FOXP3 Gene Variants in Patients with Systemic Lupus Erythematosus: Association with Disease Susceptibility in Men and Relationship with Abortion in Women.

BACKGROUND: FOXP3, an important transcription factor of regulatory T cells has shown a contribution to the development of various autoimmune diseases. OBJECTIVES: To investigate the influence of FOXP3 polymorphisms (rs3761548 and rs2294021) on systemic lupus erythematosus (SLE) susceptibility and patients' characteristics. METHODS: Genotyping was performed on 265 patients with SLE and 404 healthy controls using PCR-RFLP. Patients' demographic, laboratory, and clinical information were all documented. The relationship between the SNPs and patients' characteristics was statistically analyzed. RESULTS: The frequency of C/- genotype in male patients was significantly higher than in the healthy male controls, whereas the frequency of A/- genotype was lower (OR=0.53; 95% CI=0.28-1.00, p=.05). Analysis of the correlation between these SNPs and the patients' characteristics showed a longer disease duration in the rs3761548 C/- carriers and a correlation with arthralgia in both SNPs. In the females, there was a significant association between CC haplotype and disease susceptibility (OR=0.6, CI=0.38-0.94, p=.027). A significant association of both SNPs with the history of abortion was also detected. The frequencies of the rs3761548 AA (p=.006) and the rs2294021 CC genotypes (p=.038) and AC/AC combination (p=.033) were higher in women who had an abortion. We found a correlation between the rs3761548 AC genotype and the decreased C4 level and cardiovascular involvement, and the rs2294021 CC genotype with ESR, neurological involvement, and photosensitivity. CONCLUSIONS: FOXP3 rs3761548 C/- genotype association with disease susceptibility in male patients, an association of both SNPs with the abortion risk in female patients, and the correlation between these SNPs and several clinical features of the patients suggest their association with the disease development and pathology.

Association Between IL-17A, FOXP3, and CTLA4 Genes Expression and Severity of Lupus Nephritis

INTRODUCTION: Elevated levels of interleukin 17A (IL-17A) have been found in systemic lupus erythematosus (SLE). Forkhead box protein P3 (FOXP3) activates T-regulation lymphocytes and is a master regulator cell function. The cytotoxic T-lymphocyte-associated protein 4 (CTLA4) gene plays a similar role. We investigated the role of these expressions in SLE patients with/without nephritis. METHODS: The present study was a case-controlled study including 49 patients with SLE and 26 healthy controls. The genes expression of IL-17A, FOXP3, and CTLA4 were measured by quantitative Real-Time PCR. The relation between lupus nephritis and disease activity with IL-17A, FOXP3, and CTLA4 genes expression was evaluated. RESULTS: IL-17A, FOXP3, and CTLA4 expressions in T-cells were significantly higher in SLE patients than controls (P < .0001). When comparing the nephritis group and no nephritis group to the control group individually, the expression of mentioned genes is also higher (P < .05). There was no significant difference regarding IL-17A, FOXP3, and CTLA4 genes expression in the nephritis group and no nephritis group (P > .05). But there was a low expression of FOXP3 and IL-17A in patients with the higher stage of nephritis (P < .05). CONCLUSION: Our findings elevated IL-17A, FOXP3, and CTLA4 expressions significantly contribute to SLE pathophysiology. This study provides new insight into the function of IL-17A, FOXP3, and CTLA4 in disease setting. The heterogeneity of SLE patients is reflected in the multiple abnormalities found in the immune system. Finding such variations can provide targets for better manipulation of the immune system. DOI Code: DOI: 10.52547/ijkd.6537

Effects of combined aerobic and anaerobic exercise training on cytokine profiles in patients with systemic lupus erythematosus (SLE); a randomized controlled trial.

BACKGROUND: Systemic lupus erythematosus (SLE) is an inflammatory rheumatic disease characterized by production of autoantibodies and organ damage. Elevated levels of cytokines have been reported in SLE patients. Physical activity could be considered one of the factors that affect the immune system status and function. The aim of the present study was to evaluate the effects of an 8-week supervised aerobic and anaerobic training program on the immune system of SLE patients through evaluation of serum cytokine levels. METHODS: This cross-sectional study included 24 SLE patients selected between September 2015 and March 2016. The patients were randomly divided into two groups, including exercise (n = 14) and control (n = 10) groups. The exercise group participated in an 8-week combined supervised exercise training program consisting of three times per week in 60-min exercise sessions. After collection of whole peripheral blood, peripheral blood mononuclear cells (PBMCs) were isolated from the blood samples. Following RNA extraction and cDNA synthesis, the expression levels of IFN-γ, TNF-α, IL-6, IL-2, IL-4, IL-5, IL-9, IL-10, IL-13, IL-17A, IL-17F, IL-21, and IL-22 were determined using in-house SYBER Green-based real-time polymerase chain reaction (PCR). Lastly, the data obtained were analyzed using t-test. RESULTS: The mean and standard deviation of age were 29.00 ± 3.19 and 21.50 ± 5.52 in the intervention and control groups, respectively. No significant differences were found among the mean serum levels of IFN-γ, IL6, IL-9, IL-17A, IL-17F and IL-21 among SLE patients in the intervention and control groups. The mean serum levels of TNF-α, IL2, IL-4, and IL-5 decreased significantly in the intervention as compared with the control group. The mean serum levels of IL-10, IL-13 and IL-22 significantly increased in the control group after eight weeks, as compared with the intervention group. CONCLUSIONS: Our findings indicated that the 8-week supervised aerobic and anaerobic training program could result in decreased inflammatory cytokines.

The effects of garlic (Allium sativum) supplementation on inflammatory biomarkers, fatigue, and clinical symptoms in patients with active rheumatoid arthritis: A randomized, double-blind, placebo-controlled trial.

Based on the antiinflammatory properties of garlic, current study was conducted to evaluate the garlic supplement effects on serum levels of some inflammatory biomarkers, clinical symptoms, and fatigue in women with active rheumatoid arthritis. In this randomized, double-blind, placebo-controlled trial study, 70 women with RA were randomly divided into two groups: The intervention group was supplemented with 1,000 mg of garlic, and the control group received placebo for 8 weeks. At baseline and at the end of the study, clinical symptoms, fatigue, serum level of C-reactive protein (CRP), tumor necrosis factor-a (TNF-a), and erythrocyte sedimentation rate (ESR) were determined. After intervention, serum levels of CRP (p = .018) and TNF-a (p < .001) decreased significantly in the garlic group as compared with the placebo group. Also, pain intensity, tender joint count, disease activity score (DAS-28), and fatigue were significantly decreased in the intervention group compared with the control group (p < .001; for all). Swollen joint count was significantly decreased in the garlic group (p < .001), but not in the placebo group (p = .123). No significant changes were observed for ESR. Garlic supplementation by improving inflammatory mediators and clinical symptoms can be considered as a potential adjunct treatment in patients with RA. However, further studies with larger duration are needed.

A randomized, double-blind, placebo-controlled clinical trial, evaluating the garlic supplement effects on some serum biomarkers of oxidative stress, and quality of life in women with rheumatoid arthritis.

AIM: Rheumatoid arthritis (RA), is a prevalent immune-inflammatory disease, which is associated with disabling pain. Oxidative stress might play a role in RA pathogenesis and outcomes. According to the antioxidant properties of garlic, the current study was performed to evaluate the garlic supplement effects on some serum levels of oxidative stress biomarkers, and quality of life in patients with rheumatoid arthritis. METHODS: Seventy women with RA participated in this randomized, double-blind, placebo-controlled, parallel-design trial. The patients were randomly divided into two groups, receiving two tablets of either 500 mg garlic or placebo daily for 8 weeks. Serum levels of total antioxidant capacity (TAC) and malondialdehyde (MDA) and quality of life were determined at baseline and end of week 8. A health assessment questionnaire (HAQ) was used to evaluate the quality of life related to health. RESULTS: Of 70 patients enrolled in the trial, 62 subjects were included in the final analysis. At the end of the study, there was a significant increase in serum levels of TAC in the garlic group as compared with the placebo group (26.58 ± 77.30 nmol of Trolox equivalent/ml vs 16.11 ± 0.92 nmol of Trolox equivalent/mL; P = .026). In addition, MDA levels were significantly decreased in the intervention group compared with the control group (-0.82 ± 1.99 nmol/mL vs 0.36 ± 2.57 nmol/mL; P = .032). Pain after activity and HAQ scores decreased in the garlic group compared with the placebo (-11.96 ± 13.43 mm vs -0.06 ± 13.41 mm; P < .001, 0.17 ± 20 vs 0.05 ± 0.15; P < .001, respectively). CONCLUSIONS: The findings suggest that garlic supplementation for 8 weeks resulted in significant improvements in oxidative stress, HAQ in women with RA.

Value of serum glucose-6-phosphate isomerase in patients with rheumatoid arthritis and correlation with disease activity: A case-control study.

BACKGROUND: Rheumatoid arthritis (RA) is a common multisystemic autoimmune disease with peripheral joint involvement. Many autoantibodies have been introduced in the course of RA; some of them have diagnostic and prognostic value. In this study, our aim is to determine the value of serum glucose-6-phosphate isomerase (G6PI) antigen (Ag) as a diagnostic and prognostic marker in RA. MATERIALS AND METHODS: Eighty-seven known cases of RA who referred to an outpatient clinic of Shiraz University of Medical Sciences and 76 healthy controls were selected. Serum G6PI Ag was measured using sandwich enzyme-linked immunosorbent assay method, and the enzyme level was compared in the patient and control group, we also compared the enzyme level of patient group with disease activity, disease duration, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), and anti-cyclic citrullinated peptide (anti-CCP) antibody (Ab). The data were analyzed using SPSS V 16 software. RESULTS: Positivity for G6PI was detected in 34.5% (30/87) of RA patients and 9.2% (7/76) of control group (P < 0.001). There was no significant correlation between enzyme level and disease activity, disease duration, ESR, CRP, RF, and anti-CCP Ab. CONCLUSIONS: Overall, in our study, although there was a significant difference in serum G6PI Ag between patient and control group, no significant correlation was detected between serum G6PI level and disease activity score, ESR, CRP, and anti-CCP Ab, but relative correlation with ESR and disease duration could be challenging. G6PI Ag could be introduced as a diagnostic marker in RA, but its role as a prognostic marker is controversial.

Discernment scheme for paraquat poisoning: A five-year experience in Shiraz, Iran.

AIM: To evaluate various schemes for paraquat poisoning and different variables that influence the outcome of acute paraquat poisoning. METHODS: In a cross-sectional study, the information about all cases of acute paraquat poisoning who were admitted to teaching hospitals affiliated to Shiraz University of Medical Sciences, in a five year period (September 2010 to September 2015) were evaluated. The variables included: Demographic data, medical assessment, therapeutic options, laboratory findings, and the outcomes. Data were analyzed using SPSS, version 22. Significant difference between groups was tested using t-test for continues outcomes and χ2 test for categorical. The significance level was considered to be P < 0.05. RESULTS: A total of 104 patients (66.3% male) were evaluated. The mean age of the female patients was 22.81 ± 9.87 years and the male patients' was 27.21 ± 11.06 years. Ninety seven (93.3%) of all the cases were suicide attempts with mortality rate of 43.2%. Despite the necessity for emergency hemodialysis during the first 6 h of intoxication, none of the patients had dialysis during this time. Immunosuppressive and corticosteroid medications were not administrated in adequate dosage in 31.1% and 60% of the patients, respectively. Ingestion of more than 22.5 cc of paraquat and increase in creatinine level were the most important predictors of mortality. CONCLUSION: Treatment should start immediately for these patients. Moreover, creating a clinical guideline according to the findings can have an impact on the treatment procedure which seems to be necessary.

Investigation of the human FCRL1, 2, and 4 gene expressions in patients with rheumatoid arthritis.

The Fc receptor-like (FCRL) molecules have recently been shown to contribute to the pathogenesis of certain autoimmune disorders. In this study, we investigated the expression levels of FCRL1, 2 and 4 in blood mononuclear cells from rheumatoid arthritis patients using real-time PCR. The mRNA of these molecules was detected in 44.4 % (FCRL1), 53.3 % (FCRL2) and 31.1 % (FCRL4) of patients. Comparatively, 31.1 % (FCRL1), 51.1 % (FCRL2) and 26.6 % (FCRL4) of controls expressed these genes. Analysis of gene expressions in FCRL-positive patients demonstrated a lower FCRL4 gene expression in patients compared to controls (P < 0.001). In FCRL2-positive patients, a significant positive correlation between FCRL2 expression and the erythrocyte sedimentation rate (P < 0.001), anti-cyclic citrullinated peptide antibody (P = 0.033) level and disease activity score (DAS28, P = 0.016) was found. In conclusion, decreased FCRL4 expression and association of FCRL2 expression with inflammatory markers and disease activity suggested the contribution of these molecules to RA inflammatory processes.

Periodontal Health Status among Patients with Behçet's Disease.

We aimed to evaluate the relationship of individual periodontal parameters with the severity of Behçet's disease (BD) and attempt to find the correlation between chronic periodontitis and BD. In this study, 74 registered subjects attending Behçet's clinic with BD symptoms were recruited. The diagnosis was based on the criteria presented by the international study group for BD (ISG) and the total clinical severity score was determined for each patient. All individuals underwent clinical examination to assess oral and periodontal status and presence of oral ulcers. Periodontal clinical parameters of bleeding on probing (BOP), probing depth (PD), and clinical attachment level (CAL) and also hygiene index (HI) and decayed, missing, and filled (DMF) teeth were noted and analyzed to assess the correlation with severity of BD. There was no significant correlation between DMF and severity of BD. The strong association was found between periodontal parameters (BOP, PD, and CAL) and the severity of BD (P < 0.001). It seems that relation of BD to oral health is higher up in severe forms of BD and periodontal diseases. Clinical association between the diseases might be due to a common underlying etiopathogenesis of periodontitis and BD.

No Definite Association between Human Parvovirus B19 Infection and Behçet Disease.

BACKGROUND: The etiology of the Behçet disease (BD) has remained obscured. There have been studies to show the association of BD to infections like herpes simplex, hepatitis, and parvovirus B19 however, the findings are rather controversial. MATERIALS AND METHODS: We selected 55 patients with the best matched symptoms of BD and measured the loads of B19 DNA in their plasma by quantitative real time PCR and verified their seropositivity by ELISA. All findings were compared to the results from 42 healthy persons. RESULTS: Patients showed a wide spectrum of BD symptoms. Serologic studies showed high prevalence of B19 IgG among the tested patients which was not statistically different with the healthy population (72.7% vs. 85.7%, respectively). Similarly, the prevalence of B19 IgM between patients and controls was not different (18% vs. 11.9%, respectively). No correlation was found between the presence of anti-B19 antibodies and the clinical observations. Only one person from the patient and control groups had detectable levels of B19 DNA without any difference or correlation with the disease symptoms. CONCLUSION: Our data could not establish an association between B19 parvovirus infection and Behçet disease, although there have been reports of such correlation. Nevertheless, there might be indirect relation in genetically susceptible individuals after viral infections. More studies on designed animal models and surveys on patients should be done to resolve this controversy.

Patients with Active Rheumatoid Arthritis Have Lower Frequency of nTregs in Peripheral Blood.

BACKGROUND: Patients with rheumatoid arthritis (RA) suffer from wide ranges of autoimmune reactions in joints. The mechanism of which is generally unknown and maybe associated with Treg deregulation. OBJECTIVE: To compare the frequency of nTregs in peripheral blood of patients with active rheumatoid disease with healthy individuals. METHODS: Twenty five newly diagnosed patients with active RA disease were selected based on the clinical and laboratory criteria before starting their therapies. Treg cells in peripheral blood samples were enumerated by immune staining and flowcytometry analysis. RESULTS: Clinical and laboratory results were in favor of active disease in all the studied patients although they showed variations in Disease Activity Score-28 (DAS-28). Compared to the healthy controls, RA patients had significantly lower frequency of CD4+CD25hi or CD4+CD25+FoxP3+ natural regulatory T cells. In spite of that, there were no significant differences between patients and healthy controls in respect to the CD4/CD8 ratio. Interestingly, more CD4+CD25-FoxP3+ cells were found in peripheral blood of patients. The frequencies of the Tregs did not show strong associations with the DAS-28. CONCLUSION: We showed lower abundance of nTregs in peripheral blood of RA patients which highlights the significance of these cells in RA.

Efficacy and safety of topical Matricaria chamomilla L. (chamomile) oil for knee osteoarthritis: A randomized controlled clinical trial.

OBJECTIVE: To assess the efficacy and safety of topical Matricaria chamomilla (Chamomile) oil in patients with knee osteoarthritis. METHOD: Patients were randomized and treated with topical chamomile oil, diclofenac or placebo, 3 times/day for 3 weeks. They were allowed to use acetaminophen as analgesic. The patients were asked about their total acetaminophen use. Moreover, they were assessed in the terms of pain, physical function and stiffness by using Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) questionnaire at the enrolling and weekly. RESULTS: Chamomile oil significantly reduced the patients' need for acetaminophen (P = 0.001) compared with diclofenac and placebo. However, there were no significant differences in WOMAC questionnaire domains. The patients did not report any adverse events by using chamomile oil. CONCLUSION: Chamomile oil decreased the analgesic demand of patients with knee osteoarthritis. In addition, it may show some beneficial effects on physical function, and stiffness of the patients.

Refractory Angioedema in a Patient with Systemic Lupus Erythematosus.

Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

Anti-cardiolipin and anti-Neutrophil Cytoplasmic Antibodies in Iranian patients with Behcet's disease.

BACKGROUND: The prevalence of anti-Neutrophil Cytoplasmic Antibodies (ANCAs) and anti-Cardiolipin Antibodies (anti-CL Ab) in Behcet's Disease (BD) and also their roles in vascular involvement is controversial. OBJECTIVE: To assess the prevalence of ANCAs and anti-CL Ab as well as their correlations with clinical manifestations in Iranian patients with BD. METHODS: In this case/control study, the sera from 88 patients with BD and 88 healthy controls were evaluated. The levels of ANCAs and anti-CL Ab were measured using indirect ELISA method. RESULTS: The levels of anti-CL, anti-PR3 and anti-MPO (Myeloperoxidase) IgG autoantibodies between BD patients and healthy controls were not statistically different (p=0.21, p=0.28 and p=0.74, respectively). In addition, there were no significant deferences between BD patients with and without vascular involvement in the levels of anti-CL (1.42 ± 1.24 GPLU/ml and 1.58 ± 1.18 GPLU/ml, respectively; p=0.71), anti-PR3 (0.0 ± 0.0 U/ml and 0.08 ± 0.27 U/ml, respectively; p=0.10) and anti MPO (0.48 ± 0.23 U/ml and 0.52 ± 0.22 U/ml, respectively; p=0.41) IgG autoantibodies. Nevertheless, mean titer of anti-CL IgG was higher in male patients with skin rash than those without skin rash (2.2 ± 0.88 GPLU/ml and 1.11 ± 1.22 GPLU/ml, respectively; p=0.017). CONCLUSION: While anti-CL, anti-PR3 and anti-MPO IgG autoantibodies do not play a major role in susceptibility to BD or pathogenesis of vascular involvement in our patients, anti-CL Ab might be involved in skin lesion development in Iranian male BD patients. However, the results should be confirmed in other studies.

Infliximab for patients with neuro-Behcet's disease: case series and literature review.

This study aims to report the therapeutic effects of anti-tumor necrotic factor antibody, infliximab, for treatment of neuro-Behcet's disease (NBD) and to review the literature. We described four patients (all male, median age 40 years old) who fulfilled the International Study Group criteria for Behcet's disease (BD) and presented with neurological complication. Demographic and clinical characteristics of the patients, dose, therapeutic effects, and adverse drug reaction (ADR) of infliximab were reported. Two patients had secondary progressive, one relapsing progressive, and one primary progressive course (median duration of BD and NBD 11 and 2 years, respectively). Two patients each received infliximab with 3 and 5 mg/kg infusions, respectively. The patients received infliximab for median of 22 weeks. Clinical responses were unsatisfactory for two patients on 3 mg/kg regimen; and good in two patients on 5 mg/kg and monthly intravenous 500-1,000 mg cyclophosphamide. Varicella zoster infection was seen as a major ADR in one patient. Our results with infliximab were not as promising as the previous reports. Infliximab, 5 mg/kg per dose with adjuvant immunosuppressive therapy, is probably more effective than other regimens.

Quantification of regulatory T cells in peripheral blood of patients with systemic lupus erythematosus.

Regulatory T cells (Tregs) are supposed to stop immune responses in the course of immune activation. However, chronic activation of immune system in systemic lupus erythematosus (SLE) and many other autoreactive disorders are evidence of malfunction of this system. Therefore, it is plausible to quantify presence of these cells in different diseases. Forty-one patients with diagnosis of SLE were enrolled in this study. Patients were divided into two groups of patients with active and inactive disease based on the disease activity score. Flow cytometry analysis was used to determine the frequency of regulatory T cells in peripheral blood according to high expression of CD25 and intracellular Forkhead/winged-helix (Foxp3). Further 30 healthy individuals considered as control group. Significantly less CD4+CD25hi regulatory T cells were detected in active patients (P < 0.001) compared to healthy individuals. The percentage of CD4+CD25hi cells were inversely correlated with the SLEDAI disease score in patients with active disease (r = -0.837, P < 0.0001). Patients with active disease had lower frequencies of CD4+Foxp3+ cells. However, increased frequencies of CD4+Foxp3+ T cells were observed in peripheral blood of patients with inactive disease compared with active patients or healthy individuals (P < 0.010). Moreover, a significant difference between the proportion of CD4+CD25-Foxp3+ population in healthy controls and patients with active disease was shown (P < 0.0005). Presence of lower frequencies of Tregs in patients with SLE could be evaluated as an immune turbulence and could be employed as a target for immunotherapeutic manipulation. However, controversies need to be resolved.

Raised concentration of soluble form of vascular endothelial cadherin and IL-23 in sera of patients with Behçet's disease.

Behçet's disease (BD) is a chronic multisystem vasculitis disease that can affect any organ and usually is combined with hyperactivation of neutrophils. Involvement of inflammatory cytokines such as interleukin (IL)-12 in BD has been shown before. However, IL-12 shares a p40 subunit with IL-23, which has additional inflammatory effects apart of IL-12. IL-23 increases neutrophils' transmigration and therefore could contribute in BD induction or progression. Moreover, endothelial cells express vascular endothelial cadherin adhesion molecule (VE-cadherin), which plays critical roles in angiogenesis and endothelial integrity. VE-cadherin may shed into the circulation in a soluble form (sVE-cadherin), and inflammatory cytokines can increase this process. Therefore, a correlation between IL-23 concentration and amount of sVE-cadherin was proposed. We enrolled 44 healthy persons and 53 patients with BD of different disease activities and examined their serum concentrations of IL-23 and sVE-cadherin. A significant correlation was found between the concentrations of these two factors among patients only. Comparing sVE-cadherin mean concentration in patients and controls showed a significant difference, which for IL-23 was not considered significant. Results showed higher IL-23 in sera of patients with uveitis. Moreover, there was a meaningful correlation between IL-23 content and disease activity. These results could extend the biological effects of IL-23 in BD and introduce sVE-cadherin as a potential new biomarker in the course of BD pathogenesis.

Pattern of ankylosing spondylitis in an Iranian population of 98 patients.

The prevalence and pattern of ankylosing spondylitis (AS) can vary from country to country, according to genetic and environmental factors. This study aims to analyze the patterns of disease in a population of Iranian patients with AS. We performed a prospective study (2002-2007) analyzing 98 patients with diagnosis of AS according to the modified New York criteria. Selected patients underwent complete clinical (initial symptom, axial and peripheral involvement, heel enthesitis, extra-articular manifestations) and radiological (sacroiliac, lumbar, thoracic, and cervical spine) investigations, and these data were compared with sex, age at onset, and HLA-B27. There was predominance of men (71.4%), adult onset (>16 years, 90.8%), and positive HLA-B27 (73.4%). Family history of AS was noted in 14.3% of the patients. The predominant initial symptoms were inflammatory low back pain (44.2%). Radiological findings included syndesmophytes in 34.7% and "bamboo spine" in 16.3% of patients. Acute anterior uveitis was noted in 44.9% of patients. Male sex was associated with involvement of shoulder (P = 0.001). Female sex and juvenile-onset AS were associated with extra-articular involvement. Positive HLA-B27 was associated with hip involvement (P = 0.042) and adult-onset AS (P = 0.035). Analysis of the patterns of disease in this population of 98 southern Iranian patients with AS revealed that female sex and juvenile-onset AS were associated with extensive extra-axial involvement; and HLA-B27 was associated with hip involvement.