davatchi

Pemphigus Vulgaris (PV) is often complicated by osteoporosis. Although corticosteroid therapy undoubtedly plays a causative role, inflammation associated with PV may also contribute to osteoporosis. This study was designed to determine the prevalence of osteoporosis in patients with PV before corticosteroid therapy and to compare these findings with those reported previously in healthy volunteers. Newly diagnosed patients with PV, who had not received systemic corticosteroids, were enrolled. Bone mineral density (BMD) was measured both in the lumbar spine (L1-L4) and hip region. Data were compared with those of a healthy Iranian population. The association between the disease duration and severity and BMD was evaluated. A total of 50 patients (27 women) with a mean age of 42.6±14.5 years were enrolled. Osteoporosis was seen in 7 (14%) patients, 3 (11.1%) women, 4 (17.4%) men, and in both genders it was more common when compared to the population of healthy Iranians (8.2% in women and 4.9% in men). Osteopenia was found in 26 (52%) patients, 13 women and 13 men. Although both osteopenia and osteoporosis were more common in severe disease, neither the duration nor the severity of PV showed a statistically significant association with osteopenia or osteoporosis. The presence of a higher than expected rate of osteoporosis in patients with PV argues for osteoporosis screening and efforts aimed at prevention and early initiation of treatment to prevent unnecessary morbidity.

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin or mucosa. Since low vitamin D status has been linked to many immune disorders, we designed this study to compare the vitamin D status in PV patients with healthy controls. In this case-control study, vitamin D status of 32 newly diagnosed PV patients was compared with 36 healthy control subjects. All patients were selected from the specialized dermatology departments of Razi Hospital, Tehran University of Medical Sciences in a 2-year period (2009-2010). The severity of the disease was estimated according to Harman’s scores. Serum concentration of 25(OH)D was measured by Roche Elecsys System. Data were analyzed by independent t-test. Both groups were similar based on sex, age and body mass index. The mean duration of disease was 5.57±0.93 months. The mean oral and skin severities were 1.81±0.20 and 2.31±0.17 respectively, based on Harman’s scores. Serum 25(OH)D was significantly lower in PV patients compared to controls (-8.90; 95% CI, 2.29-15.51 and P = 0.009). There was a negative correlation between vitamin D level and the oral severity of disease (r = -0.39 and P = 0.02). PV patients had significantly lower serum level of 25(OH)D compared to healthy subjects which might contribute to worsen the disease. These data indicate the importance of improving vitamin D level in pemphigus patients.

Pemphigus is an autoimmune blistering mucocutaneous disorder. Common treatments include corticosteroids and immunosuppressive drugs. This study aimed to assess the therapeutic effects of oral prednisolone along with the common adjuvant therapy in pemphigus vulgaris. Eighty-seven patients with pemphigus vulgaris from the first stage of a previously randomized clinical trial were enrolled in the present non-blinded clinical trial. The patients were divided into four groups and treated accordingly with prednisolone alone (P; N = 23), prednisolone and azathioprine (P/A; N = 23), prednisolone and mycophenolate mofetil (P/M; N = 21), and prednisolone and cyclophosphamide (P/C; N = 20). These patients were followed-up for an extended one-year period. The primary localization of the recurrence occurred in the oral cavity of 7, 6, 2, and 5 patients in the P, P/A, P/M, and P/C groups, respectively. There was no significant difference between them (P = 0.40). The mean total dose of prednisolone administered in groups P, P/A, P/M, and P/C was accordingly 7.5, 8.4, 9.2, and 8.6 mg/day. Minor recurrence of the disease in the above-mentioned groups was observed in 7 (30.4%), 5 (21.7%), 6 (28.6%), and 7 (35.0%) of the patients, respectively. With regard to the minor recurrence of the disease, there was no significant difference among the four treatment groups (P = 0.80). Since in this follow-up study no therapeutic benefit of oral prednisolone and common adjuvant therapy was found in terms of the number of minor and major recurrences, the extent to which treatment of PV can be improved upon treatment with these agents remains to be elucidated.

Pemphigus is a rare autoimmune blistering disease with different phenotypes. The evaluation of therapeutic interventions requires a reliable, valid and feasible to use measurement. However, there is no gold standard to measure the disease activity in clinical trials. In this study we aimed to introduce the pemphigus vulgaris activity score (PVAS) measurement and to assess the convergent validity with the experts’ opinion of disease activity. In PVAS scoring, the distribution of pemphigus vulgaris antigen expression in different anatomical regions is taking in to account with special consideration of the healing process. PVAS is a 0-18 scale, based on the extent of mucocutaneous involvement, type of lesion and the presence of Nikolsky’s sign. The sum of the scores of total number of lesions, number of different anatomic regions involvement and Nikolsky’s sign is weighted by the type of lesion. In the present study, PVAS was assessed in 50 patients diagnosed with pemphigus vulgaris by one dermatologist. Independently, five blinded experts scored all the patients through physician’s global assessment (PGA). The convergent validity with experts’ opinion was assessed. The Spearman coefficient of correlation showed the acceptable value of 0.751 (95%CI: 0.534- 0.876). PVAS is a valid, objective and simple-to-use scoring measurement. It showed a good correlation with PGA of pemphigus disease activity in Iranian patients with pemphigus vulgaris.

A common Human Leukocyte Antigen (HLA) class II allele, DQβ1*03:01, seems to be associated with Bullous pemphigoid (BP) in Caucasians whereas previous studies in other ethnic groups showed other HLA class II alleles as genetic predisposing factors for BP. To investigate the association of HLA class II alleles and haplotypes with BP in Iranian population. Fifty patients with Bullous pemphigoid and 180 geographically matched, healthy individuals as control group enrolled into this study. HLA typing of class II (DR and DQ alleles) was carried out using polymerase chain reaction based on sequence-specific primers method. Class II DQA1 and DQB1 typing showed a significantly higher frequency of HLA-DQA1*05:01 (45% vs. 33%, p=0.03), HLA-DQB1*03:01 (36% vs. 23.6%, p=0.02) and HLA-DQB1*04:01 (4% vs. 1.6%, p=0.04) in the BP patients compared with controls. For DRB1 allele frequencies, there were no significant disease associations. The frequency of DRB1*08:01/DQA1*05:01/DQB1*03:01 (3% vs. 0%, p=0.02) haplotype showed an increase among patients compared with controls. Our data suggest that Iranian patients with BP present the same genetic predisposition linked to HLA-DQB1*03:01 previously reported in Caucasians

Pemphigus vulgaris (PV) is an autoimmune bullous disorder that is fatal if left untreated. High dose systemic corticosteroids are the basis of therapy. The addition of immunosuppressive agents has improved the disease outcome and reduced the required corticosteroid dose and related toxicity. Mycophenolate mofetil is increasingly used as a steroid-sparing agent in immunotherapy of PV. Herein، we tried to appraise the efficacy of mycophenolate mofetil and topical clobetasol in the control of the major relapses of pemphigus vulgaris. Seventeen patients with severe relapse of pemphigus vulgaris were included in this study. All patients had complete remission on/off therapy before this period of recurrence. The patients were treated with 2g/day mycophenolate mofetil and 25-35g/day topical clobetasol propionate ointment. All patients were monitored for the side effects of therapy. The patients were followed for a mean period of 12. 7 months. The average length of time from initiating mycophenolate to 50% control (partial remission)، which occurred in all patients، was 6±1. 17 weeks. Fifteen patients achieved complete remission averagely at week 20. 8±7. 70. The average duration of followup after complete disease control was 8 months (ranging from 2-13. 5 months). Three patients were free of lesions for more than 12 months and 10 for more than 6 months. No important mycophenolate mofetil related complication was observed during treatment. The combination of mycophenolate mofetil and topical corticosteroid can be used to control PV relapses and taper-off corticosteroid.

Pemphigus vulgaris is a rare autoimmune disorder characterized by cutaneous and mucosal blistering. Surprisingly، the management of oral lesions has been detailed only infrequently. As current topical therapies for oral lesions are of limited efficacy، application of calcineurin inhibitors is considered to be a potential option. The aim of this essay was to investigate the efficacy of tacrolimus 0. 1% ointment (Protopic®) versus triamcinolone acetonide 0. 1% paste (Volon-A®) in the treatment of oral pemphigus vulgaris. Fifteen patients were involved in a prospective randomized trial with a split- mouth design. After two weeks of administering study drugs، oral lesions were monitored and quantified pain and mucosal surface involvement scores were obtained. Within 14 days، the degree of involvement and pain scores significantly reduced in both tacrolimus-treated and triamcinolone-treated sites، but there was no significant difference between them. No severe adverse events were observed. This study showed that tacrolimus could be as effective as triamcinolone acetonide in the topical treatment of oral pemphigus vulgaris.

The aim of the article is demonstrating an application of multiple imputation (MI) for handling missing clinical data in the setting of rheumatologic surveys using data derived from 10291 people participating in the first phase of the Community Oriented Program for Control of Rheumatic Disorders (COPCORD) in Iran. Five data subsets were produced from the original data set. Certain demographics were selected as complete variables. In each subset, we created a univariate pattern of missingness for knee osteoarthritis status as the outcome variable (disease) using different mechanisms and percentages. The crude disease proportion and its standard error were estimated separately for each complete data set to be used as true (baseline) values for percent bias calculation. The parameters of interest were also estimated for each incomplete data subset using two approaches to deal with missing data including complete case analysis (CCA) and MI with various imputation numbers. The two approaches were compared using appropriate analysis of variance. With CCA, percent bias associated with missing data was 8.67 (95% CI: 7.81-9.53) for the proportion and 13.67 (95% CI: 12.60-14.74) for the standard error. However, they were 6.42 (95% CI: 5.56-7.29) and 10.04 (95% CI: 8.97-11.11), respectively using the MI method (M=15). Percent bias in estimating disease proportion and its standard error was significantly lower in missing data analysis using MI compared with CCA (P< 0.05). To estimate the prevalence of rheumatic disorders such as knee osteoarthritis, applying MI using available demographics is superior to CCA.

To find the possible role of plasma homocysteine level as a contributing factor in venous and arterial thrombosis in patients with Behcet’s disease (BD). In a case control study, two groups of BD patients were included: 47 with thrombosis and 49 without thrombosis. All patients fulfilled the International Study Group Criteria for BD and the confirming diagnostic procedures for vascular thrombosis were either Doppler sonography or angiography. Forty-nine controls were selected by consecutive sampling among age and sex matched healthy subjects. Plasma homocysteine level was measured by ELISA in all. The clinical and laboratory characteristics of the disease were compared between the two groups of BD patients. Comparisons were done by ANOVA and Chi square tests; correlations were analyzed with Pearson test. The mean plasma homocysteine level was significantly higher in BD patients (14.9±13.9 mMol/L) than in healthy controls (9.9±6.7 mMol/L), P<0.02. The difference was also significant when comparing the three groups by ANOVA: BD patients with thrombosis (24.2±13.2 mMol/L), BD patients without thrombosis (5.9±7.0 mMol/L), and healthy controls (P<0.0001). We found no correlation between plasma homocysteine level and any organ involvement other than thrombosis. The mean plasma homocysteine level was lower in HLA-B51 positive BD patients (11.6±12.1 vs. 21.7±16.3 mMol/L, P<0.05), but the difference was not significant in those with thrombosis (20.9±13.2 vs. 29.5±12.7 mMol/L, P=0.18). Hyperhomocysteinaemia may be an independent risk factor for vascular thrombosis in patients with BD. This is the first study showing a negative correlation between HLA-B51 and plasma homocysteine level.

Rheumatology program at Tehran University was established by Fereydoun Davatchi in 1969. Later, Reza Moghtader, Firouz Panahi, and Amir-Khan Chafizadeh joined him. The first rheumatology outpatient clinic was founded in 1970. In 1974, the first rheumatology fellowship department was established along with a small research laboratory. The department expanded gradually to become the Rheumatology Research Center (RRC) in 1981. RRC was officially recognized in 1994 by the Ministry of Health and in 2001 as the Center of Excellence for Rheumatology in Iran. There are 15 rheumatologists who are the teaching staff at RRC. The center has trained up to 45 rheumatologists since 1985. RRC has conducted about 150 research projects (national and international collaborative projects). It has published 14 yearly books and collaborated in the publication of seven international books. RRC has presented 207 papers in local and 337 papers in international conferences. RRC has published 50 papers in local and 178 papers in international journals.

Psoriatic arthritis is defined as an inflammatory arthritis associated with psoriasis and is classified as a seronegative HLA-B27-associated spondyloarthropathy. We aimed to study the clinical and paraclinical presentations of psoriatic arthritis in 150 Iranian patients. Demographic, clinical, and paraclinical data obtained from 150 patients enrolled in a retro- and introspective study were analyzed. The female:male ratio was 1.11:1. The mean age at the onset of arthritis was 33.3 years. Arthritis occurred after psoriasis in 73%, preceded it in 17%, and simultaneously in 10% of the patients. The patterns of joint involvement were asymmetric oligoarthritis (46%), polyarthritis (5%), axial involvement (16%), distal interphalangeal joint involvement (23%), and mutilating arthritis (2.2%). There was no correlation between distal interphalangeal joint involvement and nail disease (P=0.33). Polyarthritis was more common in women (P=0.03) and axial involvement was more in men (P=0.02). Patients with spondylitis were younger at the disease onset (P<0.02). Dactylitis was seen in 12% of the patients. Enthesopathy (20.7%) was more common in patients with axial involvement (P=0.008) and joint deformity (15%) was more common in patients with polyarthritis or distal interphalangeal joint involvement (P<0.0002). Four patients had ocular inflammation. Anemia was detected in 24%, high erythrocyte sedimentation rate in 79%, and abnormal urinary indices in 9% of the patients. There was a significant correlation between HLA-B27 (16%) and axial involvement (P=0.01) as well as joint erosions (16%) and distal interphalangeal joint involvement (P<0.0001). Thirty-four patients (26.8%) showed radiographic findings of sacroiliitis, while only 14 of them had axial involvement. Oligoarthritis was the most common type of joint involvement in these Iranian patients. We found no correlation between distal interphalangeal joint involvement and nail changes, although it was seen with more erosive changes and joint deformity. In comparison with other studies, extra-articular manifestations were not common in our patients.

Anti-desmoglein 3 and 1 autoantibodies are involved in the pathogenesis of pemphigus diseases. Our objective was to assess the value of ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris. Based on clinical presentation and histopathologic confirmation for the diagnosis of the pemphigus, 38 patients took part in the study. Sera of the patients were tested by desmoglein 1 and desmoglein 3 ELISA. Also, direct immunofluorescence was performed for all patients which revealed positive results in 36 patients (94.7%). ELISA was positive in 37 of 38 pemphigus patients (Sensitivity: 97.3%). The relationship between desmoglein 1 index values and skin severity was statistically significant (p<0.05). Desmoglein 3 index values increased with oral severity although this was not statistically significant. Iranian patients similar to Indian patients had higher positive anti-desmoglein 1 autoantibodies. Desmoglein-ELISA test is appropriate in the diagnosis of pemphigus. Desmoglein 1 index value is statistically correlated with the severity of pemphigus vulgaris.

There is some data in the literature on the association of HLA-B5 and some manifestations of Behçet''s disease (BD), especially ocular lesions. We studied 433 patients to see if there was any relationship between B51 and the manifestations of the disease. Clinical manifestations of BD were compared in patients having HLA-B51 (155 patients) and those lacking HLA-B51 (278 patients). Oral aphthosis, genital aphthosis, skin manifestations, joint manifestations, gastrointestinal manifestations, phlebitis and neurological manifestations showed no significant difference in patients with and without HLA-B51. Ophthalmologic manifestations were seen in 52% of patients having B51 and in 42% of patients lacking it (χ2: 4.451, P = 0.035). Considering different lesions of ocular manifestations separately, no significant difference was found regarding the presence or absence of B51. Pathergy phenomenon was detected in 55% of B51 positive patients and in 45% of B51 negative patients (χ2: 4.111, P = 0.043). It seems that HLA-B51 may play a role in the pathogenesis of Behçet''s disease, but cannot be used as predictive value for the occurrence of organ involvement, except for the eye.

Indirect immunofluorescence (IIF) has been used to identify and measure autoantibody levels in pemphigus vulgaris but data about relationship between clinical severity of disease and antibody titers by IIF have been conflicting. We conducted this cross-sectional study to correlate the severity of oral and/or cutaneous involvement in patients with pemphigus vulgaris based on Pemphigus Area and Activity Score with IIF titers. Sixty-one new pemphigus vulgaris patients were included in this study. Human prepuce was used as substrate for IIF and assessment of disease severity was based on Pemphigus Area and Activity Score. The mean±SD age was 44.04±30.46 years, with a range of 18 to 79 years. IIF was positive in 56 (91.8%) patients. There was a significant relationship between total disease score and IIF titers ((P<0.001). Also a significant relationship was found between skin score (P=0.04) and mucosal score (P=0.04) with IIF titers. Our results show that there is a significant relationship between disease activity based on Pemphigus Area and Activity Score and antibody titers by IIF. Further studies are recommended to determine the usefulness of this technique for monitoring disease.

This study was conducted to determine the prevalence of oral aphthosis in a normal population in Iran, using the data of the WHO-ILAR COPCORD study in Iran. We conducted this study in Tehran, the capital of Iran which was selected as the COPCORD study field. In 22 districts of Tehran, 50 clusters were randomly selected. Of the selected houses, 4,096 households were visited and 10,291 persons were interviewed (response rate of 75%). Out of the 10291 subjects interviewed, 2592 had aphthous ulcers which translated to a prevalence of 25.2% (95% confidence interval: 24.4% to 26.0%). The prevalence of oral aphthosis was rather high in this normal population.

In addition to humoral immunity associated with anti-desmoglein antibodies, cellular immunity and mediators including cytokines are involved in the pathogenesis of pemphigus vulgaris. In this study we evaluated the level of IL-2, IFN-γ, TNF-α, IL-4, and IL-10 in the sera of patients with pemphigus vulgaris before and after treatment. A total number of 71 new patients with pemphigus vulgaris were included in the study. The above mentioned cytokines were measured in patients with a mild disease (20 bullae or less) and a severe disease (60 bullae or more) using ELISA method before and 4 weeks after treatment with 2 mg/kg/day prednisolone and 2mg/kg/day azathioprine. We also measured IL-4 and IL-10 in 69 mildly and 70 severely affected patients only at the beginning of the study. All patients had muco-cutaneous phenotype. Patients with a mild disease had mild mucosal involvement and patients with a severe disease had moderate to severe mucosal involvement. Serum levels of IL-2 and IFN-γ were also measured in 27 normal controls. In the total study population, the level of IL-2 decreased from 103.9 pg/ml to 82.79 pg/ml after treatment (p=0.05). Comparing cytokines between 2 groups (severe and mild), the level of IL-2 before treatment showed significantly lower figures in severe patients (147.27 versus 67.38, p=0.04). On the other hand, IFN-γ after treatment was significantly higher in severe patients (0.75 versus 0.42, p=0.04). Mean level of IL-2 is lower in severe pemphigus vulgaris patients than that of mild disease. This finding indicates that, in pemphigus vulgaris, IL-2 level negatively correlates with the severity of the disease and widespread underlying autoimmune process. The data also suggests that the level of IFN-γ directly correlates with the severity of the disease.

Chondromalacia patella is the most common cause of mechanical knee pain in young women. Q angle and knee alignment are important clinical parameters for biomechanics of the patellofemoral joint. To identify the prevalence of knee malalignment and to find out if there is a correlation between knee mal alignment and Q angle with age at onset of disease. All patients under 40 years old complaining of mechanical knee pain with positive shrug sign and normal knee radiography who were referred to Amir A''lam Rheumatology Clinic during the period of September 2000 to September 2002 were included in this study. The patients were examined by a rheumatologist for measuring Q angle and detection of knee malalignment. Age at onset of disease was defined as initiation of knee pain according to the patient''s history. The cases were 260 with 189 females. Prevalence of knee mal alignment was 32.4%. The mean age at onset of disease was 22.8 ± 7.08 years. The mean age at onset in patients with knee mal alignment (21.41 ± 5.66 years) was significantly lower than the mean age at onset in those without knee malalignment (23.6 ± 7.43 years) (p= 0.002). There was a positive correlation between Q angle and age at onset of disease (r=0.17,p= 0.006). It seems reasonable to identify knee malalignment in chondromalacia patella patients and perform proper management to postpone progression of disease. There are many other factors that influence age at onset of disease, so further investigation is recommended.

The natural history of ocular lesions in Behçet''s disease is toward severe loss of vision/blindness in few years, whereas cytotoxic drugs have changed the outcome. Several open labeled cohort studies showed pulse cyclophosphamide (PCP) to be the best choice. Since one third of these patients are resistant to PCP it is important to know how much improvement one can expect from the responders. To address this question, we selected patients who improved or maintained their baseline visual acuity after treatment. From a cohort of 528 patients (1056 eyes), 753 eyes were selected. At the beginning of the study PCP was given for one month as 0.75 to 1 g in perfusion, and then followed every 2 to 3 months. Prednisolone was also given as 0.5 mg/kg/daily and then tapered upon controlling inflammation. The mean±SD number of pulses was 11.5±8.5/month with follow-up of 20.6±19.8 months. Different disease activity indices such as visual acuity, posterior uveitis, retinal vasculitis, total inflammatory activity index (TIAI), total adjusted disease activity index (TADAI) were calculated at baseline and at last evaluation. The mean visual acuity improved from 2.4 to 4.4. The mean indices for posterior uveitis, retinal vasculitis, TIAI and TADAI improved from 2.2 to 1, 2.7 to 1.4, 19.3 to 9, and 27.2 to 20.5 respectively. The P value was less than 0.001 for all comparisons. Pulse cyclophosphamide is able to improve ocular lesions of Behçet''s disease; therefore it may be used as a first choice, especially in retinal vasculitis.

Behçet’s disease (BD) which is classified among vasculitides is a systemic disease with various manifestations. Its clinical course is characterized by attacks and remissions. Till now, two nationwide surveys of BD from Iran and Japan and 4 major case series from Turkey, Korea, Morocco and England have been reported. Clinical picture of BD is dominated by mucous membrane manifestations, including oral aphthosis - seen in 96.8% of patients in Iran, 98.2% in Japan, 100% in Turkey, 97.5% in Korea, 100% in Morocco and 100% in England- and genital aphthosis which is seen less frequently- 65.3% in Iran, 73.2% in Japan, 88.2% in Turkey, 56.7% in Korea, 83.5% in Morocco and 89% in England. Skin aphthosis is not frequent but it is the most characteristic lesion of BD. Ocular manifestations include anterior uveitis, posterior uveitis and retinal vasculitis. Joint manifestations include arthralgia, monoarthritis, oligo/poly arthritis, and ankylosing spondylitis. Other manifestations include neurological, gastrointestinal and cardiopulmonary manifestations, vascular involvement, orchitis and epididymitis. Erythrocyte sedimentation rate is usually elevated. Urinary abnormalities are infrequent and transient. Positive pathergy test has been reported in 57.4% of patients in Iran, 44% in Japan, 57% in Turkey, 40% in Korea, 68% in Morocco and 32% in England. Lesions usually heal without sequela, except for lesions of eyes, brain and vascular system. The major cause of morbidity is the ocular lesion, which could lead to severe loss of vision or blindness.

Behçet’s disease (BD) is a multisystem disorder with potential ability to influence the pregnancy outcome and pregnancy is associated with several physiologic alterations which can lead to potential changes in the course of the disease. We studied 77 pregnancies in 69 women with BD. The disease activity was calculated by two methods for three periods of before, during and after pregnancy to evaluate changes induced by pregnancy. The pregnancy outcome and the newborns’ status were also evaluated. In 31 pregnancies (40.3%) no change was observed in the disease activity during pregnancy. The disease activity improved in 21 (27.3%) and aggravated in 25 (32.4%) pregnancies. After the delivery, the disease activity did not change in 31 patients (40.3%). It improved in 23 (29.85%) and aggravated in 23 patients (29.85%). We had full term delivery in 62 pregnancies (80.5%) and a failure in 15 cases (19.5%). Our results show that the effect of pregnancy on BD was not the same in all patients. The delivery had variable effects on the disease activity, with changes in 59.7% of cases. Comparison of the disease manifestations between patients with and without abortion showed no significant difference except for the peripheral joint and eye involvement which were significantly higher in patients with abortion. No neonatal BD was seen in our cases. The 19.5% failure rate of pregnancy must be a major concern when deciding for a new pregnancy in a patient with BD. It would be even more important in patients with eye and joint involvement.

Background –The ocular lesions of Behcet’s disease (BD) naturally progress toward severe loss of vision or blindness. Cytotoxic drugs are the main treatment. To the best of our knowledge, no controlled study has ever been performed to show their efficacy. This study was designed to evaluate the short-term efficacy of intravenous pulse cyclophosphamide (PCP). Methods – In a randomized double blind controlled crossover study, 35 consecutive patients meeting both the International and Classification Tree criteria for BD and suffering from active posterior uveitis and/or retinal vasculitis were randomly assigned to either PCP or placebo group. Both groups received prednisolone (0.5 mg/kg/day). PCP was administered as 1 g per square meter of the body surface once monthly to the PCP group and normal saline to the placebo group. After 3 months, the two groups were interchanged. Disease activity index (DAI) and visual acuity (VA) were calculated. The study was done at Behcet’s Unit, Rheumatology Research Center, Tehran University of Medical Sciences after approval of the Ethics Committee and patients’ consentResults – The mean VA improved from 3.7 to 4.9 (t = 3.309, p < 0.002) in the PCP group and from 4.4 to 4.5 (t = 0.317, p = 0.75) in the placebo group. The difference was significant (t = 2.402, p < 0.02). Other parameters improved more remarkably in the PCP group than in the placebo group, but differences were not statistically significant Conclusion – This study shows the efficacy of the combination of PCP and prednisolone over prednisolone alone.

Large vessel involvement is one of the hallmarks of Behcet’s disease (BD) but its prevalence varies widely due to ethnic variation or environmental factors. The aim of this study is to find the characteristics of vasculo-Behcet (VB) in Iran. In a cohort of 4769 patients with BD, those with vascular involvement were selected. Different manifestations of disease were compared with the remaining group of patients. A confidence interval at 95% (CI) was calculated for each item. Vascular involvement was seen in 409 cases (8.6%; CI, 0.8). Venous involvement was seen in 396 cases, deep vein thrombosis in 294 (6.2%; CI, 0.7), superficial phlebitis in 108 (2.3%; CI, 0.4) and large vein thrombosis in 45 (0.9%; CI, 0.3). Arterial involvement was seen in 28 patients (25 aneurysms and 4 thromboses). Thirteen patients showed both arterial and venous involvement. The mean age of the patients with VB was slightly higher (P<0.03), but the disease duration was significantly longer (P<0.0003). VB was more common in men. As the presenting sign, ocular lesions were less frequent in VB (P<0.0006), while skin lesions were over 2 times more common in these cases (P<0.000001). VB was associated with a higher frequency of genital aphthosis, skin involvement, joint manifestations, epididymitis, CNS lesions and GI involvement. The juvenile form was less common in VB (P<0.03). High ESR was more frequent in VB (P=0.000002), but the frequency of false positive VDRL, pathergy phenomenon, HLA-B5 or HLA-B27 showed no significant difference between the two groups. In Iranian patients with BD, vascular involvement is not common and large vessel involvement is rare. It may be sex-related, and is more common in well-established disease with multiple organ involvement and longer disease duration.

BACKGROUND—Iran is among the countries with rather high prevalence of Behcet''s disease (BD). We present here our latest data on different aspects of the disease. Materials AND METHODS—The present investigation is a prospective cohort study carried out on the data of patients presented in our BD registry during the past 28 years. The data were collected on a standard protocol comprising 100 items. These items included demographic features (such as sex, age of onset, age of diagnosis, date of the first visit, and ethnic origin), type of the presentation, different clinical manifestations of the disease, and paraclinical findings (including CBC and platelet count, Erythrocyte sedimentation rate, VDRL/RPR test, urinalysis, HLA typing, and Pathergy skin test). A confidence interval (CI) at 95% was calculated for each item. RESULTS—A total number of 5,059 patients were analyzed. The annual incidence rate was 280 patients in the last 5 years. The male/female ratio was 1.19/1 and the mean age at onset was 26 ± 9.8 (CI: 0.3). As the first manifestations, oral aphthosis (OA) was the most frequent (81%, CI: 1.1). The prevalence of variuos manifestations were OA: 97% (CI: 0.5), genital aphthosis: 65% (CI: 1.3), skin: 69% (CI: 1.3), ocular: 56% (CI: 1.4), joint: 34% (CI: 1.3), CNS: 3% (CI: 0.5), vascular: 8.5% (CI: 0.8), GI: 8% (CI: 0.8), and epididymitis: 10% (CI: 1.1). The laboratory findings were as follows: high ESR: 53% (CI: 1.4), urine abnormality: 10% (CI: 0.9), positive pathergy test: 57% (CI: 1.4), HLA B5: 52.5% (CI: 1.4), HLA B51: 34% (CI: 5.1), and HLA B27: 9% (CI: 0.8).CONCLUSION—Recent survey in Iran revealed a remarkable decrease of the incidence rate of BD and a tendency toward milder forms of the disease. Our data show more similarity with those of Turkey and Japan than with the western parts of the world.

The association of Behcet’s disease (BD) and ankylosing pondylitis (AS) is still a matter of debate. As the presence of sacroiliac joint (SIJ) involvement is an essential criterion in diagnosis of AS, we decided to determine the revalence of SIJ involvement in BD and compare it with that of a control group. Patients & We randomly selected two groups of 199 BD patients and 168 non-BD cases (the controls). All cases were over 20 years of age. Standard anteroposterior radiographs of the SIJ were obtained and interpreted by two rheumatologists and a radiologist blinded to the diagnosis. To determine the severity of the condition, the following 5-point scale was employed: Normal (0), pseudo-widening (1), sclerosis (2), erosion (3), and bony fusion (4). To eliminate any doubts, only grades 3 and 4 were considered as sacroiliitis. Both groups were separately evaluated for age (30, and >30), and gender. Results were compared using Chi square test. The groups were matched for age and sex: There were 98 (49.2%) females in BD vs. 91 (54.2%) in the control group (p=0.35). The meanSD age was 358.3 years in BD and 3510 in control group (p=1). The SIJ was involved in 9 (4.6%) patients in BD and 7 (4.2%) patients in control group (p=0.93). Comparisons between the results of the unisexual cohorts revealed no significance either (p=0.68 for males, and p=0.64 for females). The age subdivisions (under- and over-30) again showed no significant difference (p=0.96 and p=0.69 for under- and over-30 patients, respectively). The presence of radiographic signs of SIJ involvement is not mandatory for the diagnosis of AS.