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IntroductionSystemic sclerosis (SSc) is an autoimmune disorder causing progressive fibrosis of internal organs and skin, leading to increased thickness and rigidity. It frequently results in malnutrition, which can decrease survival rates and physical performance, while also imposing financial burdens on healthcare systems and society. Multimodal nutritional interventions may encompass various approaches, including nutritional counselling, consumption of oral nutritional supplements and disease-specific dietary regimens based on nutritional needs. The present clinical trial is designed to examine the effectiveness of multimodal nutritional intervention on nutritional status, quality of life, food intake, body composition and fatigue in patients with SSc.Methods and analysisThis is a parallel, randomised, placebo-controlled clinical trial that will investigate the impacts of multimodal nutritional interventions in SSc patients. 46 qualified SSc participants will be chosen at random and given multimodal nutritional intervention or dietary recommendations for a period of 8 weeks. The primary outcomes of this study are changes in nutritional status and quality of life. The secondary outcomes include changes in body composition, fatigue, body weight, body mass index and calf circumference. Statistical analysis will be conducted using the SPSS software (Version 24).Ethics and disseminationThe current trial received approval from the Medical Ethics Committee of Tehran University of Medical Sciences, Tehran, Iran (IR.TUMS.MEDICINE.REC.1403.345). The findings will be submitted to international, peer-reviewed publications and presented at national and international conferences.Trial registration numberIRCT20220208053971N4.

Systemic sclerosis (SSc) is a disease of connective tissue with high rate of morbidity and mortality highlighted by extreme fibrosis affecting various organs such as the dermis, lungs, and heart. Until now, there is no specific cure for the fibrosis occurred in SSc disease. The SSc pathogenesis is yet unknown, but transforming growth factor beta (TGF-β), endothelin-1 (ET-1), and Ras-ERK1/2 cascade are the main factors contributing to the tissue fibrosis through extracellular matrix (ECM) accumulation. Several studies have hallmarked the association of ET-1 with or without TGF-β and Ras-ERK1/2 signaling in the development of SSc disease, vasculopathy, and fibrosis of the dermis, lungs, and several organs. Accordingly, different clinical and experimental studies have indicated the potential therapeutic role of ET-1 and Ras antagonists in these situations in SSc. In addition, ET-1 and connective tissue growth factor (CTGF) as a cofactor of the TGF-β cascade play a substantial initiative role in inducing fibrosis. Once initiated, TGF-β alone or in combination with ET-1 and CTGF can activate several kinase proteins such as the Ras-ERK1/2 pathway that serve as the fundamental factor for developing fibrosis. Furthermore, Salirasib is a synthetic small molecule that is able to inhibit all Ras forms. Therefore, it can be used as a potent therapeutic factor for fibrotic disorders. So, this review discusses the role of TGF-β/ET-1/Ras signaling and their involvement in SSc pathogenesis, particularly in its fibrotic situation.

Coronavirus disease 2019 (COVID-19) has transformed into a worldwide challenge, since its outbreak in December 2019. Generally, patients with underlying medical conditions are at a higher risk of complications and fatality of pneumonias. Whether patients with systemic autoimmune diseases or vasculitides, are at increased risk for serious complications associated with COVID-19, is not established yet. Computed tomography (CT) has been employed as a diagnostic tool in the evaluation of patients with clinical suspicion of severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection with a reported sensitivity of higher than reverse transcription polymerase chain reaction (RT-PCR) test. Multifocal bilateral ground-glass opacities (GGOs) with peripheral and posterior distribution and subsequent superimposition of consolidations are considered the main imaging features of the disease in chest CT. However, chest CT images of underlying rheumatologic or autoimmune diseases or vasculitides, such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Behçet disease, and granulomatosis with polyangiitis, especially those with extensive lung involvement can overshadow or obliterate features of COVID-19. In addition, CT findings of such diseases may resemble manifestations of COVID-19 (such as ground glass opacities with or without superimposed consolidation), making the diagnosis of viral infections, more challenging on imaging. Comparing the imaging findings with prior studies (if available) for any interval change is the most helpful approach. Otherwise, the diagnosis of COVID-19 in such patients must be cautiously made according to the clinical context and laboratory results, considering a very high clinical index of suspicion on imaging.

ObjectivesInterstitial lung disease (ILD) is an important manifestation of autoimmune diseases that can lead to morbidity and mortality. Although several autoantibodies have been linked with ILD presentation and adverse outcomes, the association of anti-Ro52 antibody with ILD is less studied. Hence, we investigated this association in various autoimmune diseases in the current study.DesignWe designed a systematic review and meta-analysis and did a comprehensive search from inception until 2 January 2023.Data sourcesA systematic search was conducted in four electronic databases: PubMed, Web of Science, Scopus and Embase.Eligibility criteriaObservational studies that reported ILD diagnosis (outcome) and anti-Ro antibody (exposure) status in any autoimmune conditions (population) were included. The association between rapidly progressive ILD (RP-ILD) and anti-Ro52 was studied in idiopathic inflammatory myopathies (IIM).Data extraction and synthesisCollected data included study characteristics and ORs with 95% CIs. Quality assessment was performed using a modified version of the Newcastle-Ottawa Scale for cross-sectional studies. Random effects meta-analysis was used to pool the effect estimates.ResultsA total of 2353 studies were identified, from which 59 articles met the eligibility criteria. Anti-Ro52/SSA positivity was associated with ILD in all autoimmune disease subgroups: IIM (OR=3.08; 95% CI: 2.18 to 4.35; p value<0.001; I2=49%), systemic lupus (OR=2.43; 95% CI: 1.02 to 5.79; p=0.046; I2=71%), Sjogren (OR=1.77; 95% CI: 1.09 to 2.87; p=0.021; I2=73%), systemic sclerosis (OR=1.71; 95% CI: 1.04 to 2.83; p=0.036; I2=43%), mixed connective tissue disease (OR=3.34; 95% CI: 1.82 to 6.13; p<0.001; I2=0%). Additionally, anti-Ro52-positive myopathy patients were more likely to have simultaneous RP-ILD (OR=2.69; 95% CI:1.50 to 4.83; p<0.001; I2=71%).ConclusionAnti-Ro52/SSA positivity is associated with a higher frequency of ILD diagnosis in various autoimmune diseases. Anti-Ro52/SSA is also linked with a more severe lung involvement (RP-ILD). Future studies can investigate the benefits of screening for anti-Ro52 and its association with ILD development.PROSPERO registration numberCRD42022381447.

Objectives To present the clinical characteristics, disease course, management, and outcomes of COVID-19 infection in patients with Behcet’s disease (BD). Methods In this retrospective cohort study, we retrieved BD patients with definite diagnosis of COVID-19 infection. Demographic data, comorbidities, features related both to BD and COVID-19 infection, treatments, and outcomes were collected. Comparisons between patients with or without hospitalization were performed. All statistical analyzes were performed using SPSS version 25. We considered p  < 0.05 statistically significant. Results We identified 61 episodes of COVID-19 infection in 59 BD patients. The prevalence was 0.69%. The median age was 45 years ( IQR  = 20), and the median disease duration was 162 months ( IQR  = 195). BD features were similar except for higher rate of arterial involvement and positive pathergy test in infected patients. Thirty-five episodes (62.5%) happened in non-active patients; 39% had a comorbid disease. COVID manifestations were the same as the general population. Flu-like symptoms were the most common (85%), followed by fever (66%), ageusia/anosmia (56%), headache (51%), and pulmonary involvement (48%). There was no change in BD symptoms in 74%. Fifteen patients (25.4%) were hospitalized, and one patient (1.7%) died. Receiving glucocorticoids ( p  < 0.03) and cytotoxic drugs ( p  < 0.02) were associated with an increased rate of hospitalization. Conclusion The incidence of COVID-19 infection in BD patients was not higher than general population in Iran. They showed milder form of disease with lower morbidity and mortality rate. Most were on immunosuppressive drugs, or had a comorbidity apart from BD. No significant effect on BD course was shown. Key Points • The incidence of COVID-19 infection in patients with Behcet’s disease is not higher. • They showed milder form of infection with lower morbidity and mortality rate. • No significant effect on Behcet’s disease course was shown with COVID19 infection. • BD patients can be managed according to the guidelines used for general population.

Background A strong correlation was previously found between mean platelet volume (MPV), red blood cell distribution width (RDW), and the severity of signs and symptoms in patients suffering from inflammatory and autoimmune diseases. The current study evaluated these correlations in patients with Behçet’s disease (BD) as well the relationship between MPV and RDW and disease activity score on the Iranian Behçet’s Disease Dynamic Activity Measurement (IBDDAM). Methods This cross-sectional study included 319 patients with BD for whom demographic and epidemiological data, IBDDAM scores, and duration of illness was recorded. Blood samples were then obtained and the relationships between their disease status and manifestations and their laboratory parameters were evaluated with statistical models to find possible correlations. Results Our analysis showed a significantly higher RDW in patients with BD who had ocular manifestations ( p  < 0.001) and oral aphthae ( p  = 0.004). Patients with active BD had higher RDW ( p  < 0.001) and MPV ( p  < 0.001) in comparison to those with currently inactive BD. Similarly, patients who had any type of ocular manifestation had higher RDW ( p  < 0.001) and MPV ( p  < 0.001). Regression analyses identified a statistically significant model for the effect of RDW and MPV in predicting active BD status ( p  < 0.001), as well as its significant relationship with active ocular manifestations ( p  < 0.001). Conclusion BD was found to be associated with an increase in MPV and RDW, particularly during active phases. RDW and MPV were also found to have predictive value for screening to detect BD activity and its ocular complications.

A 55‐year‐old male patient with diffuse cutaneous systemic sclerosis (dcSSC) since 2018 presented with a history of arrhythmia. He had been stable for 5 years with pantoprazole, diltiazem, and mycophenolate mofetil (MMF); vitamin E; and vitamin D until he developed arrhythmia. Different evaluations revealed left bundle branch block, wall motion abnormality, mildly reduced systolic function, diffused interstitial fibrosis, and lesions in the left circumflex artery (LCX) and left anterior descending artery (LAD) and stenosis in LCX, without significant improvement following percutaneous coronary intervention for LCX stenosis. Holter monitoring demonstrated persistent ventricular premature beats and couplets. Arrhythmia was not responsive to bisoprolol therapy, and it was not feasible to perform cardiac ablation. Suspecting MMF–induced arrhythmia, MMF was discontinued, which led to a reduction in arrhythmia and symptom improvement after 9 months. This case report emphasized a possible heart‐related complication of MMF, which healthcare providers should consider when prescribing medication to patients.

Background Patients with autoimmune disorders are highly susceptible to infections including cytomegalovirus (CMV) leading to serious complications ranging from asymptomatic to severe systemic diseases. Case Presentation The first case was a 44‐year‐old woman with systemic lupus erythematosus (SLE) referred to the Rheumatology ward due to a necrotizing ulcer on the hand's finger and multiple ulcerative lesions on her lips and tongue. She had pancytopenia and tested positive for both herpes simplex virus (HSV) and CMV polymerase chain reaction (CMV‐PCR). She was treated with ganciclovir for 14 days and subsequently recovered. The second case was a 55‐year‐old man, a known sarcoidosis case, admitted to the hospital due to lower extremities weakness and intraoral ulcers. The lab findings revealed leukopenia and elevated levels of ESR and CRP. Viral markers were all negative except for the CMV‐PCR test. Electromyography and nerve conduction velocity (EMG‐NCV) showed subacute axonal motor polyneuropathy in the lower limbs. Due to the high titers of CMV (500,000 copies/mL), he was treated with ganciclovir for 2 weeks and the symptoms improved dramatically. Conclusion CMV infection in patients with inflammatory rheumatic diseases may lead to rare manifestations that can be misdiagnosed as a flare of the underlying disease. Autoimmune disorder patients are vulnerable to cytomegalovirus (CMV) infections, which can present with symptoms that mimic flare‐ups of the underlying disease. We present two cases: a 44‐year‐old woman with systemic lupus erythematosus and a 55‐year‐old man with sarcoidosis, both of whom developed unusual manifestations linked to CMV infection, confirmed by PCR. Treatment with Ganciclovir led to significant improvement, emphasizing the need for careful consideration of CMV in autoimmune patients with atypical symptoms.

Behçet’s disease (BD) is a chronic disorder that involves multiple organs and is pathologically considered as a form of vasculitis. The current study aims to assess the metric properties of platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) in assessing BD disease activity. Three-hundred-nineteen patients with BD were enrolled in this cross-sectional study. Demographic and epidemiological data, including IBDDAM, time since the onset, and medication and manifestation history were recorded. Complete blood counts (CBC), NLR, and PLR were assessed by analyzing blood samples. On the last visit, patients were assessed for active manifestations of disease. IBDDAM and ocular IBDAAM scores were calculated for activity of disease in each patient. Both PLR and NLR were higher in patients with active BD (Mann–Whitney U test, p-value < 0.05). Patients with active ocular manifestation had significantly higher NLR and PLR (Mann–Whitney U test, p-value < 0.05). These ratios, however, were not associated with other active BD manifestations. A value of NLR > 2.58 had 46% sensitivity and 85% specificity for the diagnosis of active ocular manifestations (AUC: 0.690). NLR had a significant, though, weak positive correlation with IBDDAM (Spearman’s rho = 0.162; p-value < 0.05) and ocular IBDDAM (Spearman’s rho = 0.159; p-value < 0.05). Active Behçet’s presented with higher NLR and PLR ratios; however, there was only a modest correlation between NLR and BD activity (IBDDAM score). Also, NLR and PLR have significant relationship with ocular features of BD patients.

Diagnosis of intestinal vasculitis is often challenging due to the non-specific clinical and imaging findings. Vasculitides with gastrointestinal (GI) manifestations are rare, but their diagnosis holds immense significance as late or missed recognition can result in high mortality rates. Given the resemblance of radiologic findings with some other entities, GI vasculitis is often overlooked on small bowel studies done using computed tomography/magnetic resonance enterography (CTE/MRE). Hereon, we reviewed radiologic findings of vasculitis with gastrointestinal involvement on CTE and MRE. The variety of findings on MRE/CTE depend upon the size of the involved vessels. Signs of intestinal ischemia, e.g., mural thickening, submucosal edema, mural hyperenhancement, and restricted diffusion on diffusion-weighted imaging, are common in intestinal vasculitis. Involvement of the abdominal aorta and the major visceral arteries is presented as concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels. Such findings can be observed particularly in large- and medium-vessel vasculitis. The presence of extra-intestinal findings, including within the liver, kidneys, or spleen in the form of focal areas of infarction or heterogeneous enhancement due to microvascular involvement, can be another radiologic clue in diagnosis of vasculitis. The link between the clinical/laboratory findings and MRE/CTE abnormalities needs to be corresponded when it comes to the diagnosis of intestinal vasculitis.