these patients, 11 had a history of vascular thrombosis and thrombophlebitis and nine had CNS manifestations. The association of ACLA with vascular thrombosis or CNS manifestation of Behçet's disease was statistically not significant.

Analysis of peripheral blood lymphocyte subsets in normal Saudi males by flow cytometry.

Lymphocyte immunophenotyping using flow cytometer has become an important tool for clinical patient management as well as for research and epidemiological studies. We examined the distribution of CD3 (all T cells), CD4 (T helper/inducer cells), CD8 (T suppressor/totoxic cells), CD16 (natural killer cells) and CD19 (B cells) in 150 healthy Saudi male blood donors using flows cytometry. The two-color labeled cells were analyzed by using the flow cytometer (FACScan, Becton-Dickinson, San Jose, California, USA) and the dual fluorescent subsets were discriminated by Simultest software. The distribution of T lymphocytes, B lymphocytes, and natural killer (NK) cells were similar to those reported in other populations as well as in normal Caucasian expatriate donors (all males) (n = 40) who were included in this study as controls. However, a significantly decreased CD4/CD8 ratio was observed in most Saudi blood donors. These lower ratios were due to decreased CD4 together with an increase in CD8 cells. Significant (P<0.00001) difference in CD4/CD8 ratio in our study may be due to environmental factors such as ultraviolet radiation and stress (heat) as well as some genetic factors.

Transferrin C subtypes in patients with rheumatoid arthritis.

Transferrin (Tf) subtypes were investigated in 128 patients with rheumatoid arthritis (RA) and the frequencies of TfC subtypes were compared with the results in normal individuals. The frequencies of the Tf genes: C1, C2, C3, D1, and D2 were 0.4765, 0.3867, 0.0742, 0.0390 and 0.0234, respectively. The frequency of TfC2 gene was significantly higher in these patients (0.3867) compared to the value in the control group (C2 = 0.247). The relative risk of RA in association with TfC1C2 type was 2.0, while it was 0.18 in association with TfC1C1 type and the results were statistically significant. This paper confirms the significant association between TfC2 and RA. Furthermore, it appears from our results that TfC1 homozygous phenotype is protective for the development of RA. The results are discussed in the light of earlier suggestions that the TfC2 subtype confers an increased risk of cellular damage by enhancing hydroxyl radical formation, although it is possible that there exists a genetic linkage of Tf variant to some other locus which is influencing susceptibility to RA.