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Objective Behçet's disease ( BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group ( ISG) clinical diagnostic criteria led to their reassessment. Methods An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, 'leave-one-country-out' cross-validation and clinical judgement were employed to develop new International Criteria for BD ( ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. Results For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4-95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9-92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD. [ABSTRACT FROM AUTHOR]

Background The classic treatment for pemphigus vulgaris is prednisolone. Immunosuppressive drugs can be used in association. Objective To compare the efficacy of Azathioprine in reducing the Disease Activity Index (DAI). Patients and methods A double blind randomized controlled study was conducted on 56 new patients, assigned to two therapeutic groups: (i) prednisolone plus placebo; (ii) prednisolone plus Azathioprine. Patients were checked regularly for 1 year. 'Complete remission' was defined as healing of all lesions after 12 months, and prednisolone <7.5 mg daily, (DAI ≤ 1). Analysis was done by 'Intention To Treat' (ITT) and 'Treatment Completed Analysis' (TCA). Results Both groups were similar in age, gender, disease duration, and DAI. Primary endpoint: By ITT and TCA, the mean DAI improved in both groups with no significant difference between them. The difference became significant for the last trimester (3 months; ITT: P = 0.033, TCA: P = 0.045). Secondary endpoint: The total steroid dose decreased significantly in both groups, with no significant difference between them, except for the last trimester (ITT: P = 0.011, TCA: P = 0.035). The mean daily steroid dose decreased gradually in both groups becoming statistically significant in favour of azathioprine, in the last trimester, especially at 12th months (ITT: P = 0.002, TCA: P = 0.005). Complete remission was significant at 12 months only for TCA (AZA/Control: 53.6%/39.9%, P = 0.043). Limitations Sample size was rather small to demonstrate all differences. Other limitations include the choice of primary and secondary endpoints and the unavailability to measure thiopurine methyltransferase activity. Conclusion Azathioprine helps to reduce prednisolone dose in long-run. [ABSTRACT FROM AUTHOR]

Background: Osteoarthritis is the most common form of arthritis in the world. This study presents the evidence on the prevalence of symptomatic hand osteoarthritis in urban community. To add to the evidence on the prevalence of symptomatic hand osteoarthritis in urban community. Methods: Inhabitants (age≥15 yrs) in 22 randomly selected districts (Tehran) participated in a Community-Oriented Program for Control of Rheumatic Diseases (COPCORD) evaluating major rheumatic disorders, including osteoarthritis. Eventually, 10, 291 inhabitants completed a Questionnaire (75% response-rate). Trained interviewers asked participants whether they had had any pain, swelling, tenderness, or morning stiffness in the right/ left hand during previous seven days. Participants underwent a complete physical examination if they had any musculoskeletal complaint or extraarticular manifestation of rheumatic disease. Osteoarthritis was defined as presence of palpable nodules (Heberden's/ Bouchard's nodes, CMC1's squaring), pain, tenderness, swelling, or a combination of them on that joint (DIP-PIP-MCP-CMC1). Clinical hand osteoarthritis was positive if at least one joint showed osteoarthritis. Results: Symptomatic hand osteoarthritis was present in 2.8%(CI; 2.3-3.4) (52.6% female, mean age; 37.1±16.3). Prevalence was higher in females (4.3% vs. 1.3%, p=0.000) and increased with age (0.1% in people <30 versus 23% in people >70, p=0.000). The most common signs in the DIP, PIP and CMC1 joints were bony enlargement, followed by tenderness and pain on movement. Conclusions: our study confirms the evidence of high prevalence of symptomatic hand osteoarthritis in an urban community. The prevalence, pattern of hand joints involvement and relationship with age and sex in this study performed in an Eastern community resemble those in Westerners, which calls for further attention by appropriate services. [ABSTRACT FROM AUTHOR]

Behçet’s disease (BD) is a chronic inflammatory disease characterized by oral aphthous ulcers, genital ulcers, uveitis and skin lesions. Etiology and pathogenesis of BD are not fully elucidated, but the association with human leukocyte antigen (HLA)-B51 or B*5101 has been repeatedly reported. Previous studies have shown that there are few sequence variations in the protein-coding region of B51, while there is a report on many variations in the 5′-flanking region and intron. In this study, HLA-B*5101 gene from 37 individuals including Japanese, Turkish, Jordanian and Iranian patients and healthy controls were fully sequenced to further clarify the B*5101 gene in association with BD. We found that all the patients and healthy controls carried B*510101 with no variation in the 5′-flanking region, exon and intron. However, seven polymorphisms were found in the 3′-flanking region. These polymorphisms composed of six haplotypes that were shared and stretched over the ethnic groups, suggesting that the susceptibility to BD was conferred by the B*510101 itself and not by any genes in linkage disequilibrium with B*510101. In addition, phylogenetic analyses of B*510101 showed that the 3′-flanking sequences followed an evolutional divergence differently from that of the other regions, implying that a unifying selection might operate to conserve B*510101. [ABSTRACT FROM AUTHOR]

Objective: There are some clues that measurement of triglyceride (TG) and high-density lipoprotein (HDL) and their correlation with tumor necrosis factor alpha (TNF-α), soluble TNF-α receptor type 1 and type 2 (sTNFR1, sTNFR2) in serum could be valuable in the assessment of disease activity of systemic lupus erythematosus (SLE) patients. Methods: In this cross-sectional study, fasting blood samples were obtained from 86 SLE patients referred to the Rheumatology Research Center of Tehran University in Shariati Hospital, Iran. Disease activity was determined by using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). TG and HDL obtained after overnight fasting were analysed by routine chemistry. Levels of circulating TNF-α, sTNFR1, and sTNFR2 were determined by enzyme-linked immunosorbent assay. Results: Triglyceride levels were associated with SLEDAI ( r = 0.59, P < 0.001), TNF-α ( r = 0.27, P < 0.01), and with sTNFR1 ( r = 0.54, P < 0.001); on the contrary, HDL levels were negatively associated with SLEDAI ( r = –0.29, P < 0.007), TNF-α ( r = –0.27, P < 0.01), and sTNFR1 ( r = –0.35, P < 0.001). The correlation of TG and HDL with sTNFR2 were ( r = 0.13, P > 0.23) and ( r = –0.17, P > 0.1), respectively. In multiple logistic regression models, the levels of TNF-α and HDL were omitted. Conclusion: Dyslipoproteinemia with high TG/low HDL levels correlates with disease activity in SLE, and enhanced activity in the TNF-α/sTNFR system. With results of this study and the same studies, serum levels of TG, HDL, TNF-α, sTNFR1, sTNFR2 are valuable markers for estimation of disease activity in SLE. [ABSTRACT FROM AUTHOR]

Abstract: Behcet''s disease (BD) is a vasculitis progressing by attacks and remissions. There are actually four nationwide surveys of BD; Iran (5059 patients), Japan (3316 patients), China (1996 patients), and Germany (590 patients). There are four major case series in the world, Turkey (2147 patients), Korea (1155 patients), Morocco (1034 patients), and UK (419 patients). Other series are based on less than 200 patients. Mucous membrane manifestations: Oral aphthosis is seen in 98% of patients (average from the above-mentioned countries). Genital aphthosis occurs less frequently (73%). Skin manifestations are seen in 74% of patients. Behcet''s pustulosis (pseudofolliculitis) is seen in 61% of patients in Iran. Erythema nodosum is seen in 22% of patients in Iran. They are more frequent in China and Korea. Ocular manifestations are seen in 51% of patients (anterior uveitis, posterior uveitis retinal vasculitis). Joint manifestations are seen in 39% of patients (arthralgia, monoarthritis, oligo/polyarthritis, ankylosing spondylitis). Neurological manifestations are seen in 7.3% of patients. They are mainly central manifestations due to parenchymal lesions. Gastrointestinal (GI) manifestations are seen in 9% of patients (produced by aphthous ulcers of the intestinal tract). Vascular involvement occurs in 11% of patients. Arterial involvement is rare. In Iran it is seen in 0.6% of patients. Deep vein thrombosis is seen in 6%, large vein thrombosis in 1.2%, and superficial phlebitis in 2.3%. Other lesions: Orchitis and epididymitis were seen in 5.6% of patients in Iran. Cardio-pulmonary manifestations were rare, seen in only 1.4% of patients in Iran. Laboratory findings: Erythrocyte sedimentation rate was normal in 43.5% of cases in Iran. Urinary abnormalities were infrequent and transient (9.8% in Iran). Proteinuria was seen in 2%, hematuria in 4.6%, leukocyturia in 5.1%, and cast in 0.3% of cases. Pathergy test was positive in 57.4% and HLA-B51 in 34% of patients. Conclusion: BD is a systemic disease with various manifestations. It progresses by attacks and remissions. Lesions usually heal without sequela, except for eyes, brain, and vascular system. The main manifestations are mucocutaneous. The major cause of morbidity is the ocular lesion, which leads to severe loss of vision or blindness. [Copyright &y& Elsevier]

Background: Bone mineral density (BMD) of the spine and the femoral neck are accurate indicators of the bone mass and thus useful predictors of fracture risk. Dual energy X-ray absorptiometry (DEXA) is the easiest, yet the most precise and non-invasive technique. The need for a population-specific normative BMD data is vital in preventing mislabelling or misdiagnosis of osteopenia or even osteoporosis. Aim: This study was performed to determine the lumbar vertebral and the proximal femur BMD measured by DEXA in 280 normal Iranian men and women. Method: Subjects were selected randomly from different social economic classes in Tehran. Normal subjects were selected for each decade and both sexes. BMD was measured with a Hologic QDR 1000+ densitometer, for the lumbar spine (L1, L2, L3, L4, L1–L4) and the femoral neck (neck, trochanter, intertrochanter, ward triangle, total). Data were treated by polynomial approximation (3rd degree). Results: In women, the highest BMD recorded was 1.020 g/cm2 for the lumbar spine (mean L1–L4) at the age of 31 years, and 0.832 for the femoral neck at the age of 34. In men, the highest BMD recorded was 0.987 g/cm2 for the lumbar spine (mean L1–L4) at the age of 36, and 0.907 for the femoral neck at the age of 30. The highest BMD in spine was lower in men than women. Conclusion: The BMD of both lumbar spine and femoral neck (in both sexes) was lower in this study than the Hologic standards. [ABSTRACT FROM AUTHOR]

Chondromalacia patella, which is characterized by softening of the patellar cartilage, is the most common cause of anterior knee pain in young women. The aim of this study was to identify the clinical features of patients with chondromalacia patella in Iran. All patients under 40 years, complaining of mechanical knee pain who were referred to Amir A’lam Rheumatology Unit, with positive shrug sign and normal knee X-rays during the period September 2000 to September 2002, were included in this study. After physical examination and knee radiography, patients with knee arthritis, knee osteoarthritis and knee periarthritis were excluded. Patients with the clinical diagnosis of chondromalacia patella were studied. The demographic data, clinical disease characteristics and disease course were recorded. There were 260 patients. They were predominantly female (F : M, 2.6 : 1), in the third decade of life and a mean age of 22.8 years at the onset of disease. Bilateral involvement was found in 92% of patients. The first manifestation was knee pain. The history of trauma or swelling of the knee occurred in about 20% of cases. The history of dislocation was 3%. Sitting with flexed knees, stairs, and the use of Turkish WCs aggravated the knee pain. About one-third had knee malalignment, mostly mild genu varus. Patella alta was seen in 1.6%. Q-angle more than 15° was seen in 90.8%. Mean Q-angle was 21.9°, mean patellar angle was 122.6°, and mean intercondylar angle was 141.5°. All patients had the shrug sign. About 90% had Rabot test and crepitation, 3.5% had knee effusion, and 1% had knee laxity. Lower limb discrepancy was seen in 6.2% and spinal deformity in 10%. Ninety-three percent of the patients were treated by conservative (medical) therapy. So the classic case of chondromalacia patella is a woman in her third decade of life with mechanical knee pain and positive shrug test. [ABSTRACT FROM AUTHOR]

Aim To study the effect of acetazolamide on cystoid macular oedema (CMO) in patients with Behcet's disease.Patients and methods A total of 67 eyes of 35 Behcet's patients with chronic, but well-controlled uveitis, and CMO were randomised into a double-masked, crossover trial comparing the effect of acetazolamide vs placebo. The patients received an initial 4-week course of either 250?mg acetazolamide twice daily (b.i.d.) or placebo, followed by a 4-week washout period. They then received a 4-week course of the reverse study medication. An improvement in visual acuity and fundus fluorescein angiographic findings was assessed.Results In total, 29 patients (55 eyes) completed the trial and were available for analysis. Of the 29, 16 men and 13 were women. The age range was 13-50 years (mean 33.6 years). Patients on acetazolamide showed a slightly better improvement of angiographic signs (at least by one grade improvement) over that of placebo (12 vs five eyes). They also had less deterioration of angiographic signs over that of placebo (three vs seven eyes). However, these findings were not statistically significant (P=0.99). Acetazolamide had no statistically significant effect (P=0.53) on the improvement of visual acuity of patients over that of placebo (13 vs eight eyes), nor on the deterioration of visual acuity (three vs 11 eyes).Conclusion Despite seemingly favourable results, the 4-week course of acetazolamide (250?mg b.i.d.) has no statistically significant effect on the improvement of the visual acuity and the fluorescein angiographic findings in Behcet's patients with CMO.Eye (2003) 17, 762-766. doi:10.1038/sj.eye.6700464 [ABSTRACT FROM AUTHOR]

We have previously suggested that in a Japanese population the susceptible locus for Behçet's disease (BD) is HLA-B51 itself. To confirm this finding in another population, we performed HLA class I typing using the PCR-SSP method and analyzed eight polymorphic markers distributed within 1100 kb around the HLA-B gene using automated sequencer and subsequent automated fragment detection by fluorescent-based technology with the DNA samples of 84 Iranian patients with BD and 87 healthy ethnically matched controls. As a result, three microsatellite alleles (MICA-A6, MIB-348, C1-4-1-217) and HLA-B51 were found to be strongly associated with BD. Of these alleles HLA-B51 is the most strongly associated allele. There were no alleles that were increased in allele frequency at any microsatellite loci centromeric of MICA or telomeric of HLA-B51. Therefore, HLA-B51 was confirmed to be by far the most strongly associated gene with BD in an Iranian population. [ABSTRACT FROM AUTHOR]

Abstract: It is well known that Behçet’s disease (BD) is strongly associated with human leukocyte antigen (HLA) B51 in many ethnic groups. However, there has been no published report as yet with respect to this association among the Iranian people. Furthermore, since it is now known that the B51 antigen can be encoded by 21 alleles, B*5101-B*5121, we performed HLA-B*51 allele typing as well as HLA class I genotyping of 48 Iranian patients with this disease. As a result, the frequency of the B*51 allele was significantly higher (62.1%) in the patient group as compared with the ethnically matched control group (31.8%) (Pc=0.067, R.R.=3.51). In the genotyping of B*51 alleles, 33 out of the 36 B*51-positive patients possessed B*5101 and the remaining 3 carried B*5108. This study revealed that Iranian patients with BD also had a strong association with HLA-B51. In addition, this significantly high incidence of HLA-B*51 was found to be caused by an increase in both the HLA-B*5101 and HLA-B*5108 alleles. However, there was no significant difference in the HLA-B*51 allelic distribution between the patient and control groups. [ABSTRACT FROM AUTHOR]

No fewer than five sets of diagnostic criteria for Behçet's disease are currently in use. The International Study Group have therefore undertaken an extensive study of existing criteria and have developed recommended new international criteria based on the collection of data from 914 patients with Behçet's disease and 308 controls, of whom 886 (97%) and 97 respectively had mouth ulceration. It is recommended that these new criteria should replace the existing criteria sets although further studies are required to evaluate their performance in new patient groups. [ABSTRACT FROM PUBLISHER]

Hereditary sensory neuropathy type I (HSN-I) is a rare genetic disorder that usually begins in childhood, predominantly affecting the peripheral nerves of the lower limbs, and occasionally also those of the upper extremities. Foot deformities such as pes cavus, pes planus, sensory loss, painless injuries or burns, recurrent foot ulceration resulting in osteonecrosis and osteolysis subsequently necessitate amputations. Herein, we describe a 45-year-old man with progressive foot ulceration and self-amputation during previous 20 years. The diagnosis of HSN was made because of a typical clinical pattern and similar occurrence in his brother. Laboratory investigations showed peripheral sensory neuropathy and a high erythrocyte sedimentation rate. [ABSTRACT FROM AUTHOR]

Behçet disease is a multisystem vasculitis characterized by mucosal aphthosis, primarily in the oral and genital mucosa. Only a few cases of involvement of the nasal mucosa have been reported in the literature, and its true prevalence is not known. We conducted a cross-sectional study of 400 consecutively presenting patients with confirmed Behçet disease (according to classification-tree criteria) to determine the incidence of nasal mucosal involvement, to identify its particular characteristics, and to determine if there are any statistically significant differences in clinical and paraclinical parameters between Behçet disease patients with nasal involvement and those without. To the best of our knowledge, this is the first systematic evaluation of nasal involvement in patients with Behçet disease to be published in the literature. Data analysis was performed with Statistical Package for the Social Sciences software (SPSS), and a confidence interval (CI) at 95% was calculated for each finding. Involvement of the nasal mucosa was seen in 31 of the 400 patients (7.8%; CI: 5.1 to 10.5). Among those 31 patients, the most common nasal symptom was dysosmia, which was seen in 15 patients (3.8% of the total population; CI: 1.9 to 5.7); other nasal symptoms included obstruction in 10 patients (unilateral in 9), ulcers in 2, pain in 2, and a burning sensation and discharge in 1 each. No patient reported a history of epistaxis or nasal itching. Abnormal signs were present in only 16 patients (4.0%; CI: 2.1 to 5.9); they included nasal cartilage deformity in 6, unilateral nasal obstruction in 4, postnasal discharge in 3, nonaphthous ulcer in 3, and crusted ulcer in 2 (2 patients each had 2 abnormal signs). No case of aphthous ulcer, nasal discharge, nasal scar or deformity, septal perforation, or nodular or granulomatous cartilage lesion was found. There were no statistically significant differences in clinical and paraclinical disease manifestations between those patients who had nasal mucosal involvement and those who did not. [ABSTRACT FROM AUTHOR]