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Lupus erythematosus (LE) is an autoimmune disease. The mortality rate and causes of death are different in various countries. The present study is done to find out the causes of mortality in the systemic lupus erythematosus (SLE) patients in lupus unit of Rheumatology Research Center and other wards of Shariati Hospital. This study was retrospective according to medical records of patients who were referred to Lupus Unit of Rheumatology Research center, ICU, Gynecology and Nephrology wards of Shariati hospital during 10 years, from 1991 till 2001. Of 2021-recorded patient’s files, 165 (7.8%) deaths were already recorded. 84.8% were female and 15.2% male. The duration of disease was 36±12 months. The mean duration of follow up from entering the study till time of death was 25.9±16.8 months. The causes of mortality were as follows: infection (12.1%), respiratory (8.5%), CNS (13.9%), kidney (3.6%), malignancy (1.2%), unknown (7.8%), active lupus (3%). Infection accompanied with other causes (27.9%) , respiratory with other causes (11.5%), CNS with other causes (5.5%) and renal with other causes (4.8%). Infections are the most common cause of death in SLE Iranian patients, which is compatible with some multicentric studies. But in some previous studies myocardial infarction was reported as the main cause of death.

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. [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]

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]

Presents a new set of internationally agreed diagnostic criteria for Behcet's disease. Methods; Results; Discussion.

At present, some clinical presentations and serological parameters such as products of complement activation and elevation of autoantibodies (e.g. dsDNA antibodies), erythrocyte sedimentation rate (ESR), levels of C-reactive protein (CRP), or cytokines such as IL-2/IL-2 receptor, IL-6 and IL-10 are used as indirect serological markers with variable degrees of significance. To date, no specific serological parameter is available to assess disease activity in SLE. Soluble serum thrombomodulin is a new marker of endothelial cell injury and vasculitis. The objective of this study was to determine in vivo soluble thrombomodulin as a marker of disease activity in SLE pa-tients and compare serum thrombomodulin in SEL patients with and without renal involvement and inactive SLE patients. Sixty-four patients fulfilling ACR criteria with proven SLE with differ-ent disease activities were tested for serum levels of Thrombomodulin and dsDNA by ELISA. C3, C4 and FANA were also measured by standard laboratory tests. The clinical disease activity was evaluated by the Systemic Lupus Erythematosus Activity Index (SLEDAI). Elevated soluble thrombomodulin had significant correlations with an increased ANA level (P= 0.037), decrease level of C3 (P =0.017), increase of SLEDAI (P=0.003) and strongly associated with renal in-volvement in SLE. Thrombomodulin level and Anti dsDNA in active patients with and without re-nal disease were higher than inactive patients. In SLE, serum thrombomodulin seems to be impor-tant marker for evaluation of disease activity.

Isolated hematuria and its association with proteinuria, and urinary cast and systemic lupus erythematosus (SLE) disease activity, and decision for renal biopsy is a dilemma for physician in SLE patients. The aim of this study was to investigate 1. whether isolated hematuria is associated with active SLE, 2. to determine duration between hematuria and proteinuria and urinary cast, and 3. to determine renal histological type in SLE patients with isolated hematuria. All episodes of isolated hematuria between 1981 and 1997 were identified from Lupus Unit, Rheumatology Research Center database. Isolated hematuria was defined as >5 RBC/hpf in the absence of urinary infection and other renal manifestations. Relation of hematuria was assessed with proteinuria and urinary cast and SLE disease activity. Needle renal biopsy was done in 19 SLE patents with isolated hematuria. 4.42% (31/700) of our cohort had at least one episode of isolated hematuria. Out of 31 patients in whom the isolated hematuria was the first documented renal manifestation, 11 patients (35.48%) developed another renal manifestation (25.8% proteinuria and 9.67% casts). 54.54% (6/11) of patients developed proteinuria and urinary cast within 3 months. The mean time for development of a second renal manifestation for the patients with isolated hematuria was 19.9 months. Renal needle biopsy was performed for 19 patients (5.78% type IV, 63.15% type III and 21.50% type II). The results of the present study indicate that isolated hematuria is not rare in SLE patients. Also, there was no significant relationship between isolated hematuria and anti ds-DNA, C3, C4 and major organ involvement in patients with SLE. Our study suggests that SLE patients who have isolated hematuria should undergo renal biopsy and that isolated hematuria should be considered a manifestation of active renal SLE.

Abstract Background Gluten sensitive enteropathy (GSE) is an autoimmune enteropathy triggered by the ingestion of gluten-containing grains in susceptible individuals. Recurrent aphthous stomatitis (RAS) may be the sole manifestation of GSE. The aim of this study was to determine the prevalence of gluten sensitivity enteropathy (GSE) in a large group of patients with RAS and assess the efficacy of gluten free diet (GFD) on the improvement of aphthous lesions in those who were diagnosed with GSE. Methods Two hundred and forty seven patients with RAS were included. The patients had at least three aphthous attacks per year. Patients were screened by IgA anti-endomysial antibody (EMA), IgA anti tissue transglutaminase (TTG) and serum IgA level. Those with a positive serology underwent endoscopic biopsies of the duodenal mucosa and patients with negative serology were excluded. The diagnosis of GSE was based on a positive serological test and abnormal duodenal histology. For patients with GSE, gluten free diet was recommended. Results Six out of 247 RAS patients had positive TTG test alone, and one had positive EMA and TTG. All 7 patients with positive serologic tests underwent duodenal biopsies. Histological findings were compatible with GSE in all of them (Marsh I in four patients, Marsh II in two patients and Marsh IIIB in one another.). The mean age of GSE patients was 27.42 ± 10.56 (range, 13 to 40) years old. They were suffering from RAS for an average duration of 4.5 years. All of the 7 GSE patients had not responded to the routine anti-aphthae medications, including topical corticosteroids, tetracycline and colchicine. Four patients who adhered to a strict gluten-free diet showed noticeable improvement in their aphthous lesions over a period of 6 months. Conclusion A significant minority (e.g. 2.83%) of RAS patients have GSE. This could be compared with the 0.9% prevalence of GSE in the general population of Iran. This study suggests that evaluation for celiac disease is appropriate in patients with RAS. Additionally, the unresponsiveness to conventional anti-aphthae treatment could be an additional risk indicator.

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]