Systemic Rheumatic Diseases Research Articles

Sexual dysfunction in women with systemic autoimmune rheumatic disorders: a systematic review and meta-analysis.

In women with systemic autoimmune rheumatic diseases (SARDs), female sexual dysfunction (SD) remains underestimated. We aimed to explore the prevalence and correlates of SD in females with SARDs. We performed a systematic review and meta-analysis of studies assessing the prevalence of SD and the pooled Female Sexual Function Index (FSFI) scores in this setting (PROSPERO: CRD42021287346). We searched PubMed, EMBASE and Cochrane Central Register of Controlled Trials (CENTRAL) databases and grey literature until February 2022. We evaluated the quality of the selected records using the Hoy Risk of Bias tool. A random-effects meta-analysis of proportions with the double arcsine transformation was conducted. Subgroup and sensitivity analyses, as well as meta-regression of important correlates, were conducted. We included 68 studies with 5457 females diagnosed with a SARD (mean age: 43.7 [12.9] years). The overall SD prevalence was 63% (95% CI: 56, 69%, I2 = 94%) and the overall FSFI total score was 19.7 points (95% CI: 18.4, 21, I2 = 97%). Including only sexually active females, the SD prevalence was estimated as 60% (95% CI: 53, 67%, I2 = 88%), whereas the FSFI total score was 22 points (95% CI: 20.8, 23.1, I2 = 93%). Across the different SARDs, women with Sjögren's syndrome and systemic sclerosis reported the highest levels of SD (74%, 95% CI: 58, 87%, I2 = 84% and 69%, 95% CI: 54, 83%, I2 = 94%, respectively). Sexual function in females with SARDs seems to be severely impaired, irrespective of the type of SARD. Screening and treatment of SD in females with SARDs should become an integral part of healthcare clinical practice.

Temporal trends in COVID-19 outcomes among patients with systemic autoimmune rheumatic diseases: from the first wave through the initial Omicron wave

ObjectivesTo investigate temporal trends in incidence and severity of COVID-19 among patients with systemic autoimmune rheumatic diseases (SARDs) from the first wave through the initial Omicron wave.MethodsWe conducted a retrospective...

Impact of kidney biopsy on the management of patients in the rheumatology department: retrospective study

Kidney involvement is a common manifestation of the systemic autoimmune rheumatic diseases. Kidney biopsy is the gold standard for the diagnosis of kidney diseases, however this method has not yet become the standard-of-care in rheumatology practice. To assess the diagnostic value of kidney biopsy in the management of patients of the rheumatology department. In this retrospective observational study we analyzed the medical documentation including kidney morphology findings in the patients of the Department of Rheumatology at Tareev Clinic of Internal Diseases. All patients included in the research had signs of kidney involvement and had undergone needle biopsy of the kidney or re-evaluation of the kidney tissue received previously. From June 2016 to October 2021, 3110 patients were admitted to the rheumatology department. Among them 63 (2%) underwent kidney biopsy and were included in the study. Twenty (32%) were male. Mean age was 42.513.9 years. The most common preliminary diagnoses before kidney biopsy were ANCA-associated vasculitis (n=17), systemic lupus erythematosus (n=12), and AA-amyloidosis associated with inflammatory joint diseases (n=7). In 14 (27%) patients diagnosis was unspecified at the time of biopsy. Among 49 patients with established preliminary diagnosis morphological findings were in line 38 (78%) with the pre-liminary diagnosis. However, in 11 (22%) patients morphological findings resulted in the change of the diagnosis. In all 14 patients with unspecified condition kidney biopsy helped to establish clinical diagnosis. Ultrasound evaluation demonstrated hematoma formation in 18 (31%) patients, and among them two required blood component transfusions. Our study demonstrates significant value and safety of kidney biopsy in the patients with autoimmune rheumatic conditions. We suggest that kidney biopsy should be implemented in the management of this category of patients.

Different COVID-19 outcomes among systemic rheumatic diseases: a nation-wide cohort study.

ObjectivesTo investigate Covid-19-associated risk of hospitalization and death in rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis (PsA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) in comparison with the general population during pandemic’s first year and compare their overall mortality with 2019.MethodsInterlinking nation-wide electronic registries, we recorded confirmed Covid-19-associated infections, hospitalizations and deaths, and all-cause deaths between 1-March-2020 and 28-February-2021 in all adults with RA, AS, PsA, SLE, and SSc under treatment (n = 74 970, median age 67.5, 51.2, 58.1, 56.2, 62.2 years, respectively) and in matched (1:5) on age, sex, and region of domicile random comparators from the general population. Deaths from all causes during 2019 were also recorded.ResultsCompared with the general population incidence rates (IR) for Covid-19-associated hospitalization were higher in RA [IR ratio (IRR):1.71(1.50–1.95)], SLE [2.0(1.4–2.7)] and SSc [2.28(1.29–3.90)], while Covid-19-associated death rates were higher in RA [1.91(1.46–2.49)]. When focusing only on SARS-CoV-2 infected subjects, after adjusting for age and gender, the odds ratio for Covid-19 associated death was higher in RA [1.47(1.11– 1.94)] and SSc [2.92(1.07–7.99)] compared with the general population. All-cause mortality rate compared with the general population increased in RA during the first pandemic year (IRR : 0.71) with reference to 2019 (0.59) and decreased in SSc (IRR : 1.94 vs 4.36).ConclusionCovid-19 may have more severe impact in patients with systemic rheumatic disease than the general population. Covid-19-related mortality is increased in subgroups of patients with specific rheumatic diseases, underscoring the need for priority vaccination and access to targeted treatments.

Outcomes of COVID-19 and Factors Associated With Its Severity Among Hospitalized Patients With and Without Systemic Rheumatic Disease During the First Wave of the Pandemic in New York City.

Conflicting data exist regarding whether patients with systemic rheumatic disease (SRD) experience more severe outcomes related to COVID-19. Using data from adult patients hospitalized with COVID-19 in New York City during the first wave of the pandemic, we evaluated whether patients with SRD were at an increased risk for severe outcomes. We conducted a medical records review study including patients aged ≥18 years with confirmed SARS-CoV-2 infection hospitalized at 3 NewYork-Presbyterian sites, March 3-May 15, 2020. Inverse probability of treatment weighting was applied to a multivariable logistic regression model to assess the association between SRD status and the composite of mechanical ventilation, intensive care unit admission, or death. Of 3710 patients hospitalized with COVID-19 (mean [SD] age, 63.7 [17.0] years; 41% female, 29% White, and 34% Hispanic/Latinx), 92 (2.5%) had SRD. Patients with SRD had similar age and body mass index but were more likely to be female, ever smokers, and White or Black, compared with those without SRD. A higher proportion of patients with versus without SRD had hypertension and pulmonary disease, and used hydroxychloroquine, corticosteroids, and immunomodulatory/immunosuppressive medications before admission. In the weighted multivariable analysis, patients with SRD had an odds ratio of 1.24 (95% confidence interval, 1.10-1.41; p < 0.01) for the composite of mechanical ventilation, intensive care unit admission, or death, compared with patients without SRD. During the initial peak of the pandemic in New York City, patients with versus without SRD hospitalized with COVID-19 had a 24% increased likelihood of having severe COVID-19 after multivariable adjustment.

Modern aspects of the problem of obliterating thrombangiitis

To present some pathogenesis, diagnostics and treatment of thromboangiitis obliterans (TAO) discussion items in accordance with the contemporary views on the nomenclature, classification, pathogenesis and treatment of rheumatic diseases. Materials and methods. 115 patients with thromboangiitis obliterans under observation in Interregional Consultative Center for the patients with systemic rheumatic diseases were examined. The complex analysis of peripheral circulation was carried out by ultrasonic scintiangiography. The methods of study included: morphological analysis of biopsy, autopsic and operating material by histochemical methods; immunofluorescent method was used for detecting the immune complexes. In 224 patients with systemic vasculitides (SV) serological markers, including the wide spectrum of autoantibodies, C-reactive protein, complement, von Willebrand factor antigen as a marker of defeat of vascular wall were detected. Results. Most diagnostically significant criteria were: age younger than 45 years, male sex, smoking, distal vascular lesions of the extremities and confirmation of thromboangiitis obliterans by musculocutaneous biopsy. The involvement of small- and medium-sized arteries and also thrombophlebitis and phlebemphraxis were typical. Reliable diagnosis could have been considered when scintiangiography data that testified about the contraction or occlusion of distal arteries of extremities, and the results of biopsy were presented. Morphological substratum was a destructive-productive or productive thrombovasculitis of middleand small-sized arteries and veins. Necrotic destructive-infiltrative and destructive-proliferative arteriitis, accompanied by fibrinoid necrosis of the arterial wall and by its infiltration with neutrophils were revealed with the productive. The thromboses were revealed with these arteriitis more frequently, and in the wall of such arteries immune complexes were observed. Ischemic manifestations were connected not only with arterial stenosis, but also with microangiosclerosis. Differential diagnostic signs of atherosclerosis were represented. Involving of small- and middle-sized arteries was typical for thromboangiitis obliterans and large- and middle- sized arteries — for atherosclerosis. The thrombophlebitis and phlebothrombosis were characteristic for TAO. No specific laboratory tests were available to confirm TAO diagnosis. It should be noted the specific diagnostic tests and positive serological markers, characteristic for other SV nosologic forms were absent. Some questions of nosologic specification, pathogenesis and principles of therapy were discussed. It should also be noted that the spectrum of TAO interpretation changed since the beginning of the XXI century. The disease requires multidistsiplinary approach both in diagnostics and refinement of nosologic belonging, pathogenesis and designation of pathogenetic therapy.

Disease flare in patients with dermatomyositis following COVID-19 vaccination

To the Editor: Since December 2020, COVID-19 vaccinations have effectively prevented severe SARS-CoV-2 disease. The American College of Rheumatology recommends that individuals with systemic autoimmune rheumatic diseases receive COVID-19 vaccination given increased severe illness risk.1 Despite the benefits of vaccination, fear of disease flare postvaccination continued to drive vaccine hesitancy.2

Targeting Multiple End Organs in Lupus and Other Systemic Rheumatic Diseases by Inhibiting Bruton's Tyrosine Kinase.

Bruton tyrosine kinase (Btk) plays a vital role in activating and differentiating B-cells and regulating signaling in myeloid cells. Indeed, the potential use of Btk inhibitors in preventing lupus has been reported. Here, we extend these observations to 4 additional models of end-organ inflammation: (a) BWF1 lupus nephritis mice, (b) anti-GBM nephritis, (c) bleomycin-induced systemic sclerosis like skin disease, and (d) bleomycin-induced lung disease. In agreement with the previous studies, BTK inhibitor (BTKB66) treatment was effective in treating lupus nephritis in terms of reducing renal damage both functionally and histologically, accompanied by significant decrease in proteinuria. Both low-dose and high-dose BTKB66 profoundly blocked renal disease in the anti-GBM nephritis model, with efficacy that was comparable to that seen with dexamethasone. This study provides the first evidence that BTK inhibition has both therapeutic and preventative effects in bleomycin-induced SSc-like disease, in terms of reducing skin thickness, fibrosis, collagen deposition, and inflammation. Likewise, significantly lower lung inflammatory cell infiltration was observed after treatment with BTKB66. Therapeutic benefit was associated with lower numbers of macrophages, proliferating macrophages and activated T-cells in the respective injured organs. The observation that these immune cells play key roles in driving end organ inflammation in multiple systemic rheumatic diseases have broad implications for the use of BTKB66 in managing patients with systemic rheumatic diseases where multiple end organs are afflicted, including lupus and systemic sclerosis.

Immunity after COVID-19 Recovery and Vaccination: Similarities and Differences.

The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with a robust immune response. The development of systemic inflammation leads to a hyperinflammatory state due to cytokine release syndrome during severe COVID-19. The emergence of many new SARS-CoV-2 variants across the world deteriorates the protective antiviral immunity induced after infection or vaccination. The innate immune response to SARS-CoV-2 is crucial for determining the fate of COVID-19 symptomatology. T cell-mediated immunity is the main factor of the antiviral immune response; moreover, SARS-CoV-2 infection initiates a rapid B-cell response. In this paper, we present the current state of knowledge on immunity after COVID-19 infection and vaccination. We discuss the mechanisms of immune response to various types of vaccines (nucleoside-modified, adenovirus-vectored, inactivated virus vaccines and recombinant protein adjuvanted formulations). This includes specific aspects of vaccination in selected patient populations with altered immune activity (the elderly, children, pregnant women, solid organ transplant recipients, patients with systemic rheumatic diseases or malignancies). We also present diagnostic and research tools available to study the anti-SARS-CoV-2 cellular and humoral immune responses.

Performance of the Revised Classification Criteria for Systemic Autoimmune Rheumatic Diseases and Their Overlap Syndromes.

Objective We evaluated the performance of the revised classification criteria for assessing different systemic autoimmune rheumatic diseases and their overlap syndromes. Methods A total of 652 patients with or highly suspected of having systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM)/dermatomyositis (DM) or rheumatoid arthritis (RA)were included in this study. The 1997 revised American College of Rheumatology (ACR) and the 2019 European League Against Rheumatism (EULAR)/ACR criteria for SLE, the 1980 ACR and the 2013 ACR/EULAR criteria for SSc, the criteria by Bohan and Peter and the 2017 EULAR/ACR criteria for PM/DM, and the 1987 revised ACR and 2011 ACR/EULAR criteria for RA were used for disease classification. Results The old and new criteria and a clinical diagnosis were used to respectively classify 103, 106 and 105 SLE patients; 35, 47 and 58 SSc patients; 18, 23 and 33 PM/DM patients; and 297, 389 and 468 RA patients. Sensitivity increased from 82.9% to 92.4% in SLE, from 56.9% to 79.3% in SSc, from 54.5% to 66.7% in PM/DM, and from 62.6% to 80.8% in RA. SLE-SSc was the predominant type of clinical overlap syndrome, while SLE-RA was the most classifiable. Conclusion The revised classification criteria for all the diseases showed an improved sensitivity, and SLE-overlap syndrome was predominant, regardless of the criteria sets.

The promise of precision medicine in rheumatology.

Systemic autoimmune rheumatic diseases (SARDs) exhibit extensive heterogeneity in clinical presentation, disease course, and treatment response. Therefore, precision medicine - whereby treatment is tailored according to the underlying pathogenic mechanisms of an individual patient at a specific time - represents the 'holy grail' in SARD clinical care. Current strategies include treat-to-target therapies and autoantibody testing for patient stratification; however, these are far from optimal. Recent innovations in high-throughput 'omic' technologies are now enabling comprehensive profiling at multiple levels, helping to identify subgroups of patients who may taper off potentially toxic medications or better respond to current molecular targeted therapies. Such advances may help to optimize outcomes and identify new pathways for treatment, but there are many challenges along the path towards clinical translation. In this Review, we discuss recent efforts to dissect cellular and molecular heterogeneity across multiple SARDs and future directions for implementing stratification approaches for SARD treatment in the clinic.

Iatrogenic Fracture in a Patient of Ankylosing Spondylitis Planned for Surgical Correction of Chin-on-Chest Deformity

Ankylosing spondylitis (AS) is a progressive systemic chronic inflammatory rheumatic disease that causes arthritis of spine and sacroiliac joints and finally results in contracture or bony fusion of these joints. The ankylosed spine has increased propensity to fracture due to rigidity, long lever arms, and osteoporosis. However, iatrogenic fracture occurring in AS patients with chin-on-chest deformity during positioning has not been reported. A 46-year-old male, known case of AS, presented with progressive deformity of neck for the last 6 months due to C5 fracture with C4/5 dislocation. He had chin-on-chest deformity with chin-brow vertical angle of 106o with spastic tetraparesis (mJOA score 6, Nurick grade-3). The patient was put on sustained halo-gravity traction by gradually increasing the weight up to 30 lbs for 4 weeks and then was planned for deformity correction. After intubation and application of same traction, we found anterior wedge opening and fracture through C6/7 disc space. His neurology was found to be same as in the preoperative state on neural monitoring and wake up test. Front and back instrumented fusion was done with anterior bone grafting. Postoperatively, he was maintained on halo-vest immobilization for three months. By the end of first year, the fracture had united well and he was mobilizing well with mJOA score 13 and Nurick grade-1. AS patients are at higher risk of fracture and need utmost care while positioning during surgery. Traction or any corrective maneuver must be done carefully.

A Brief Acceptance and Commitment Therapy Group Intervention on Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a systemic autoimmune rheumatic disease of unknown etiology that mainly affects women of reproductive age. SLE causes a series of physical and psychological symptoms that, due to its chronic course, lead to a diminished quality of life. Acceptance and Commitment Therapy (ACT) is accumulating evidence supporting its usefulness in improving the psychological problems associated with SLE although research remains scarce. The aim of this study was to study the efficacy of a 10-hour brief ACT intervention protocol in group format in 15 patients with SLE from Navarra. Nine belonged to the experimental group (n1 = 9) and six to the control group (n2 = 6). Measures related to psychological flexibility processes and quality of life were taken before the intervention (pre), at the end (post), at one month and after three months (follow-up). The participants in the experimental group showed a substantial improvement in the degree of acceptance of pain and in variables focused on quality of life with respect to the control group. The improvements tended to be sustained over time. These results suggest that brief, group-based ACT interventions show promise for intervention in patients with SLE.

Clinical characteristics of idiopathic interstitial pneumonias with anti-Ro52/tripartite motif-containing 21 antibodies

Antibodies to Ro52/tripartite motif-containing 21 (TRIM21), referred to as anti-Ro52, are found in patients diagnosed with diverse systemic autoimmune rheumatic disease and associated with interstitial lung diseases. However, little is known about the clinical characteristics of anti-Ro52 in patients with idiopathic interstitial pneumonias (IIPs). We aimed to analyze the prevalence, co-existent autoantibodies, and clinical characteristics of anti-Ro52 in patients with IIP. The study enrolled 288 patients diagnosed with IIP. Clinical, laboratory and radiographic findings of IIP patients were compared between anti-Ro52 positives and negatives. Anti-Ro52 (20/288; 6.9%), anti-ARS (18/288; 6.3%), and anti-Ro60/SS-A (16/288; 5.6%) were the most common autoantibodies detected in IIP patients. Among 20 IIP patients who had anti-Ro52, anti-ARS was present in 8 (40%) patients. The criteria for interstitial pneumonia with autoimmune features (IPAF) were significantly better fulfilled by patients with anti-Ro52 than those without (P = 0.001). Meeting serological domain (P < 0.001) and Raynaud’s phenomenon (P = 0.009) were significantly more common in the anti-Ro52-positive patients. Anti-Ro52-positive IIP patients have clinical features consistent with IPAF. Anti-Ro52 may have an important role in detecting the autoimmune phenotype in IIP patients.

Altered Balance of Pro-Inflammatory Immune Cells to T Regulatory Cells Differentiates Symptomatic From Asymptomatic Individuals With Anti-Nuclear Antibodies.

Systemic Autoimmune Rheumatic Diseases (SARDs) are characterized by the production of anti-nuclear antibodies (ANAs). ANAs are also seen in healthy individuals and can be detected years before disease onset in SARD. Both the immunological changes that promote development of clinical symptoms in SARD and those that prevent autoimmunity in asymptomatic ANA+ individuals (ANA+ NS) remain largely unexplored. To address this question, we used flow cytometry to examine peripheral blood immune populations in ANA+ individuals, with and without SARD, including 20 individuals who subsequently demonstrated symptom progression. Several immune populations were expanded in ANA+ individuals with and without SARD, as compared with ANA- healthy controls, particularly follicular and peripheral T helper, and antibody-producing B cell subsets. In ANA+ NS individuals, there were significant increases in T regulatory subsets and TGF-ß1 that normalized in SARD patients, whereas in SARD patients there were increases in Th2 and Th17 helper cell levels as compared with ANA+ NS individuals, resulting in a shift in the balance between inflammatory and regulatory T cell subsets. Patients with SARD also had increases in the proportion of pro-inflammatory innate immune cell populations, such as CD14+ myeloid dendritic cells, and intermediate and non-classical monocytes, as compared to ANA+ NS individuals. When comparing ANA+ individuals without SARD who progressed clinically over the subsequent 2 years with those who did not, we found that progressors had significantly increased T and B cell activation, as well as increased levels of LAG3+ T regulatory cells and TGF-ß1. Collectively, our findings suggest that active immunoregulation prevents clinical autoimmunity in ANA+ NS and that this becomes impaired in patients who progress to SARD, resulting in an imbalance favoring inflammation.

Sudden hearing loss as initial manifestation in patients with systemic autoimmune rheumatic diseases: case series

Sudden hearing loss as initial manifestation in patients with systemic autoimmune rheumatic diseases: case series

Fever Correlation with Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) Concentrations in Patients with Isolated Polymyalgia Rheumatica (PMR): A Retrospective Comparison Study between Hospital and Out-of-Hospital Local Registries.

Background: Polymyalgia rheumatica (PMR) is the most common systemic inflammatory rheumatic disease affecting the elderly. Giant cell arteritis (GCA) is a granulomatous vasculitis affecting the aorta and its branches associated with PMR in up to 20% of cases. In recent studies based on university hospital registries, fever correlated with the erythrocyte sedimentation rate (ESR) but not with C-reactive protein (CRP) concentrations at the time of diagnosis in patients with isolated PMR. A long delay to a PMR diagnosis was suggested to explain this discrepancy, possibly caused by laboratory alterations (for instance, anemia of chronic disease type) that can influence only ESR. We performed a retrospective comparison study between the university hospital and two out-of-hospital public ambulatory databases, searching for any differences in fever/low-grade fever correlation with ESR and CRP. Methods: We identified all patients with newly diagnosed PMR between 2013 and 2020, only including patients who had a body temperature (BT) measurement at the time of diagnosis and a follow-up of at least two years. We considered BT as normal at <37.2 °C. Routine diagnostic tests for differential diagnostics were performed at the time of diagnosis and during follow-ups, indicating the need for more in-depth investigations if required. The GCA was excluded based on the presence of suggestive signs or symptoms and routine ultrasound examination of temporal, axillary, subclavian, and carotid arteries by experienced ultrasonographers. Patients with malignancies, chronic renal disease, bacterial infections, and body mass index (BMI) > 30 kg/m2 were excluded, as these conditions can increase CRP and/or ESR. Finally, we used the Cumulative Illness Rating Scale (CIRS) for quantifying the burden of comorbidities and excluded patients with a CIRS index > 4 as an additional interfering factor. Results: We evaluated data from 169 (73 from hospital and 96 from territorial registries) patients with newly diagnosed isolated PMR. Among these, 77.7% were female, and 61.5% of patients had normal BT at the time of diagnosis. We divided the 169 patients into two cohorts (hospital and territorial) according to the first diagnostic referral. Age at diagnosis, ESR, CRP, median hemoglobin (HB), and diagnostic delay (days from first manifestations to final diagnosis) were statistically significantly different between the two cohorts. However, when we assessed these data according to BT in the territorial cohort, we found a statistical difference only between ESR and BT (46.39 ± 19.31 vs. 57.50 ± 28.16; p = 0.026). Conclusions: ESR but not CRP correlates with fever/low-grade fever at the time of diagnosis in PMR patients with a short diagnosis delay regardless of HB levels. ESR was the only variable having a statistically significant correlation with BT in a multilevel regression analysis adjusted for cohorts (β = 0.312; p = 0.014).

Long-term exposure to fine particulate matter and ozone and the onset of systemic autoimmune rheumatic diseases: an open cohort study in Quebec, Canada

ObjectivesTo estimate associations between fine particulate matter (PM2.5) and ozone and the onset of systemic autoimmune rheumatic diseases (SARDs).MethodsAn open cohort of over 6 million adults was constructed from provincial physician billing and hospitalization records between 2000 and 2013. We defined incident SARD cases (SLE, Sjogren’s syndrome, scleroderma, polymyositis, dermatomyositis, polyarteritis nodosa and related conditions, polymyalgia rheumatic, other necrotizing vasculopathies, and undifferentiated connective tissue disease) based on at least two relevant billing diagnostic codes (within 2 years, with at least 1 billing from a rheumatologist), or at least one relevant hospitalization diagnostic code. Estimated PM2.5 and ozone concentrations (derived from remote sensing and/or chemical transport models) were assigned to subjects based on residential postal codes, updated throughout follow-up. Cox proportional hazards models with annual exposure levels were used to calculate hazard ratios (HRs) for SARDs incidence, adjusting for sex, age, urban-versus-rural residence, and socioeconomic status.ResultsThe adjusted HR for SARDS related to one interquartile range increase in PM2.5 (3.97 µg/m3) was 1.12 (95% confidence interval 1.08–1.15), but there was no clear association with ozone. Indirectly controlling for smoking did not alter the findings.ConclusionsWe found associations between SARDs incidence and PM2.5, but no relationships with ozone. Additional studies are needed to better understand interplays between the many constituents of air pollution and rheumatic diseases.

Autoimmune liver diseases in systemic rheumatic diseases.

Systemic rheumatic diseases (SRDs) are chronic, inflammatory, autoimmune disorders with the presence of autoantibodies that may affect any organ or system. Liver dysfunction in SRDs can be associated with prescribed drugs, viral hepatitis, alternative hepatic comorbidities and coexisting autoimmune liver diseases (AILDs), requiring an exclusion of secondary conditions before considering liver involvement. The patterns of overlap diseases depend predominantly on genetic determinants with common susceptible loci widely distributing in both disorders. In AILDs, it is important to identify the overlapping SRDs at an early stage since such a coexistence may influence the disease course and prognosis. Commonly co-occurring SRDs in AILDs are Sjögren syndrome (SS), rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) in autoimmune hepatitis (AIH), and SS, RA or systemic sclerosis in primary biliary cholangitis. Owing to different disease complications and therapies, it is imperative to differentiate between SLE liver involvement and SLE-AIH overlap disease. Therapeutic options can be personalized to control coexisting conditions of liver autoimmunity and rheumatic manifestations in AILD-SRD overlap diseases. The collaboration between hepatologists and rheumatologists can lead to significant advances in managing such a complex scenario. In this review, we provide a comprehensive overview on coexisting AILDs in different SRDs and the therapeutic approach in managing these overlap diseases.

КЛИНИКО-ДИАГНОСТИЧЕСКОЕ ЗНАЧЕНИЕ АНТИНУКЛЕАРНЫХ АНТИТЕЛ В ПРАКТИКЕ ПЕДИАТРА (ЧАСТЬ I)

In recent decades, there has been an increase in the incidence of various autoimmune, including rheumatic diseases, while their debut has been shifted to an earlier age. The clinical picture of rheumatic diseases is distinguished by severe polymorphism; therefore, laboratory diagnostic methods are important for diagnostic search. One of the most widely used tests in the rheumatological practice is the detection and identification of antinuclear antibodies (ANA). ANA are a large group of autoantibodies (auto-Abs) directed against various cell structures, including not only the nucleus but also the nuclear membrane, mitotic apparatus, cytoplasm components and cell organelles, as well as cell membranes. Different methods are used for testing, but the method of ANA detection through indirect immunofluorescence antinuclear antibody test (IF-ANA) is recommended as a “gold standard” with human laryngeal carcinoma HEp-2 cells being recognized as a standard substrate. IF-ANA with HEp-2 has the highest diagnostic sensitivity compared with other methods, it includes the phase of serial dilutions of positive serums and a visual analysis of the type of glow, the list of which currently has 30 patterns. ANA glow types are caused by various auto-Abs that respond with antigens located in the nucleus and cytoplasm of the HEp-2 cells line. Assessment of the type of glow in conjunction with clinical and laboratory data allows planning of further examination with confirming tests to determine the specificity of ANA, some of which, in particular, are auto-Abs to DFS70 antigen, which in the absence of other types of autoantibodies, are more likely to doubt the presence of a systemic rheumatic disease. The diagnostic specificity of various screening techniques for ANA detection is relatively low, they are detected with a fairly high frequency in patients with other diseases and in healthy individuals of different gender and age, including children.