Systemic Rheumatic Diseases Research Articles

Treat to target in Behcet's disease: Should we follow the paradigm of other systemic rheumatic diseases?

During the last decades the efficacy of biologic agents, mainly of anti-TNFs, in controlling the activity of serious manifestations of Behcet's Disease (BD) has been established. On the other hand, the clinical heterogeneity of BD has precluded the validation of a widely-accepted composite index for disease assessment and for target disease-state definitions, such as low disease activity and remission, and the testing of their implementation in clinical practice. Therefore, in contrast to other systemic rheumatic diseases, a treat-to-target strategy has not yet been developed in BD. There are several challenges towards this approach, including standardization of outcome measures for assessing the disease activity in each-affected organ and construction of a composite disease activity index. The challenges for the development of a treat-to-target strategy and possible solutions are discussed in this position paper, which stemmed from a round table discussion that took place in the 19th International Conference on BD.

Impaired neutralizing antibodies and preserved cellular immunogenicity against SARS-CoV-2 in systemic autoimmune rheumatic diseases

Reports on vaccine immunogenicity in patients with systemic autoimmune rheumatic diseases (SARDs) have been inconclusive. Here, we report the immunogenicity of heterologous prime-boost with an inactivated vaccine followed by an adenoviral vector vaccine in patients with SARDs using anti-RBD antibodies, neutralizing capacity against Omicron BA.2 [plaque-reduction neutralization test (PRNT)], T cell phenotypes, and effector cytokine production at 4 weeks after vaccination. SARD patients had lower median (IQR) anti-RBD-IgG levels and neutralizing function against the Omicron BA.2 variant than the healthy group (p = 0.003, p = 0.004, respectively). T cell analysis revealed higher levels of IFN-γ- and TNF-α-secreting CD4 + T cells (p < 0.001, p = 0.0322, respectively) in SARD patients than in the healthy group. Effector cytokine production by CD8 + T cells was consistent with Th responses. These results suggest that this vaccine regimen revealed mildly impaired humoral response while preserving cellular immunogenicity and may be an alternative for individuals for whom mRNA vaccines are contraindicated.

Association between systemic rheumatic diseases and dementia risk: A meta-analysis

Epidemiological studies have been conducted on the relationship between systemic rheumatic diseases (SRDs) and dementia. Therefore, we focused on determining the extent of alliances bounded by SRDs, along with the risk of dementia. Two independent reviewers assessed all studies retrieved from the PubMed, EMBASE, Scopus, and Web of Science databases between January 1, 2000 and November 30, 2021. Only observational studies that estimated the possibility of dementia in participants with SRD were considered. The random-effects model was applied to forecast pooled risk ratios (RRs) and 95% confidence intervals (CI). Heterogeneity among the studies was evaluated using the Q and I2 statistics. The quality of the included studies was assessed using the Newcastle-Ottawa Scale. Funnel plots were used to calculate the risk of bias. Seventeen observational studies with 17,717,473 participants were recruited. Our findings showed that among the participants with SRDs, those with osteoarthritis, systemic lupus erythematosus, and Sjogren's syndrome were highly related to an elevated risk of dementia (pooled RR: 1.31; 95% CI: 1.15-1.49, p<0.001; pooled RR: 1.43; 95% CI: 1.19-1.73, p<0.001; and pooled RR: 1.26; 95% CI: 1.14-1.39, p<0.001, respectively). However, participants with rheumatoid arthritis (RA) were not associated with an increased risk of dementia (pooled RR: 0.98; 95% CI: 0.90-1.07, p<0.001). This systematic review and meta-analysis demonstrated an increased dementia risk among SRDs participants, except for RA.

Editorial introductions.

Editorial introductions.

Different antibody-associated autoimmune diseases have distinct patterns of T follicular cell dysregulation

Autoantibodies are produced within germinal centers (GC), in a process regulated by interactions between B, T follicular helper (Tfh), and T follicular regulatory (Tfr) cells. The GC dysregulation in human autoimmunity has been inferred from circulating cells, albeit with conflicting results due to diverse experimental approaches. We applied a consistent approach to compare circulating Tfr and Tfh subsets in patients with different autoimmune diseases. We recruited 97 participants, including 72 patients with Hashimoto’s thyroiditis (HT, n = 18), rheumatoid arthritis (RA, n = 16), or systemic lupus erythematosus (SLE, n = 32), and 31 matched healthy donors (HD). We found that the frequency of circulating T follicular subsets differed across diseases. Patients with HT had an increased frequency of blood Tfh cells (p = 0.0215) and a reduced Tfr/Tfh ratio (p = 0.0338) when compared with HD. This was not observed in patients with systemic autoimmune rheumatic diseases (RA, SLE), who had a reduction in both Tfh (p = 0.0494 and p = 0.0392, respectively) and Tfr (p = 0.0003 and p = 0.0001, respectively) cells, resulting in an unchanged Tfr/Tfh ratio. Activated PD-1+ICOS+Tfh and CD4+PD-1+CXCR5–Tph cells were raised only in patients with SLE (p = 0.0022 and p = 0.0054), without association with disease activity. Our data suggest that GC dysregulation, assessed by T follicular subsets, is not uniform in human autoimmunity. Specific patterns of dysregulation may become potential biomarkers for disease and patient stratification.

Complicated ways of diagnosing common variable immune deficiency (case report)

Common variable immunodeficiency (CVID) is one of the most frequent forms of primary immunodeficiencies with predominant antibody deficiency. Unlike most other primary immunodeficiencies, this variant often manifests in adults thus creating certain difficulties in its detection. Manifestations of common variable immune deficiency are very diverse: infectious syndrome (respiratory tract infections, septic arthritis), diarrheal clinical pattern of both infectious and non-infectious genesis, autoimmune syndrome (autoimmune cytopenias, systemic rheumatic diseases), lymphoproliferative syndrome (benign lymphoid proliferation, lymphomas), interstitial lung disease and sarcoidosis-like granulomatous changes are quite common. Due to the lack of a distinct clinical pattern, the common variable immunodeficiency is often diagnosed too late. Such patients are observed for a long time by various specialists, and the absence of pathogenetic therapy (intravenous immunoglobulins) leads to steady progression of the disease and, often, to lethal outcome. The article presents a clinical observation illustrating the difficulties in making this diagnosis. A woman hospitalized with pneumonia presented with a mass in colonic submucosa. After excluding tuberculosis and lymphoma, a diagnosis of colon lipoma was made on the basis of histological examination. A few years later, the patients submandibular lymph nodes were periodically enlarged. Upon repeated histological examination, the diagnosis of granulomatous necrotizing lymphadenitis was established, the patient was referred to a rheumatologist to rule out systemic vasculitis. This diagnosis was not confirmed, but further examination revealed a sharp decrease in the gamma fraction of serum proteins. Therefore, an immunologists counseling was recommended to rule out immunodeficiency. The following blood serum analysis revealed an extremely low IgG and IgM content, with absence of detectable immunoglobulin A. On the basis of these findings, the diagnosis of common variable immune deficiency was made for the first time. Replacement therapy with high-dose intravenous immunoglobulins with control of pre-transfusion Ig levels was recommended, with further transition to a supporting treatment schedule. Hence, one may state that general practitioners are still poorly aware of primary immunodeficiencies, especially if non-infectious manifestations dominating in the clinical pattern. Routine analysis of total protein content and protein fractions can provide information that allows to suspect deficiency of antibodies and, therefore, to assess contents of distinct serum immunoglobulins in order to confirm CVID diagnosis.

A RARE CASE OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-NEGATIVE PAUCI- IMMUNE FOCAL NECROTIZING AND CRESCENTIC GLOMERULONEPHRITIS PRESENTING WITH DIFFUSE ALVEOLAR HEMORRHAGE

A RARE CASE OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-NEGATIVE PAUCI- IMMUNE FOCAL NECROTIZING AND CRESCENTIC GLOMERULONEPHRITIS PRESENTING WITH DIFFUSE ALVEOLAR HEMORRHAGE

Diagnostics of Interstitial Lung Diseases - Practical Instructions with a Focus on Rheumatologic Systemic Diseases

Interstitial lung diseases (ILD) are etiologically heterogeneous with unknown and known causes like rheumatologic systemic diseases differing in their therapeutic and prognostic consequences. In consensus between pulmonologists, rheumatologists, radiologists, and pathologists, we developed practical instructions for ILD diagnosis in rheumatologic systemic diseases, in particular because ILD can present in early stages of rheumatic systemic diseases. ILD diagnosis is based on clinical assessment results including a detailed medical history, physical examination, focused laboratory tests, radiology with a high-resolution computed tomography, lung function, and histopathology also to differentiate it from cardiac and infection associated lung diseases. The ILD diagnosis is made in a multidisciplinary discussion leading to therapeutic and prognostic consequences. The occurrence of acute exacerbations is especially critical. They are often the causes for ILD progression and are associated with considerable mortality.

Indications for diagnostic use of nuclear medicine in rheumatology: A mini-review.

Nuclear medicine techniques allow important insights not only into oncologic, neurologic, and infectious conditions, but also for the assessment of rheumatic diseases. This review provides a brief, update on the potential role of nuclear imaging in rheumatology, especially on 18F-fluorodeoxyglucose (FDG) positron emission tomography for the diagnosis of giant cell arteritis and other large vessel arteritis according to international recommendations. Besides, the potential role of this and other nuclear imaging techniques for the rheumatologic practice are summarized. With 18F-fluoride as tracer for positron emission tomography, a new option for bone scintigraphy comes up, whereas the use of a semiquantitative sialoscintigraphy is no more supported for classification of Sjögren's syndrome according to current recommendations. Other techniques are used for different organ manifestations in systemic rheumatic diseases like for myocardial infarction and apoplectic insult.

Understanding COVID-19 Risk in Patients With Immune-Mediated Inflammatory Diseases: A Population-Based Analysis of SARS-CoV-2 Testing.

ObjectiveTo investigate the incidence of and factors associated with SARS–CoV‐2 testing and infection in immune‐mediated inflammatory disease (IMID) patients versus matched non‐IMID comparators from the general population.MethodsWe conducted a population‐based, matched cohort study among adult residents from Ontario, Canada, from January 2020 to December 2020. We created cohorts for the following IMIDs: rheumatoid arthritis (RA), psoriasis, psoriatic arthritis, ankylosing spondylitis, systemic autoimmune rheumatic diseases, multiple sclerosis (MS), iritis, inflammatory bowel disease (IBD), polymyalgia rheumatica, and vasculitis. Each patient was matched with 5 patients without IMIDs based on sociodemographic factors. We estimated the incidence of SARS–CoV‐2 testing and infection in IMID patients and non‐IMID patients. Multivariable logistic regressions assessed odds of SARS–CoV‐2 infection.ResultsWe studied 493,499 patients with IMIDs and 2,466,946 patients without IMIDs. Patients with IMIDs were more likely to have at least 1 SARS–CoV‐2 test versus patients without IMIDs (27.4% versus 22.7%), but the proportion testing positive for SARS–CoV‐2 was identical (0.9% in both groups). Overall, IMID patients had 20% higher odds of being tested for SARS–CoV‐2 (odds ratio 1.20 [95% confidence interval 1.19–1.21]). The odds of SARS–CoV‐2 infection varied across IMID groups but was not significantly elevated for most IMID groups compared with non‐IMID comparators. The odds of SARS–CoV‐2 infection was lower in IBD and MS and marginally higher in RA and iritis.ConclusionPatients across all IMIDs were more likely to be tested for SARS–CoV‐2 versus those without IMIDs. The risk of SARS–CoV‐2 infection varied across disease subgroups.

A journey inside the elderly-onset primary Sjögren’s syndrome, looking for useful tips for the geriatrician

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune rheumatic disease where xerophthalmia, xerostomia and presence of anti-SSA and anti-SSB antibodies are typical features. Its prevalence is higher in over-65 aged population than in other age groups. In the elderly, pSS diagnosis comes up against a whole series of critical points that may favor its misdiagnosis. The aim of our article is to discuss the most relevant of them: the frequent occurrence of a seronegative subset, the presence of systemic features not related to sicca syndrome, the sicca syndrome as iatrogenic manifestation and the possibility of a biopsy false negative of labial salivary glands.

Anti-DFS70 antibodies in systemic lupus erythematosus: Prevalence in a large Chinese cohort and an unexpected association with anti-dsDNA antibodies by a long-term follow-up.

ObjectiveMonospecific autoantibodies to dense fine speckles 70 (DFS70) antigen are purported to aid in excluding systemic autoimmune rheumatic diseases (SARD) such as systemic lupus erythematosus (SLE). However, the non-isolated anti-DFS70 still has a certain prevalence in SLE patients, and the clinical significance remains unclear. We aimed to investigate the prevalence, clinical relevance, and value of long-term monitoring of anti-DFS70 antibodies in SLE patients.MethodsAnti-DFS70 antibodies were measured by enzyme-linked immunosorbent assay (ELISA) in 851 SLE patients, 211 healthy individuals, and 194 patients with other SARD (except SLE). Demographic, serological, and clinical associations of anti-DFS70 antibodies were analyzed by a stepwise multivariable logistic regression model. The correlation of anti-DFS70 with anti-dsDNA, anti-C1q, and SLE Disease Activity Index 2000 (SLEDAI-2K) was analyzed. Sixty-one SLE patients with follow-up time ranging from 2 to 57 months were measured anti-DFS70 antibodies using both ELISA and line immunoassay. The dynamic variations of anti-DFS70 antibodies were evaluated with anti-dsDNA, anti-C1q, and SLEDAI-2K during the follow-up.ResultsThe prevalence of anti-DFS70 was significantly higher in SLE (20.7% (176/851)) than in healthy individuals (9.5% (20/211), p = 0.0002) and other SARD (10.8% (21/194), p = 0.002). Multivariable analysis revealed that anti-DFS70-positive SLE patients were associated with younger age (odds ratio (OR) = 0.982; 95% confidence interval (CI) = 0.969, 0.995), higher frequencies of anti-dsDNA (OR 1.598; 95% CI 1.107, 2.306) and anti-PCNA (OR 6.101; 95% CI 2.534, 14.688), and higher levels of serum IgG (OR 1.097; 95% CI 1.067, 1.129) and were more likely to be accompanied by mucosal ulcers (OR 5.921; 95% CI 1.652, 21.215). The O.D. value of anti-DFS70 positively correlated with levels of anti-dsDNA (r = 0.183, p < 0.0001) and anti-C1q (r = 0.181, p < 0.0001), respectively, but not with SLEDAI-2K (p = 0.920). During the follow-up, 49 (42 negative and 7 positive) patients remained stable with anti-DFS70 levels. The other 12 patients experienced significant changes in anti-DFS70, and 83.3% (10/12) of them showed similar trends between anti-DFS70 and anti-dsDNA by evaluation of dynamic variations.ConclusionAnti-DFS70 antibodies seem to be prevalent in Chinese SLE patients. The positive association of anti-DFS70 with anti-dsDNA and consistent dynamic variation between anti-DFS70 and anti-dsDNA during the follow-up suggested a potential relationship between anti-DFS70 and anti-dsDNA in patients with SLE.

Systemic lupus erythematosus: history and modernity

Systemic lupus erythematosus (SLE) is a systemic autoimmune rheumatic disease of unknown etiology, characterized by hyperproduction of non-organ-specific autoantibodies to various components of the cell nucleus and the development of immune-inflammatory damage to internal organs. Currently, SLE is considered as a prototype of a systemic human autoimmune pathology, the central mechanism of immunopathogenesis of which is a violation of immunological tolerance to self-antigens, due to a complex interaction of genetic, epigenetic, environmental factors. The publication discusses the history of the study of SLE, the contribution of Russian scientists (V.A. Nasonova and others) to the study of th is problem, current trends in clinical and scientific research related to the improvement of diagnostic criteria and pharmacotherapy of this disease.

Intracellular Sensing of DNA in Autoinflammation and Autoimmunity.

Evidence has shown that DNA is a pathogen-associated molecular pattern, posing a unique challenge in the discrimination between endogenous and foreign DNA. This challenge is highlighted by certain autoinflammatory diseases that arise from monogenic mutations and result in periodic flares of inflammation, typically in the absence of autoantibodies or antigen-specific T lymphocytes. Several autoinflammatory diseases arise due to mutations in genes that normally prevent the accrual of endogenous DNA or are due to mutations that cause activation of intracellular DNA-sensing pathway components. Evidence from genetically modified murine models further support an ability of endogenous DNA and DNA sensing to drive disease pathogenesis, prompting the question of whether endogenous DNA can also induce inflammation in human autoimmune diseases. In this review, we discuss the current understanding of intracellular DNA sensing and downstream signaling pathways as they pertain to autoinflammatory disease, including the development of monogenic disorders such as Stimulator of interferon genes-associated vasculopathy with onset in infancy and Aicardi-Goutières syndrome. In addition, we discuss systemic rheumatic diseases, including certain forms of systemic lupus erythematosus, familial chilblain lupus, and other diseases with established links to intracellular DNA-sensing pathways, and highlight the lessons learned from these examples as they apply to the development of therapies targeting these pathways.

Immunogenicity and safety of the mRNA-based BNT162b2 vaccine in systemic autoimmune rheumatic diseases patients.

BackgroundThe COVID-19 outbreak has led to the rapid development and administration of the COVID-19 vaccines worldwide. Data about the immunogenicity and adverse effects of the vaccine on patients with systemic autoimmune rheumatic diseases (SARDs) is emerging.AimTo evaluate Pfizer/BioNTech (BNT162b2) mRNA-based vaccine second-dose immunogenicity and safety, and the relation between them, in patients with SARDs.MethodsA total of one hundred forty tow adults who received two doses of the BNT162b2 vaccine were included in the study. The SARDs group included Ninety-nine patients and the control group (forty-three participants) comprised a mixture of healthy participants and patients who were seen at the rheumatology clinic for non-SARDs. Anti-SARS-CoV-2 IgG antibodies against the Spike protein were evaluated using a SARS-CoV-2 IgG immunoassay. A level of > 150 AU/mL was considered positive. An adverse effects questionnaire was given to the participants upon their first visit to the clinic after their BNT162b2 vaccination.ResultsOf the 142 participants, 116 were seropositive (81.7%) and 26 (18.3%) were seronegative. Of the seronegative participants, 96.2% were SARDs patients. The proportion of seropositivity in the SARDs patients treated with any immunosuppressant was significantly lower (69.9%) compared to the control group and SARDs patients not receiving immunosuppressants (96.8%). A significant negative correlation between seronegativity and treatment with rituximab, mycophenolate mofetil (MMF), and prednisone was found in the SARDs group (p = 0.004, 0.044, 0.007 respectively). No fever was observed following the BNT162b2 vaccine in seronegative patients, and the frequency of musculoskeletal adverse effects upon the second dose of the BNT162b2 vaccine was significantly higher in seropositive compared to seronegative patients and in the control group compared to the SARDs patients (p = 0.045, p = 0.02 respectively).ConclusionA decline in the immunogenicity to the second dose of BNT162b2 mRNA is seen in patients with SARDs, especially in patients treated with rituximab, MMF, and prednisone. Adverse effects of the vaccine including fever and musculoskeletal symptoms might be a signal for the acquisition of immunity in those patients.Key Points• BNT162b2 mRNA vaccine is less immunogenic in SARDs patients compared to the control group.• Rituximab, prednisone, and mycophenolate mofetil significantly reduced immunogenicity to the vaccine.• There is a correlation between immunogenicity and adverse effects of the vaccine.

Prolonged COVID-19 symptom duration in people with systemic autoimmune rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

ObjectiveWe investigated prolonged COVID-19 symptom duration, defined as lasting 28 days or longer, among people with systemic autoimmune rheumatic diseases (SARDs).MethodsWe analysed data from the COVID-19 Global Rheumatology Alliance Vaccine...

Autoimmune multimorbidity and fatigue in women with idiopathic inflammatory myopathies: an international, patient-reported, e-survey

<h2>Abstract</h2><h3>Background</h3> Idiopathic inflammatory myopathies are a group of rare systemic autoimmune rheumatic diseases with substantial heterogeneity. We aimed to investigate gender differences in patient-reported outcomes and treatment regimens of people with idiopathic inflammatory myopathies. <h3>Methods</h3> This international, patient-reported, e-survey was conducted worldwide. We used data from the COVID-19 vaccination in autoimmune disease (COVAD) study, a large-scale, international, self-reported e-survey assessing the safety of COVID-19 vaccination in patients older than 18 years with autoimmune rheumatic diseases, including idiopathic inflammatory myopathies. The COVAD study was conducted in more than 80 health-care centres, including hospitals, clinics, and universities located in more than 50 countries worldwide and on social media platforms, such as Facebook and Twitter. The COVAD e-survey was open between April 1, 2021, and Dec 31, 2021. We extracted survey data regarding demographics; autoimmune rheumatic disease diagnosis; autoimmune multimorbidity (three or more autoimmune rheumatic disease diagnoses for each patient); current corticosteroid or immunosuppressant use; and patient-reported outcomes, including fatigue and pain Visual Analogue Scale (VAS), and PROMIS short form-physical function 10a (PF-10a). Gender was reported by participants with three options (men, women, or do not wish to disclose). Patient-reported outcomes and corticosteroid or immunosuppressant use were compared between men and women. Participants with inclusion body myositis were analysed separately due to the substantial difference in treatment and disease outcomes compared with other idiopathic inflammatory myopathy subtypes. Factors affecting each patient-reported outcome were determined using multivariable analysis. <h3>Findings</h3> The survey data were extracted on Aug 31, 2021, and 1202 complete responses from participants with idiopathic inflammatory myopathies were analysed. Five patients who did not wish to disclose gender were excluded. 845 (70·6%) of the remaining 1197 were women. Women were younger than men (median 58 years [IQR 48–68] <i>vs</i> 69 years [58–75]; p=0·00010). Autoimmune multimorbidity was more common in women than in men (94 [11·1%] of 845 <i>vs</i> 11 [3·1%] of 352; p<0·0001). Corticosteroid use was similar in men and women with idiopathic inflammatory myopathies (except for inclusion body myositis), whereas the distribution of immunosuppressants was different, with higher hydroxychloroquine use in women (131 [18·3%] of 717 <i>vs</i> 11 [6·9%] of 159 in men; p=0·0082). The median fatigue VAS was significantly higher in women than in men (5 [IQR 3–7] <i>vs</i> 4 [2–6]; p=0·0036), whereas the gender difference in pain VAS (median 3 [IQR 1–5] in women <i>vs</i> 2 [0–4] in men; p=0·064) and PROMIS PF-10a scores (38 [31–45] <i>vs</i> 39 [30–47]; p=0·29) was non-significant. There were no significant differences in patient-reported outcomes and treatment in participants with inclusion body myositis. The multivariable analysis of idiopathic inflammatory myopathies (except for inclusion body myositis) revealed that female sex, residence in high-income countries, a diagnosis of overlap myositis, and autoimmune multimorbidity were independent risk factors for higher fatigue VAS. <h3>Interpretation</h3> Women with Idiopathic inflammatory myopathies frequently have autoimmune multimorbidity and increased fatigue compared with men, calling for greater attention and further research on targeted treatment approaches. <h3>Funding</h3> None.

Assessment of Quality of Life among Patients with Periphera Spondyloarthropathy

Background: Peripheral spondyloarthropathy refers to spondyloarthropathy with predominant peripheral (arthritis, enthesitis or dactylitis) involvement which leads to significant disability. Objective: To assess the quality of life in patient with peripheral spondyloarthropathy (pSpA). Methodology: The study was a cross sectional study conducted in the Department of Physical Medicine and Rehabilitation, BSMMU, Dhaka, from March 2021-February 2022. A total of 105 patients diagnosed with pSpA attending the study place were purposively selected for the study. Patients with concurrent systemic inflammatory rheumatic disease such as RA, SSc, lupus, Dermatomyositis were excluded from the study. Disease activity was measured by the validated Bengali version of The Ankylosing Spondylitis Disease Activity Score (ASDAS), including C-reactive protein (ASDAS-CRP) while health related quality of life was assessed by the validated Bengali version of Short Form Health Survey (SF) 12v2 instrument. Results: The mean age of the study participants was 38.8(±9.8) years where 68 (64.8%) were male. The mean duration of disease was 4.3(±3.3) years where 55 (52.4%) had up to 3 years’ duration of disease. The mean ASDAS-CRP was 3.9 ±0.8 where 69(65.7%) had very high Disease Activity Score. All (100.0%) study participants had arthritis and inflammatory back pain. HLA B27 was present in most of the study participants (N=101, 96.2%) and 99 (94.3%) had enthesitis. The mean Physical Component Summary (PCS) and Mental Component Summary (MCS) was 34.0(±7.8) and 41.3 (±7.7) respectively. There was weak negative correlation between age and PCS of SF-12 scores (r=-0.233, p=0.017). There was negative correlation between duration of the disease and PCS of SF-12 scores (r=-0.339, p&lt;0.001) and MCS (r=-0.290, p=0.003). Again, there was moderate inverse correlation ASDAS-CRP and PCS of SF-12 scores (r=-0.406, p&lt;0.001) and MCS of SF-12 scores (r=- 0.461, p&lt;0.001). Conclusion: Majority of the patients .....

The Co-Relation between Disease Activity and Quality of Life among Patients with Peripheral Spondyloarthropathy

Background: More disability and incapacity to work and a poorer quality of life in spondyloarthritis (SpA) in the presence of enthesitis is associated with higher disease activity. Objective: To find out the co-relation between disease activity and quality of life among patients with peripheral spondyloarthropathy. Methodology: From March 2021 to February 2022, the Department of Physical Medicine and Rehabilitation at BSMMU, Dhaka, performed a cross-sectional research. A total of 105 individuals diagnosed with pSpA who were included in the trial were chosen on purpose. Excluded from the research were patients with concomitant systemic inflammatory rheumatic diseases such as RA, SSc, lupus, and Dermatomyositis. The validated Bengali version of The Ankylosing Spondylitis Disease Activity Score (ASDAS), including C-reactive protein (ASDAS-CRP), was used to measure disease activity, while the validated Bengali version of the Short Form Health Survey (SF) 12v2 was used to evaluate health-related quality of life. Results: The participants' mean age was 38.8(±9.8) years, and 68(64.8%) were male. The mean duration of sickness was 4.3(±3.3) years, with 55.4% of cases lasting less than 3 years. The mean ASDAS-CRP was 3.9(±0.08), with 69(65.7%) individuals having a very high Disease Activity Score. 100% of the individuals in the research experienced arthritis and inflammatory back pain. The majority of research subjects had the HLA B27 gene (N=101, or 96.2%), and 99(94.3%) had enthesitis. Physical Component Summary (PCS) and Mental Component Summary (MCS) mean scores were 34(±7.8) and 41(±7.7), respectively. A slight negative association existed between age and PCS scores on the SF-12 (r=-0.233, p=0.017). There was a negative connection between illness duration and PCS (r=-0.339, p=0.001) and MCS (r=-0.290, p=0.003) SF-12 scores. Again, there was a moderate negative connection between ASDAS-CRP and the PCS and MCS scores of the SF-12 (r=-0.406, p=0.001) and the MCS scores of the SF-12 (r=-0.461, p0.001). Conclusion: The Disease Activity Score of the majority of individuals with peripheral spondyloarthritis was very high. The psychological health of these patients was superior to their physical health. Both physical and mental health components were negatively linked with illness duration. There was a moderate inverse connection between ASDAS-CRP and the Physical Component Summary (PCS) and Mental Component Summary (MCS) scores of the SF-12.

Determination of the main causes, outcome, and prognostic factors of patients with rheumatologic diseases admitted to the medical intensive care unit in Southern Iran.

BackgroundSystemic rheumatic diseases (SRD) are a heterogeneous group of diseases that can involve several organ systems and occasionally requires intensive care unit (ICU) admission because of severe systemic disease, life-threatening organ involvement, or complication of treatment. The objective of this study is to determine the causes, outcome, and prognostic factors of patients with rheumatologic diseases admitted in teaching medical ICUs in southern Iran.MethodsA retrospective case review of all patients with rheumatologic diseases admitted in the academic medical ICUs in two referral hospitals in southern Iran, from March 2015 to January 2020. Patients’ data were documented from their hospital records and the cause of admission, in-hospital outcome, and prognostic factors was evaluated.ResultsNinety-one patients were included, of which 71.4% were female. Systemic lupus erythematosus (54.9%) was the most common disease. Nineteen (20.9%) patients were new cases of rheumatological disease. The most frequent symptom for admittance was dyspnea (54.9%) and hemoptysis (20.9%). The in-hospital mortality rate was 48%, and the leading cause of death was infection (29 patients; 65.5%) followed by disease activity (18 patients; 40.9%). Also, the death of 29.5% of patients was presumed due to both disease activity and infection. Factors associated with mortality included renal insufficiency (p < 0.028), infection (p < 0.001), pneumonia (p < 0.042), dyspnea (p < 0.042), loss of consciousness (p < 0.046), azathioprine consumption (p < 0.004) during 1 month before ICU admission, mechanical ventilation (p < 0.001), renal replacement therapy (p < 0.001), CNS involvement (p < 0.009), and ICU medications such as cyclosporine and azathioprine (0.03 and 0.03, respectively) or treatments such as plasmapheresis (p < 0.018).ConclusionThe ICU mortality rate of patients with SRD was high. Infection and disease exacerbation are the leading reasons for ICU admission in systemic rheumatic diseases. Intensivists must keep in mind that SRD exacerbation may require immunosuppressive agents along with lifesaving interventions, more particularly in newly diagnosed SRDs. Key Points• The ICU mortality rate of patients with SRD was high.• Infection and disease exacerbation are the leading reasons for ICU admission in systemic rheumatic diseases.• 63.8% of our patients fall into this category of new cases of rheumatologic disease and disease flare-up.