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Abstract A 17-year-old boy complaining of progressive dyspnea, fever, palpitations, a 22 mm Hg blood pressure difference between the 2 arms, and arm claudication. He had a history of psoriasis-like skin lesions and bronchiectasis. Echocardiography revealed a reduced left ventricular ejection fraction, severe eccentric aortic insufficiency, circumferential aortic wall thickening, and a dilated ascending aorta with severe atherosclerotic changes. Based on imaging findings, a TA diagnosis was suggested. During his follow-up, the patient developed strabismus, blurred vision, and right sixth cranial nerve paralysis and went into a deep coma. Unfortunately, after 6 months of treatment, he expired due to COVID-19 infection.

Takayasu arteritis (TA) is a rare granulomatous panarthritis, characterized by stenosis or obliteration of large and medium-sized arteries, such as the aorta. The onset of symptoms tends to lead to a delay in diagnosis that can range from months to years, during which time vascular disease may initiate and progress to become symptomatic. In this context, TA may present with various uncommon cardiovascular complications such as dilated cardiomyopathy, valvulitis, and myocarditis. Herein, we report on a 29-year-old female who initially presented with active myocarditis and was later incidentally diagnosed with TA.

Cardiovascular disorders are significantly associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Herein, we describe a case of myopericarditis and subsequent transient constrictive pericarditis after coronavirus disease 2019 (COVID-19). Three weeks following a mild SARS-CoV-2 illness, a 53-year-old woman was referred to the hospital with acute pleuritic chest pain, which was not attributable to any known cause and was only temporarily relieved. The pain persisted for the next few weeks until her second COVID-19 infection, which occurred 5 months after her first affliction. This time, Transthoracic echocardiography (TTE) revealed mild pericardial effusion, and cardiac magnetic resonance imaging (CMR) confirmed myopericarditis, leading to the administration of anti-inflammatory therapy for the patient. Despite a relative resolution of symptoms, her second CMR performed 8 months later revealed active perimyocarditis with transient constrictive pericarditis (CP).Additionally, fluorescent antinuclear antibody (FANA) and antimitochondrial Ab M2 (AMA) were tested positive for the first time. Thereafter, the patient was started on concurrent anti-inflammatory and immunosuppressant therapies, which were effective after 3 months. The transient CP was resolved, and there was no sign of active pericarditis on her last echocardiography. Acute pericarditis and its subsequent constrictive pericarditis are infrequent adverse outcomes of COVID-19. The unique feature of this case is the uncertainty regarding the underlying reason for cardiac complications, whether it is the first presentation of systemic lupus erythematosus (SLE) or viral-induced myopericarditis followed by a consequent transient CP.

Abstract Background Rheumatoid arthritis (RA) is an inflammatory disease that is characterized by the overproduction of cytokines. Among the newly discovered cytokines are the adipokines which are primarily produced by and released from the adipose tissue and some immune cells, as well as synovial cells. they are involved in various immune responses and inflammatory processes. However, there are controversial data regarding the pro-inflammatory or anti-inflammatory effects of adipokines in different conditions. C1q/TNF-related protein 5 (CTRP5) is a newly identified adipokine and adiponectin paralogous protein, which has been shown to be correlated with inflammatory diseases. Accordingly, the present study was designed to investigate the serum levels of CTRP5 in RA patients and evaluate any possible alterations in comparison to healthy individuals. Methods Serum CTRP-5 levels were measured in 46 patients and 22 healthy controls by ELISA. The demographic, laboratory, and clinical features of the patients were also evaluated in order to find any correlations. Results Serum levels of CTRP-5 were significantly (p

We aimed to explore the cardiovascular magnetic resonance (CMR) of Takayasu arteritis (TA) and its cardiovascular complications. CMR was conducted on 37 TA patients and 28 healthy individuals. We evaluated the CMR findings and adverse cardiovascular complications at the time of the CMR (ACCCMR). After 8 to 26 months, the major adverse cardiac and cerebrovascular events (MACCEs) were evaluated. The TA included 25 women (67.6%), aged 36 ± 16 years old, and 28 age- and sex-matched healthy controls. Left ventricular (LV) ejection fraction was significantly lower in the TA group than in the control group (51 ± 9% vs. 58 ± 1.7%; p < 0.001). Aortic mural edema was present in 34 patients (92%) and aortic mural hyperenhancement in 36 (97%). Left ventricular global longitudinal strain (LVGLS) was significantly lower in the TA group (median [interquartile range] = 13.70 [3.27] vs. 18.08 [1.35]; p < 0.001). ACCCMR was seen in 13 TA patients (35.1%), with the most common cardiac complication being myocarditis (16.2%). During a median follow-up of 18 months (8–26 months), nine patients developed MACCEs, of which the most common was cerebrovascular accident in five (13.5%). The LVGLS of the CMR had the strongest association with complications. Myocardial strain values, especially LVGLS, can reveal concurrent and future cardiovascular complications in TA patients.

Background: Rheumatoid arthritis (RA) is the most common chronic inflammatory joint disease. Complications of RA can cause low quality of life and disabilities. Nowadays, despite all the medical developments, etiology of RA is unclear. Both soft and hard tissue damages occur in RA and periodontitis due to chronic inflammation and also since tissue damage presentation and pathogenesis of RA and periodontitis are the same, this study was done for evaluating the relationship between clinical and laboratory findings in RA patients with their oral status and disease activity. Methods: This case-control study was performed on 236 patients; 118 RA patients and 118 cases of normal people. Gingivitis, dental caries and plaques, oral hygiene and severity of periodontitis were measured based on gingival index, plaque index, clinical attachment level, Decayed Missing Filled index and oral hygiene index-simplified. Disease activity was assessed according to Diseases Activity Score-28. Blood samples were taken to evaluate the level of anti-CCP, RF, ESR, CRP, and CBC. Data were analyzed by t-test and chi-square. Results: RA patients are more susceptible to periodontitis, plaque formation and dental caries. There is a relationship among RA disease and periodontitis, oral hygiene, gingivitis. There is also a reverse relationship between RF level and periodontitis severity likewise dental caries. There is no significant relationship between other laboratory findings and the oral status of patients. Conclusion: This is more likely for RA patients to experience periodontitis which can destruct alveoli bone and it can also cause early tooth loss. Regular examination and early treatment are highly recommended.

Background: The relationship between rheumatoid arthritis and poor oral hygiene has longbeen proven. Colonization of Porphyromonas gingivalis in the oral cavity can play an important role in rheumatoid arthritis. The aim of this study was to determine whether there is a relationship between rheumatoid arthritis disease and the presence of P. gingivalis. Methods:sixty patients with rheumatoid arthritis and 75 controls with matched age, sex and smoking and oral health status were selected. Provided samples from dental plaque were assessed for the presence of P. gingivalis by using bacterial culture and polymerase chain reaction (PCR). Results:Results of bacterial culture and PCR showed no significant difference between rheumatoid arthritis patients and control group in terms of the presence of P. gingivalis (P = 0.9 and P = 0.1, respectively). There was also no relationship between the number of bacterial copies and the activities of rheumatoid arthritis. Conclusion:Findings showed that there was no significant relationship between the presence of P. gingivalis in dental plaque and rheumatoid arthritis.

Background: Rheumatoid arthritis (RA) is a common chronic inflammatory disorder that can be associated with some hearing impairments. Hence, we aimed to evaluate the prevalence rate of hearing loss (HL) in RA patients. Materials and Methods: This study included 130 participants from February 2019 to March 2020 including 100 RA patients (78 females and 22 males) as RA group and 30 healthy cases (16 females and 14 males) as control group. All patients underwent pure tone audiometry, speech audiometry, tympanometry, acoustic reflex, and tone decay test by single operator and device. The rate of HL and contributing factors were then determined. Results: The mean age of RA group was 53.95 ± 7.6 years, and the mean duration of disease was 12.74 years. Rheumatoid factor was positive in 54% of patients, and the frequency rates of diabetes, chronic kidney disease, hypertension, and dyslipidemia among RA patients were 14%, 1%, 26%, and 19%, respectively. These values among RA patients with HL were 18%, 1.7%, 34%, and 27.5%, respectively. HL in RA patients was related to dyslipidemia (P = 0.011) and age (P = 0.0001). Frequency rate of conductive HL in left and right ears was 2% and 5%, respectively, and these rates for sensorineural hearing loss (SNHL) were 55% and 61%, respectively. In addition, the percent of HL in low, mid, and high frequency ranges was 18%, 19%, and 57%, respectively. Conclusions: The findings of the present research demonstrate that HL especially with SNHL and high-frequency types is common among RA patients.

Background: Sjögren’s syndrome is the second most common systemic autoimmune disease after rheumatoid arthritis, RA, with a prevalence of about 0.5% in the general population. It occurs primarily in perimenopausal women (at a ratio of women to men of 9: 1) Sjögren’s syndrome is characterized by lymphocytic infiltration to exocrine glands. The specific autoantibodies of this syndrome are against Ro (SSA) and La (SSB). B cell dysfunction occurs in patients with Sjögren's syndrome. B lymphocyte cells produce monoclonal immunoglobulin heavy and light chains addition to producing Ro (SSA) and La (SSB). Up to 25% of patients in this situation are associated with antibody production. This study aimed to evaluate the prevalence of monoclonal immunoglobulin in patients with primary Sjögren's syndrome. Methods: 31 patients with primary Sjogren's syndrome diagnosed according to the American College of Rheumatology (ACR) criteria referred to Rheumatology Clinics of Firoozgar Hospital, Tehran, during first six months of 2016, were studied. Clinical examinations and tests carried out. We analyzed serum monoclonal immunoglobulins by immunofixation electrophoresis (IFE) on agarose gels with specific antisera to IgG, IgM, IgA, and κ and λ chains. The IFE was performed with a Helena immunofixation agarose kit (Helena Laboratories, Beaumont, TX, USA) following the manufacturer's recommended procedure. The measures of Anti-Ro, anti-La, rheumatoid factor (RF), antinuclear antibodies (ANA) with immunofluorescent method, complete blood count (CBC) with differential by peripheral blood smear, an erythrocyte sedimentation rate with western green tube, dip stick urine analysis, urine specific gravity (SG) with Refractometer (Mesu Lab Instruments Co., Guangzhou, China), turbidimetry serum complement, and the complement CH50 with ELISA were done. Results: Most of (87%) patients were female. The mean age of patients was 45.32±12.02 years. The majority of the patients (94%, 29 cases), ocular sign was observed. Xerostomia were common in 84% cases (n=26). Arthralgia/arthritis and submandibular enlargement were common 48% and 19% of patients, respectively. Lymphadenopathy, lung involvement and parathyroid, each was prevalent in 16% of patients. Vasculitis in 13% and Raynaud's phenomenon in 6% of the patients were common. Liver and kidney involvements with the lowest prevalence, each was prevalent in 3% (1 person) patients. RF and the erythrocyte sedimentation rate (ESR) were abnormal in 22.58% and 12.90% of patients, respectively. The immunofluorescent antinuclear antibody (ANA or FANA) pattern, C3 and C4 complements levels were abnormal in 16.12%, 12.90% and 6.4% of patients, respectively. In 29% (n=9), monoclonal antibodies were higher than normal. IgG, IgM and IgG, were abnormal in 19.3% (n=6), 6.4% (n=2) and 3.3% (n=1), respectively. Conclusion: Monoclonal immunoglobulin levels in patients with primary Sjogren's syndrome increases. To achieve more definitive and reliable results, this study should be repeated with larger sample size and at different times.

Abstract Objectives: To investigate the on-labeled and off-labeled indications of rituximab according to available evidence and the cost in our center. Methods: This retrospective cross-sectional study was conducted between August 2016 and August 2017 at a teaching hospital affiliated to Iran University of Medical Sciences, Tehran, Iran. Patients’ demographic data and disease, indication for rituximab use, its dosage and treatment regimen and previous and concurrent treatments was assessed. The collected data were compared with the current criteria for the pattern of rituximab use. The last version of Lexicomp® acquired by Wolters Kluwer was used as the reference for on-labeled and off-labeled indications of the prescribed drug and its dosage. Level of evidences for applied indications and cost has also been evaluated. Results: A total of 85 patients received rituximab during the study period. The most frequent reasons for rituximab prescription were: multiple sclerosis (50.58%), systemic sclerosis (10.58%), rheumatoid arthritis (7.05%) and Idiopathic inflammatory myopathies (4.70%). Rituximab was used in 8 (9.4%) cases according to on-labeled indications. There was level C evidence for rituximab use in off-labeled indications in 47 (55.29%) cases according to available evidences which accounts for the highest calculated cost. Conclusion: Based on our results, rituximab was frequently administrated for off-labeled indications most of which are not supported by established levels of evidence. Strong evidence is necessary for decision making regarding its effectiveness. Keywords: Rituximab; Drug Utilization Evaluation; Multiple Sclerosis

Background: Ankylosing spondylitis (AS) is a chronic inflammatory condition associated with more cardiac manifestations than those in the normal population. In this study, we sought to determine the prevalence of cardiac involvement in patients suffering from AS without cardiovascular risk factors. Methods: The present case-control study, conducted in 2 university hospitals in Tehran from January 2016 to December 2017, recruited 67 patients with AS and 40 age- and sex-matched healthy controls. The diagnosis of AS was based on the classification criteria of the Assessment of SpondyloArthritis International Society. All the participants were examined using transthoracic echocardiography and a standard 12-lead ECG. Baseline characteristics, echocardiographic findings, and ECG features were compared between the AS and control groups using univariate analyses. Results: The median age was 33.5 (IQR25-75%: 20.5–59) years in the AS group and 35 (IQR25-75%: 26–59) years in the control group (P=0.301). The number of patients with left ventricular systolic and diastolic dysfunction was significantly higher in the patients with AS than in the controls (7.5% vs. 20.9%; P=0.067, and 22.9% vs. 5.0%; P=0.026, respectively). The number of individuals with a left-axis deviation and a left anterior fascicular block was significantly higher in the patients suffering from AS than in the control group. The number of patients with aortic valve involvement was comparable between the groups (P=0.332). Conclusion: The most common cardiac involvement in our patients with AS was left ventricular dysfunction, followed by rhythm disturbances and aortic valve insufficiency. These findings were independent of age, AS severity, and disease duration. Therefore, the implementation of cardiovascular screening can be recommended for patients with AS.

The presence of different autoimmune disorders in the same individual is called multiple autoimmune syndrome (MAS). One of these co-occurring conditions is autoimmune haemolytic anaemia (AIHA), which is characterized by the production of autoantibodies against red blood cells due to immune system malfunction and which results in severe tissue oxygenation disturbance. AIHA is not uncommon but occurs rarely in MAS; if it does, MAS is then classified as MAS type III. Herein, we describe a case of MAS type III including AIHA which was successfully treated with hydrocortisone with gradual resolution of symptoms.

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Background: Cardiac involvement in systemic sclerosis (SSc) is more prevalent than previously thought. In this study, the frequency and severity of cardiovascular involvement were assessed in SSc patients referred to Firouzgar Hospital.Methods: Fifty-eight patients with SSc, selected from the data bank of SSc patients, were reviewed for the frequency and severity of 8 organ involvements in this case series.The preliminary severity scale, published by international SSc study groups, was employed for the determination of theseverity grade in the cardiovascular system. In the cardiac scoring scale, grade 0 represents normal heart (no cardiac involvement), grade 1 denotes mild involvement [electrocardiography (ECG) conduction defect and a left ventricular ejection fraction (LVEF) of 45-49%)], grade 2 signifies moderate involvement (arrhythmia, LVEF = 40-44%), grade 3 indicates severe involvement (LVEF

Background: Cardiac involvement in systemic sclerosis (SSc) is more prevalent than previously thought. In this study, the frequency and severity of cardiovascular involvement were assessed in SSc patients referred to Firouzgar Hospital. Methods: Fifty-eight patients with SSc, selected from the data bank of SSc patients, were reviewed for the frequency and severity of 8 organ involvements in this case series. The preliminary severity scale, published by international SSc study groups, was employed for the determination of the severity grade in the cardiovascular system. In the cardiac scoring scale, grade 0 represents normal heart (no cardiac involvement), grade 1 denotes mild involvement [electrocardiography (ECG) conduction defect and a left ventricular ejection fraction (LVEF) of 45-49%)], grade 2 signifies moderate involvement (arrhythmia, LVEF = 40-44%), grade 3 indicates severe involvement (LVEF

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.