Scleroderma-overlap syndromes: capillaroscopy, laboratory, and clinical manifestations and follow-up compared to scleroderma patients.

Introduction: Overlap syndrome (OS) is a group of systemic connective tissue diseases (CTDs) that meet the criteria of two or more CTDs. In this study, we evaluated clinical, laboratory, and capillaroscopic manifestations of patients with scleroderma OS (SSc-OS) and its subgroups and follow-up progression compared to patients with limited SSc (LcSSc). Material and methods: In a 10-year cross-sectional study, we evaluated 135 adult patients (70 with SSc-OS and 65 with LcSSc) with the same skin score for their baseline and follow-up clinical, laboratory, high-resolution chest tomography (HRCT), echocardiography, and nailfold capillaroscopy data and compared them. Results: Of the 135 patients, 70 had SSc-OS, including 45 (64.3%) cases of SSc-SS (Sjögren's syndrome), 11 (15.7%) of SSc-RA (rheumatoid arthritis), 9 (12.9%) of SSc-myositis and 5 (1.7%) of SSc-SLE (systemic lupus erythematosus), and 65 had LcSSc. Lung and heart involvement and pulmonary arterial hypertension (PAH) did not differ between the two groups (p > 0.05). Musculoskeletal involvement and non-specific pattern of capillaroscopy were higher (p = 0.035 and p = 0.001), and digital ulcer (DU) and scleroderma patterns of capillaroscopy were lower in the SSc-OS group (p = 0.000).No significant relationship was found between capillaroscopic patterns and organ involvement in the two groups (p-value > 0.05). In the follow-up (3.71 ±2.63 years), new DU and progression of lung involvement (p = 0.002) and the progression in capillaroscopic patterns was lower in SSc-OS (p = 0.000). In the follow-up, new DU was not seen in the SSc-OS, with lower progression of lung involvement, skin score, and capillary damage. Conclusions: In SSc-OS patients, the most common subgroup was SSc-SS. Scleroderma OS was associated with lower major organ involvement and capillaroscopy progression than LcSSc. Major organ involvement in patients with SSc-OS was significantly lower than in LcSSc patients. In the follow-up, new DU was not seen in the SSc-OS with lower progression of lung involvement, skin score, and capillary damage.

The effect of flaxseed with or without anti-inflammatory diet in patients with rheumatoid arthritis, a randomized controlled trial.

PURPOSE: Beneficial effect of long-chain ω-3 fatty acids against symptoms of rheumatoid arthritis (RA) has been indicated in previous studies. We examined the effect of flaxseed and anti-inflammatory diet in patients with RA. METHODS: The 12-week intervention was performed on 120 patients with RA who were randomized to three groups of flaxseed (30 g/day) plus anti-inflammatory diet (AIF group), flaxseed (30 g/day) plus regular diet (RF group), and roasted wheat (30 g/day) plus regular diet (RW group). Disease Activity Score 28-joints (DAS28), health assessment questionnaire (HAQ) disability and pain, quality of life, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor, and anti-cyclic citrullinated peptides (anti-CCP) were measured before and after trial. Analysis was performed using per-protocol and intention-to-treat (ITT) approaches. RESULTS: One hundred and two patients completed the protocol. Flaxseed decreased DAS28 in RF group compared to RW (- 0.87 ± 1.11 vs. - 0.24 ± 0.78; P = 0.014). Pain severity (P ≤ 0.001), morning stiffness (P < 0.05), and disease feeling (P < 0.01) decreased significantly in AIF and RF groups. HAQ disability and quality of life measurements improved in all 3 groups, with a greater extent in AIF and RF groups (P < 0.001) compared to RW. Between-group differences were significant for DAS28, pain scores, and physical and mental health variables. ESR, CRP, anti-CCP, and rheumatoid factor were not different between groups. Results of ITT analysis did not cause much difference. CONCLUSIONS: In conclusion, flaxseed may be used as a helpful adjuvant therapy for patients with RA. Calls are open for examining the effect of anti-inflammatory diet on RA symptoms. TRIAL REGISTRATION NUMBER: Registered at irct.ir as IRCT20190923044858N1, February 6, 2020.

Adherence to the dietary approaches to stop hypertension dietary pattern and rheumatoid arthritis in Iranian adults.

OBJECTIVE: To examine the hypothesis that rheumatoid arthritis (RA) patients are less likely than healthy individuals to adhere to the dietary approaches to stop hypertension (DASH) dietary pattern. DESIGN: A multi-centre cross-sectional study involving a total of 300 eligible Iranian adults (aged >19 years; 93·0 % female) recruited during 2019-2020. Participants' actual dietary intakes were measured via self-administered 3-d dietary records. The DASH score was computed based on the energy-adjusted intakes of eight major dietary components usually emphasised (i.e. fruits, vegetables, nuts and legumes, low-fat dairy products and whole grains) or minimised (i.e. sweets, red or processed meats and sodium) in the DASH diet. The higher the DASH score of subjects, the greater their adherence to the DASH pattern. SETTING: The outpatient clinics of major general hospitals in Shiraz, Iran. PARTICIPANTS: 100 incident cases with definite RA according to the 2010 American College of Rheumatology/European League Against Rheumatism Classification Criteria for RA and 200 apparently healthy controls frequency-matched by gender and age. RESULTS: After adjusting for several potential covariates in the binary logistic regression analysis, RA cases were less likely than controls to have high adherence to the DASH pattern (OR = 0·08; 95 % CI 0·03, 0·20; P = 0·001). CONCLUSIONS: Our findings in a sample of Iranian adults revealed that RA patients are less likely than healthy individuals to adhere to the DASH dietary pattern. However, the potential causal association of greater adherence to the DASH pattern and lower risk of RA needs to be confirmed by prospective studies of high methodological quality.

Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease affecting the skin and multiple organs. Most of the evidence on ocular involvement comes from small studies. We evaluated the retinal vascular changes in patients with SSc and its associations with types, disease duration, skin score, and nailfold capillaroscopy changes. MATERIAL AND METHODS: We evaluated the demographic, clinical and nailfold capillaroscopy data of 52 patients referring to the clinic of scleroderma with SSc according to the 2013 ACR/EULAR SSc criteria. In addition, full ophthalmic examination in 52 patients and fundus photography in 40 patients were done in the ophthalmology clinic. RESULTS: There were 52 (49 women and 3 men, 17 diffuse and 35 limited) patients with SSc with mean disease duration of 8 ±5 years and mean age of 40 ±9 years. Retinal changes in the ophthalmologic examination were seen in 30.7% as increased vascular tortuosity. None of the patients had cotton wool spot, hemorrhage or hard exudate. Forty patients underwent fundus photography and 22.5% of them had vascular tortuosity. Only the presence of hemorrhage in the nailfolds was correlated with retinal tortuosity, and the other characteristics of nailfold capillaroscopy did not have any association with retinal changes. CONCLUSIONS: Retinal vascular changes were seen in about one third of our patients. There was no correlation between nailfold capillaroscopy, disease duration, type and skin score of SSc patients and retinal vascular changes. These findings suggest that the mechanisms or the quality of changes in the retinal vessels and nailfold vasculature may be different.

First report of HHV-8 viral load and seroprevalence of major blood-borne viruses in Iranian patients with systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is characterized by autoimmune manifestations, and viral infections may have a key role in the development and progression of it. This study aimed to investigate the seroprevalence of major blood-borne viruses and HHV-8 viral load in Iranian SSc patients. METHODS: In this cross-sectional study, 90 patients with a confirmed history of SSc and 90 healthy blood donors were enrolled. The frequency of HHV-8, CMV, EBV, HIV, HBV, and HCV antibodies and HHV-8 viral load were evaluated by enzyme-linked immunosorbent assay and real-time PCR assay, respectively. RESULTS: HHV-8 IgG antibody was diagnosed in 61 (67.8%) patients and 3 (3.3%) healthy individuals (p<0.0001), but its genomic DNA was not detected in the patients or healthy blood donors. CMV and EBV antibodies were detected in 100% and 88.9% of SSc patients without any significant difference with healthy population (p>0.05). None of the patients or healthy population was positive for HBsAg and HIVAb; however, HCVAb was detected in two patients. CONCLUSION: According to the results, HHV-8 antibody was uniquely increased in SSc population while its frequency in healthy population was very low. Since none of the SSc patients were positive for HHV-8 genomic DNA, the high prevalence of HHV-8 antibody in this group was not related to the real history of infection. Therefore, antibody-mediated epitope mimicry can play a role to get the high rate of seropositivity and lead to pathogeneses of SSc. Besides, CMV and EBV viral load monitoring in SSc patients can help the physician to prescribe the viral drugs to suppress the viral replication and avoid the crucial effect of reactivation.

Expression Pattern of Telomerase Reverse Transcriptase (hTERT) Variants and Bcl-2 in Peripheral Lymphocytes of Systemic Lupus Erythematosus Patients.

BACKGROUND & OBJECTIVE: It is not clear whether activated lymphocytes of patients with systemic lupus erythematosus (SLE) are more proliferative or less apoptotic. We aimed to delineate potential differences between B and T cells of SLE patients compared to healthy controls regarding the telomerase activity and apoptosis status. METHODS: In this cross-sectional case control study, Blood samples were taken from 10 SLE patients and 10 healthy controls. B and T cells were separated using magnetic cell sorting system. Telomeric repeat amplification protocol (TRAP) assay and real-time PCR were used to determine the telomerase activity and the expression of alternatively spliced variants. RESULTS: Four patients under treatment showed significant telomerase activity in their T cells. Four of the newly diagnosed patients showed telomerase activity in their B cells (20% of all patients and 40% of new onset patients). There was no specific pattern of human telomerase reverse transcriptase variant expression within the patients' lymphocytes. A significantly reduced expression of Bcl-2 was detected in B cells (P=0.018) and a trend toward lower Bcl-2 expression in T cells was seen in SLE patients compared to healthy controls. CONCLUSION: Although not definitive, our results may suggest that B cells may have more active roles during the earlier phases of the disease attack, while T cells take over when the disease reaches its chronic stages.

The Effect of Educational Program on Self-efficacy of Women with Rheumatoid Arthritis: A Randomized Controlled Clinical Trial.

BACKGROUND: Rheumatoid Arthritis (RA) is a chronic and inflammatory disorder which is the major cause of disability in adults. Patient training is a vital aspect of nursing care for people with arthritis aiming to enable them to live as independently as possible. In the present study, we aimed to evaluate the effect of the educational program on self-efficacy on women with rheumatoid arthritis. METHODS: This study was an open randomized controlled clinical trial during May to July 2013. Women (64 participants) with confirmed RA and a mean±SD age of 48.6±9.83 years were enrolled; they were randomly assigned into intervention and control groups (32 in each group). The patients in the intervention group participated in a training programs consisting of two 30-minute sessions per week for 8 consecutive weeks. Data were collected by using Arthritis Self-Efficacy Scale (ASES) and demographic questionnaire. ASES questionnaire was filled in three times by the participants: before, immediately after, and three months after the intervention. Data were analyzed using SPSS software, version 20, and statistical tests including Chi-square and t-test. RESULTS: The results showed that the mean of self-efficacy scores of the intervention group, immediately and three months after the intervention, significantly enhanced in all dimensions compared with the control group (P<0.001, P<0.001). CONCLUSION: It can be concluded that training programs is effective for improving self-efficacy in the patients suffering from RA by raising their awareness towards their disease and methods of adaptation with it. But as to the sustainability of the impact of educational courses on self-efficacy, it seems that more research is required. Trial Registration Number: IRCT201308187531N3.

A phase III, randomized, two-armed, double-blind, parallel, active controlled, and non-inferiority clinical trial to compare efficacy and safety of biosimilar adalimumab (CinnoRA®) to the reference product (Humira®) in patients with active rheumatoid arthritis.

BACKGROUND: This study aimed to compare efficacy and safety of test-adalimumab (CinnoRA®, CinnaGen, Iran) to the innovator product (Humira®, AbbVie, USA) in adult patients with active rheumatoid arthritis (RA). METHODS: In this randomized, double-blind, active-controlled, non-inferiority trial, a total of 136 patients with active RA were randomized to receive 40 mg subcutaneous injections of either CinnoRA® or Humira® every other week, while receiving methotrexate (15 mg/week), folic acid (1 mg/day), and prednisolone (7.5 mg/day) over a period of 24 weeks. Physical examinations, vital sign evaluations, and laboratory tests were conducted in patients at baseline and at 12-week and 24-week visits. The primary endpoint in this study was the proportion of patients achieving moderate and good disease activity score in 28 joints-erythrocyte sedimentation rate (DAS28-ESR)-based European League Against Rheumatism (EULAR) response. The secondary endpoints were the proportion of patients achieving American College of Rheumatology (ACR) criteria for 20% (ACR20), 50% (ACR50), and 70% (ACR70) responses along with the disability index of health assessment questionnaire (HAQ), and safety. RESULTS: Patients who were randomized to CinnoRA® or Humira® arms had comparable demographic information, laboratory results, and disease characteristics at baseline. The proportion of patients achieving good and moderate EULAR responses in the CinnoRA® group was non-inferior to the Humira® group at 12 and 24 weeks based on both intention-to-treat (ITT) and per-protocol (PP) populations (all p values >0.05). No significant difference was noted in the proportion of patients attaining ACR20, ACR50, and ACR70 responses in the CinnoRA® and Humira® groups (all p values >0.05). Further, the difference in HAQ scores and safety outcome measures between treatment arms was not statistically significant. CONCLUSION: CinnoRA® was shown to be non-inferior to Humira® in terms of efficacy at week 24 with a comparable safety profile to the reference product. TRIAL REGISTRATION: IRCT.ir, IRCT2015030321315N1 . Registered on 5 April 2015.

Acquired Hemophilia as Initial Presentation in a Patient with Systemic Lupus Erythematosus.

BACKGROUND: Acquired hemophilia is a rare bleeding disease which might be associated with some autoimmune diseases. Acquired hemophilia may be the result of autoantibodies against factor VIII. CASE PRESENTATION: In this study, we describe a 55 year old patient who developed hematoma and hematuria due to acquired hemophilia secondary to systemic lupus erythematosus (SLE). Then, she developed arthritis and thrombosis after some evaluations. Laboratory data showed prolonged aPTT, normal PT and platelet, low FVIII, high FVIII inhibitor, ANA, anti-ds-DNA, and anticardiolipin. This patient was treated with corticosteroids, FEIBA and rituximab, so she recovered and was discharged about seven days later. CONCLUSION: We should consider lupus erythematosus in patients with acquired hemophilia. Some cases of acquired hemophilia may be an SLE manifestation, so we should consider this situation to diagnose the disease properly.

Nailfold digital capillaroscopic findings in patients with diffuse and limited cutaneous systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a chronic disease with microvascular damage. Nailfold capillaroscopy is a non-invasive method used for evaluating capillaries in SSc. Its findings could be related to the internal organ involvement and SSc course. In this study, we aimed to determine the association of the capillaroscopic patterns of nailfold capillaries with the disease subtypes of SSc, disease duration, and clinical manifestations. MATERIAL AND METHODS: Seventy patients with SSc (15 cases with diffuse cutaneous SSc [DcSSc] and 55 patients with limited SSc [LcSSc]) were studied. The patients were classified into early and intermediate/late DcSSc and LcSSc regarding their disease duration. The capillaroscopy findings were classified into normal, 'early', 'active' and 'late' scleroderma patterns, and 'non-specific' changes. The association of the nailfold capillaroscopy changes and their components with clinical manifestations was also studied. RESULTS: We studied 15 DcSSc and 55 LcSSc patients. No association was found between the patterns of capillaroscopic changes and these subtypes. There were 8 early DcSSc, 7 intermediate/late DcSSc, 34 early LcSSc, and 21 intermediate/late LcSSc patients. In patients with LcSSc, the 'early' scleroderma pattern of capillaroscopy was associated with early disease based on duration. We found a direct association between some capillary components and some clinical findings. Also, some capillaroscopic components had an inverse association with some clinical manifestations. CONCLUSIONS: We found no association between the patterns of capillaroscopy and SSc subtypes; early scleroderma pattern of capillaroscopy was significantly associated with early LcSSc, compatible with the slower course of the disease in LcSSc. Subtle changes, capillary elongation, and capillary tortuosity had an inverse association with clinical manifestations and might be considered as good prognostic factors.

One decade distinct features, morbidity and mortality of scleroderma: a cross-sectional study.

OBJECTIVES: Conducting an epidemiologic study on scleroderma patients referred to hospitals and tertiary centres of rheumatologic diseases in Shiraz, located in south of Iran. METHODS: A cross-sectional study was done on patients' records registered in scleroderma outpatient clinics as well as hospitals associated with Shiraz University of Medical Sciences. Gathering data in pre-formed data sheets, descriptive analysis plus qualitative comparisons by chi-square test were done using SPSS 15. RESULTS: In 533 medical records, female to male ratio was 7.3:1. The disease is mostly seen in 3rd and 4th decades of life. More patients had negative family histories (56.1%). 37.5% of the patients had diffuse form of the disease, 36.8% had limited one, and 17.3% had overlap syndrome, mostly, by lupus erythematosus (33%). Most common first presentation was Raynaud phenomenon (40.7%). Two most prevalent clinical manifestations were skin thickening (97.2%) and gastrointestinal involvement (68.9%). Clinical presentations were compared between three most common types of the disease plus various stages of life. Among recorded capillaroscopies, active form was the most prevalent one (38.3%). In documented serologic markers, the most common positive one was anti-nuclear antibody (ANA) (75.6%). Two most common etiologies of hospitalisation were digital ulcer (30.9%) and pulmonary fibrosis (5.7%). The most common cause of death (17) was pulmonary fibrosis (35.2%). CONCLUSION: This study is the first epidemiologic survey on Iranian scleroderma patients with significantly large sample size compared to previous studies worldwide. It can thus provide some guidance for further multi-provincial, multinational and interracial studies on scleroderma.

Reliability, validity, and responsiveness of the Persian version of the rheumatoid and arthritis outcome score (RAOS) in patients with rheumatoid arthritis.

The aims of this study were to cross-culturally translate the original rheumatoid and arthritis outcome score (RAOS) into Persian and evaluate its reliability, validity, and responsiveness in a group of patients with rheumatoid arthritis (RA). The questionnaire was translated through a standard forward-backward translation. A sample of 103 patients was asked to complete the Persian RAOS, the Short Form-36 (SF-36), and the arthritis impact measurement scale-short form (AIMS2-SF). To determine test-retest reliability, the Persian RAOS was readministered to a sample of 50 patients, 3-6 days after the first visit. To evaluate responsiveness, 50 patients completed the Persian RAOS at baseline and at the end of a pharmacological intervention. Test-retest reliability and internal consistency were assessed using intraclass correlation coefficient (ICC) and Cronbach's alpha, respectively. Construct validity was assessed by comparing the results of the RAOS with the Persian SF-36 and AIMS2-SF using Spearman's correlation coefficient. Responsiveness was assessed by the calculation of effect size (ES) and standardized response means (SRM). The acceptable level of ICC > 0.70 and Cronbach's alpha > 0.70 were obtained for the most RAOS subscales. As expected, moderate to strong correlations were observed between subscales of the RAOS and the SF-36/AIMS2-SF intended to measure similar constructs. The ES range of 0.18 to 0.51 and the SRM range of 0.25 to 0.91 were obtained for the RAOS subscales. In conclusion, the Persian RAOS is a reliable, valid, and responsive outcome measure for patients with RA suffering from arthritis in the lower limb joints.

The Prevalence of Thyroid Dysfunction in Patients With Systemic Lupus Erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease caused by immune system-mediated tissue damage. Autoimmune thyroiditis (AT) is an organ-specific disease associated with production of a variety of antibodies such as antinuclear antibodies, anti-double-stranded DNA, anti-Ro antibodies and anti-cardiolipin antibodies. OBJECTIVES: The aim of this study was to evaluate the prevalence of thyroid dysfunction and thyroid auto-antibodies in patients with SLE and its relation to SLE disease and other autoantibodies. PATIENTS AND METHODS: This was a case-control study. The study included a total of 88 patients with SLE and 88 age- and sex-matched healthy volunteers as control group. Two study groups were compared regarding thyroid function test, antinuclear antibody (ANA), antibodies to double-stranded DNA (dsDNA), anti- thyroglobulin antibody (anti-Tg), and anti-thyroid peroxidase (anti-TPO) antibody. RESULTS: The mean age of SLE patients and controls were 32.16 ± 9.19 and 32.48 ± 9.47 years, respectively (P = 0.821). Patients had significantly higher prevalence (43.2% vs. 23.9%; P = 0.015) and titers (221.8 ± 570.5 vs. 78.2 ± 277.2; P = 0.036) of antibodies to Tg compared to controls. The patients had significantly lower titers of T3 compared to controls (125.2 ± 35.6 vs. 136.2 ± 26.5; P = 0.021). The titers of T4, TSH and anti-TPO antibody did not differ significantly between the two study groups. CONCLUSIONS: Thyroid dysfunction was not higher in SLE patients compared to healthy individuals. However, anti-Tg antibodies were higher in SLE patients. It has not yet been established that thyroid function tests should be performed routinely in SLE patients.

The effect of conditioning exercise on the health status and pain in patients with rheumatoid arthritis: a randomized controlled clinical trial.

BACKGROUND: Rheumatoid Arthritis (RA) is a systemic and inflammatory disease of unknown etiology which is mostly characterized by inflammation of the synovial joints. Studies have proved that most people with RA avoid doing physical activities due to fear that it may worsen the pain or cause pressure on joints, resulting in decreased muscle strength and ultimately leading to disability of patients. We aimed to investigate the effects of conditioning exercises on the health status and pain in patients suffering from RA. METHODS: In this randomized controlled clinical trial, we enrolled 66 women with confirmed RA referred to the rheumatology clinic of Hafez hospital, Shiraz, southwest Iran during May-July 2013. Balanced block randomization method was used to randomize the participants into case and control groups (two groups of 33 each).Data were collected using visual analog scale (VAS), Arthritis Impact Measurement Scales 2 short form (AIMS2-SF), and demographic questionnaire. After obtaining written informed consent, the participants in the case group were asked to participate in conditioning exercise programs including aerobic, isometric, and isotonic exercises and received a training booklet explaining the exercises that they could do at home after the intervention. RESULTS: There was a statistically significant difference between the health status scores of the patients in the case groups before and after the intervention (P=0.001). The pain score also decreased significantly in the case group compared with the control group after the intervention (P=0.003). CONCLUSION: We concluded that physical training programs, especially conditioning exercises, could improve the health status and reduce pain in patients with RA. TRIAL REGISTRATION NUMBER: IRCT201308187531N3.

Standpoints of traditional Persian physicians on geriatric nutrition.

The present article aimed to compile information on the nutritional management for geriatric people. Popular textbooks of Persian medicine from 10th to 18th century were studied to derive relative viewpoints and considerations. The temperament, which is defined as the combination of 4 main elements (fire, air, water, and soil) and 4 humors made subsequently (black bile, yellow bile, phlegm, and blood), changes during age periods. Imbalance in proportional amounts of humors in elderly should be corrected with food and medicaments having opposite nature to the current condition. Traditional foods included mostly well-cooked soups, pottages, and porridges containing fresh sheep or chicken meat. Mono-ingredient foods were also administered according to their medical properties. Nutritional recommendations were also concerned with geriatrics' physiological conditions such as constipation, sleep disorders, and memory deficits. Many of traditional geriatric nutritional requirements are relevant in the present day. However, there are still notes that may be beneficial for consideration.

High prevalence of Helicobacter pylori infection in Behcet's disease.

BACKGROUND: Behcet's disease (BD) is a multisystem disease of unknown etiology. There are several clues which may indicate an ethiopathogenesis role for Helicobacter pylori infection in this disease. METHODS: In a case control study in an out patient department, 48 patients with BD were compared to age, sex matched controls regarding presence of H. pylori infection by serology and urea breath test (UBT). RESULTS: Ongoing H. pylori infection was more prevalent among patients with BD using result of UBT with odds ratio of 3.1 (95% CI: 1.34 - 7.26, PV < 0.001). CONCLUSION: H. pylori infection may have a role in the pathogenesis of BD.

Diabetic muscle infarction and diabetic dermopathy two manifestations of uncontrolled prolong diabetes mellitus presenting with severe leg pain and leg skin lesions.

Diabetic muscular infarction (DMI) is a rare manifestation which can be seen in patients with long-standing diabetes mellitus. Patients usually come with painful swelling of an involved muscle in one extremity. MRI and biopsy histology can help diagnose this condition. Diabetic dermopathy is another manifestation of patients with diabetes.We present a patient with uncontrolled diabetes type 2 presented with pain, swelling, and a palpable tender mass in one leg along with new skin lesions. Biopsy of the skin lesion and T2-weighted MRI of the leg helped differentiate DMI and dermopathy.

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.

QT dispersion in patients with systemic lupus erythematosus: the impact of disease activity.

BACKGROUND: Patients with systemic lupus erythematosus (SLE) have increased cardiovascular morbidity and mortality. Although autopsy studies have documented that the heart is affected in most SLE patients, clinical manifestations occur in less than 10%. QT dispersion is a new parameter that can be used to assess homogeneity of cardiac repolarization and autonomic function. We compared the increase in QT dispersion in SLE patients with high disease activity and mild or moderate disease activity. METHODS AND RESULTS: One hundred twenty-four patients with SLE were enrolled in the study. Complete history and physical exam, ECG, echocardiography, exercise test and SLE disease activity index (SLEDAI) were recorded. Twenty patients were excluded on the basis of our exclusion criteria. The patients were divided to two groups based on SLEDAI: 54 in the high-score group (SLEDAI > 10) and 50 in the low-score group (SLEDAI < 10).QT dispersion was significantly higher in high-score group (58.31 ± 18.66 vs. 47.90 ± 17.41 respectively; P < 0.004). QT dispersion was not significantly higher in patients who had received hydroxychloroquine (54.17 ± 19.36 vs. 50.82 ± 15.96, P = 0.45) or corticosteroids (53.58 ± 19.16 vs. 50.40 + 11.59, P = 0.47). There was a statistically significant correlation between abnormal echocardiographic findings (abnormalities of pericardial effusion, pericarditis, pulmonary hypertension and Libman-Sacks endocarditis) and SLEADI (P < 0.004). CONCLUSIONS: QT dispersion can be a useful, simple noninvasive method for the early detection of cardiac involvement in SLE patients with active disease. Concerning high chance of cardiac involvement, cardiovascular evaluation for every SLE patient with a SLEDAI higher than 10 may be recommended. TRIAL REGISTRATION: Clinicaltrial.gov registration NCT01031797.

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.