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1.
Turk J Med Sci ; 53(5): 1084-1093, 2023.
Article in English | MEDLINE | ID: mdl-38813000

ABSTRACT

Background/aim: Immunosuppressive and immunomodulatory treatments developed in recent years as a result of a better understanding of the pathophysiology of systemic rheumatic diseases (SRDs) improve the prognosis. Despite medical advances, individuals with SRDs at any stage may require intensive care and have a high mortality rate. The aim of this study was to investigate the demographic and clinical characteristics of patients with rheumatic diseases admitted to the intensive care unit (ICU), and the factors associated with the risk of mortality. Materials and methods: This was a retrospective, cross-sectional study that included patients with rheumatic diseases in the medical ICU. Factors of ICU 28-day mortality were identified by multiple-variable logistic analysis. Results: A total of 127 patients with SRDs admitted to the medical ICU were enrolled. Systemic lupus erythematosus (SLE) (32.3%) was the most common diagnosis of SRDs in patients admitted to the ICU. The reasons for admission to the ICU were combined infection and primary SRD flare-up (35.4%), primary SRD flare-up (22%), SRD-unrelated reasons (22%), infection (17.3%), drug side effects (3.9%), and SRD-related complications (0.8%). The most common organ dysfunctions before (49.6%) and during (77.2%) admission to ICU were in the respiratory system. The 28-day mortality was 78 (61.4%). While the maximum procalcitonin, serum lactate, and blood urea nitrogen (BUN) levels were higher in the nonsurvivor group, the platelet and serum albumin levels were statistically significantly lower than those in the survivor group (p < 0.05). Acute respiratory failure (ARF), the presence of septic shock, the need for invasive mechanical ventilation (IMV), BUN level, and low platelet-lymphocyte ratio (PLR) were significant in the final multiple-variable model. Conclusion: Significant predictors of mortality in patients with rheumatic diseases may include ARF, septic shock, the need for IMV, and high BUN and low PLR levels.


Subject(s)
Intensive Care Units , Rheumatic Diseases , Humans , Male , Female , Retrospective Studies , Intensive Care Units/statistics & numerical data , Middle Aged , Cross-Sectional Studies , Rheumatic Diseases/mortality , Rheumatic Diseases/blood , Rheumatic Diseases/complications , Adult , Aged , Hospital Mortality , Lupus Erythematosus, Systemic/mortality , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis
2.
World J Gastroenterol ; 28(23): 2527-2545, 2022 Jun 21.
Article in English | MEDLINE | ID: mdl-35949355

ABSTRACT

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.


Subject(s)
Arthritis, Rheumatoid , Autoimmune Diseases , Hepatitis, Autoimmune , Liver Diseases , Lupus Erythematosus, Systemic , Rheumatic Diseases , Autoimmune Diseases/complications , Autoimmune Diseases/epidemiology , Autoimmunity , Hepatitis, Autoimmune/complications , Hepatitis, Autoimmune/diagnosis , Hepatitis, Autoimmune/drug therapy , Humans , Liver Diseases/complications , Liver Diseases/diagnosis , Liver Diseases/epidemiology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/epidemiology , Rheumatic Diseases/complications , Rheumatic Diseases/epidemiology
3.
Eur J Intern Med ; 98: 83-92, 2022 Apr.
Article in English | MEDLINE | ID: mdl-35151541

ABSTRACT

BACKGROUND: Systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM) and rheumatoid arthritis (RA) are connective tissue diseases (CTD) whose complications can lead to management in the intensive care unit (ICU). OBJECTIVES: To estimate by meta-analysis ICU mortality rates for CTD. METHODS: A systematic literature review was performed to identify articles studying critically ill CTD patients. A random-effects model was chosen for analysis. Pooled proportion mortality was calculated using aggregated-data meta-analysis with a random-effects model and assessment of heterogeneity with the I2 statistic. Risk of bias was assessed using the quality assessment tool. RESULTS: Of the 5694 individual publications, a sample of 31 independent cohorts was used for the meta-analysis totalling 5007 patients. The main cause for admission was sepsis (43%) followed by "flare-ups" (40%). The overall pooled proportion of mortality of CTD patients across all 31 studies was 33% (95%CI: 28-38%). In the IIM subgroup and that of SSc, mortality was 70% (95%CI: 46-86%) and 40% (95%CI: 25-47%), respectively. In the SLE subgroup, mortality was similar to the overall pooled mortality of 35% (95%CI: 29-42%). Subgroup mortality for RA and pSS patients was respectively 20% (95%CI: 11-33%) and 17% (95%CI: 6-41%); lower than the overall pooled mortality. Heterogeneity in each subgroup remained high. CONCLUSION: The overall pooled proportion of mortality of ICU patients with CTD was 33% (95%CI: 28-38%), with a high heterogeneity (I2= 89%). In the subgroup analysis, mortality was higher for patients with IIM and SSc.


Subject(s)
Arthritis, Rheumatoid , Connective Tissue Diseases , Lupus Erythematosus, Systemic , Myositis , Scleroderma, Systemic , Sjogren's Syndrome , Arthritis, Rheumatoid/complications , Connective Tissue Diseases/complications , Critical Illness , Humans , Lupus Erythematosus, Systemic/complications , Risk Factors , Scleroderma, Systemic/complications , Sjogren's Syndrome/complications
4.
J Clin Med ; 10(5)2021 Mar 03.
Article in English | MEDLINE | ID: mdl-33802364

ABSTRACT

Critically ill patients with systemic rheumatic diseases (SRDs) have a fair prognosis, while those with interstitial lung disease (ILD) have a poorer outcome. However, the prognosis of SRD patients with ILD admitted to the intensive care unit (ICU) remains unclear. We conducted a case-control study to investigate the outcomes of critically ill SRD-ILD patients. Consecutive SRD-ILD patients admitted to five ICUs from January 2007 to December 2017 were compared to SRD patients without ILD. Mortality rates were compared between groups, and prognostic factors were then identified. One hundred and forty critically ill SRD patients were included in the study. Among the 70 patients with SRD-ILD, the SRDs were connective tissue diseases (56%), vasculitis (29%), sarcoidosis (13%), and spondylarthritis (3%). Patients were mainly admitted for acute exacerbation of SRD-ILD (36%) or infection (34%). ICU, in-hospital, and one-year mortality rates in SRD-ILD patients were higher than in SRD patients without ILD (n = 70): 40% vs. 16% (p < 0.01), 49% vs. 19% (p < 0.01), and 66% vs. 40% (p < 0.01), respectively. Hypoxemia, high sequential organ failure assessment (SOFA) score, and admission for ILD acute exacerbation were associated with ICU mortality. In conclusion, ILD worsened the outcomes of SRD patients admitted to the ICU. Admissions related to SRD-ILD acute exacerbation and the severity of the acute respiratory failure were associated with ICU mortality.

5.
Ann Intensive Care ; 11(1): 29, 2021 Feb 09.
Article in English | MEDLINE | ID: mdl-33559763

ABSTRACT

BACKGROUND: Systemic rheumatic diseases (SRDs) are a group of inflammatory disorders that can require intensive care unit (ICU) admission because of multiorgan involvement with end-organ failure(s). Critically ill SRD patients requiring extracorporeal membrane oxygenation (ECMO) were studied to gain insight into their characteristics and outcomes. METHODS: This French monocenter, retrospective study included all SRD patients requiring venovenous (VV)- or venoarterial (VA)-ECMO admitted to a 26-bed ECMO-dedicated ICU from January 2006 to February 2020. The primary endpoint was in-hospital mortality. RESULTS: Ninety patients (male/female ratio: 0.5; mean age at admission: 41.6 ± 15.2 years) admitted to the ICU received VA/VV-ECMO, respectively, for an SRD-related flare (n = 69, n = 38/31) or infection (n = 21, n = 10/11). SRD was diagnosed in-ICU for 31 (34.4%) patients. In-ICU and in-hospital mortality rates were 48.9 and 51.1%, respectively. Nine patients were bridged to cardiac (n = 5) or lung transplantation (n = 4), or left ventricular assist device (n = 2). The Cox multivariable model retained the following independent predictors of in-hospital mortality: in-ICU SRD diagnosis, day-0 Simplified Acute Physiology Score (SAPS) II score ≥ 70 and arterial lactate ≥ 7.5 mmol/L for VA-ECMO-treated patients; diagnosis other than vasculitis, day-0 SAPS II score ≥ 70, ventilator-associated pneumonia and arterial lactate ≥ 7.5 mmol/L for VV-ECMO-treated patients. CONCLUSIONS: ECMO support is a relevant rescue technique for critically ill SRD patients, with 49% survival at hospital discharge. Vasculitis was independently associated with favorable outcomes of VV-ECMO-treated patients. Further studies are needed to specify the role of ECMO for SRD patients.

6.
Clin Rheumatol ; 39(11): 3479-3488, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32372293

ABSTRACT

OBJECTIVE: The etiologies of acute respiratory failure in patients with systemic rheumatic diseases (SRDs) requiring intensive care remain unknown. This study was undertaken to investigate the etiologies and outcomes. METHODS: A medical records review study was performed of 259 adult SRDs patients with respiratory failure admitted to medical ICU across a 5-year period. The etiologies were classified as infection, SRD exacerbation, and undetermined. The factors associated with ICU mortality were identified with multivariate logistic regression analysis. RESULTS: The etiologies of respiratory failure included infection (n = 209, 80.7%), SRD exacerbation (n = 71, 27.4%), and undetermined (n = 21, 8.1%). The most common pathogen was Pneumocystis jirovecii (39.8%), followed by Aspergillus spp. (33.2%), and cytomegalovirus (23.2%). The ICU mortality rate was 59.8%. A high acute physiology and chronic health evaluation II score (OR 1.118, 95% CI 1.054 to 1.186, p < 0.001), a PaO2/FiO2 ratio < 100 mmHg (OR 3.918, 95% CI 2.199 to 6.892, p < 0.001), and a diagnosis of dermatomyositis/polymyositis (OR 4.898, 95% CI 1.949 to 12.309, p = 0.001), vasculitis (OR 3.007, 95% CI 1.237 to 7.309, p = 0.015), and Pneumocystis pneumonia (OR 2.345, 95% CI 1.168 to 4.705, p = 0.016) were associated with increased mortality. CONCLUSIONS: Opportunistic infections and SRD exacerbation were the most common etiologies of acute respiratory failure in patients with SRDs requiring ICU admission, with high ICU mortality. Development of a standard protocol for differential diagnosis in this population might help initiate definitive therapy and improve clinical outcome. Key Points • Infections, especially with opportunistic infections, were the leading cause of acute respiratory failure in critically ill rheumatology patients, with high mortality. • Severity of illness, certain types of rheumatic diseases, and opportunistic fungal infections were associated with increased mortality. • Using a comprehensive diagnostic workup might help to confirm the infective etiology and improve outcome.


Subject(s)
Respiratory Insufficiency , Rheumatology , Adult , Critical Care , Hospital Mortality , Humans , Intensive Care Units , Medical Records , Respiratory Insufficiency/epidemiology , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy
7.
Chest ; 158(3): 1017-1026, 2020 09.
Article in English | MEDLINE | ID: mdl-32289313

ABSTRACT

BACKGROUND: Critically ill patients with systemic rheumatic disease (SRD) have benefited from better provision of rheumatic and critical care in recent years. Recent comprehensive data regarding in-hospital mortality rates and, most importantly, long-term outcomes are scarce. RESEARCH QUESTION: The aim of this study was to assess short and long-term outcome of patients with SRD who were admitted to the ICU. STUDY DESIGN AND METHODS: All records of patients with SRD who were admitted to ICU between 2006 and 2016 were reviewed. In-hospital and one-year mortality rates were assessed, and predictive factors of death were identified. RESULTS: A total of 525 patients with SRD were included. Causes of admission were most frequently shock (40.8%) and acute respiratory failure (31.8%). Main diagnoses were infection (39%) and SRD flare-up (35%). In-hospital and one-year mortality rates were 30.5% and 37.7%, respectively. Predictive factors that were associated with in-hospital and one-year mortalities were, respectively, age, prior corticosteroid therapy, simplified acute physiology score II ≥50, need for invasive mechanical ventilation, or need for renal replacement therapy. Knaus scale C or D and prior conventional disease modifying antirheumatic drug therapy was associated independently with death one-year after ICU admission. INTERPRETATION: Critically ill patients with SRD had a fair outcome after an ICU stay. Increased age, prior corticosteroid therapy, and severity of critical illness were associated significantly with short- and long-term mortality rates. The one-year mortality rate was also associated with prior health status and conventional disease modifying antirheumatic drug therapy.


Subject(s)
Critical Illness , Intensive Care Units , Rheumatic Diseases/mortality , Rheumatic Diseases/therapy , APACHE , Adrenal Cortex Hormones/administration & dosage , Age Factors , Female , France/epidemiology , Hospital Mortality , Humans , Length of Stay/statistics & numerical data , Male , Middle Aged , Organ Dysfunction Scores , Renal Replacement Therapy , Respiration, Artificial , Retrospective Studies , Risk Factors
8.
Arthritis Res Ther ; 21(1): 211, 2019 10 12.
Article in English | MEDLINE | ID: mdl-31604447

ABSTRACT

OBJECTIVE: To estimate and compare the burdens of opportunistic infections and herpes zoster in real-world practice among patients with various systemic rheumatic diseases. METHODS: This 13-year cohort study used national health insurance data to compare the incidence rates (IRs) of nine opportunistic infections among patients with five rheumatic diseases. The analyses were stratified according to follow-up duration using Poisson regression, and Cox models were used to compare the risk of first opportunistic infection. RESULTS: During 2000-2013, we identified 76,966 patients who had polymyositis/dermatomyositis (PM/DM, 2270 cases), systemic lupus erythematosus (SLE, 15,961 cases), systemic sclerosis (SSc, 2071 cases), rheumatoid arthritis (RA, 38,355 cases), or primary Sjögren's syndrome (pSS, 18,309 cases). The IR of opportunistic infections was highest for PM/DM cases (61.3/1000 person-years, 95% confidence interval [CI] 56.6-66.2), followed by SLE cases (43.1/1000 person-years, 95% CI 41.7-44.5), SSc cases (31.6/1000 person-years, 95% CI 28.3-35.1), RA cases (25.0/1000 person-years, 95% CI 24.4-25.7), and pSS cases (24.1/1000 person-years, 95% CI 23.1-25.2). Multivariable Cox analysis revealed that, relative to SLE, PM/DM was associated with a significantly higher risk of opportunistic infections (hazard ratio 1.18, 95% CI 1.08-1.29). The risk of opportunistic infections was highest during the first year after the diagnosis of all five rheumatic diseases. CONCLUSIONS: The risk of opportunistic infection was highest for PM/DM, followed by SLE, SSc, RA, and pSS. Careful observation and preventive therapy for opportunistic infections may be warranted in selected PM/DM patients, especially during the first year after the diagnosis.


Subject(s)
Cost of Illness , Opportunistic Infections/diagnosis , Opportunistic Infections/epidemiology , Rheumatic Diseases/diagnosis , Rheumatic Diseases/epidemiology , Adult , Aged , Cohort Studies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Opportunistic Infections/therapy , Retrospective Studies , Rheumatic Diseases/therapy , Taiwan/epidemiology
9.
Semin Arthritis Rheum ; 49(3): 485-492, 2019 12.
Article in English | MEDLINE | ID: mdl-31153707

ABSTRACT

OBJECTIVE: Serum C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) are the two most commonly used markers of inflammation in clinical practice. Reducing the need for these tests could lead to considerable cost savings without sacrificing the quality of patient care. METHODS: The electronic medical records of patients with systemic rheumatic diseases seen between May 2015 and June 2017 in the rheumatology clinics at a single academic medical center were retrospectively reviewed. Correlations and receiver operator characteristic (ROC) curves between serum CRP level and ESR vs serum globulin gap (the difference between levels of total protein and albumin) and albumin-to-globulin (A:G) ratio were determined. RESULTS: In two independent cohorts (discovery: 263 subjects, 446 entries; validation: 438 subjects, 1959 entries), the globulin gap and A:G ratio correlated (p < 0.001) with CRP level and ESR, with correlation coefficients being greater for ESR than for CRP level. ROC curve analyses demonstrated better area-under-curve for ESR than for CRP level. The percentages of entries with elevated globulin gap (≥4.0 g/dl) and low A:G ratio (<0.8) were ∼8.4% and ∼2.6%, respectively, and each had a positive predictive value of ≥0.960 for elevated ESR. Among patients with high globulin gap, the change in globulin gap over time faithfully reflected changes in ESR. CONCLUSION: In the subset of systemic rheumatic disease patients who harbor an elevated globulin gap, the ESR is almost always elevated. This novel observation sets the conceptual foundation and rationale for subsequent prospective studies that assess whether ESR testing in this subset of rheumatic disease patients could be reduced without sacrificing patient care. Ultimately, ordering an ESR test may often be unnecessary, thereby resulting in cost savings.


Subject(s)
C-Reactive Protein/metabolism , Rheumatic Diseases/blood , Serum Globulins/metabolism , Biomarkers/blood , Blood Sedimentation , Disease Progression , Female , Humans , Male , Middle Aged , Prognosis , ROC Curve , Retrospective Studies
10.
Acta Med Okayama ; 73(1): 85-89, 2019 Feb.
Article in English | MEDLINE | ID: mdl-30820060

ABSTRACT

Pneumocystis pneumonia (PCP) due to Pneumocystis jirovecii infection is the leading cause of fatal opportunistic infections in immunocompromised patients. We will determine whether a daily sulfamethoxazole-trimethoprim (SMX/TMP) dose of 200/40 mg was non-inferior to 400/80 mg for PCP prevention in patients with systemic rheumatic disease under immunosuppressive therapy. This is a randomized, open-label, multicenter controlled trial. The primary outcome is the rate of PCP prevention at 52 weeks. The secondary outcome is the discontinuation rate of SMX/TMP. The trial will evaluate the optimal dose of SMX/TMP for PCP prevention in patients with systemic rheumatic disease under immunosuppressive therapy.


Subject(s)
Anti-Bacterial Agents/therapeutic use , Pneumonia, Pneumocystis/prevention & control , Randomized Controlled Trials as Topic , Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use , Adult , Anti-Bacterial Agents/administration & dosage , Dose-Response Relationship, Drug , Female , Humans , Male , Multicenter Studies as Topic , Trimethoprim, Sulfamethoxazole Drug Combination/administration & dosage
11.
Semin Arthritis Rheum ; 49(1): 27-34, 2019 08.
Article in English | MEDLINE | ID: mdl-30745021

ABSTRACT

OBJECTIVE: To review the current literature, and evaluate the psychometric properties of disease damage indices in rheumatic diseases. METHODS: A search of Medline, EMBASE, and Cochrane Library databases was performed to June 2018 to identify damage indices in all systemic rheumatic diseases. Articles were included in a systematic review if indices were composite (multi-organ) in nature and if adequate detail on methodology was described. Articles pertaining to the validation of these indices were also reviewed in order to assess the psychometric properties of the indices using the Outcome Measures in Rheumatology Arthritis Clinical Trials (OMERACT) filter as a guide. RESULTS: Of the 2659 articles retrieved through the search, we identified 7 damage indices in five diseases: idiopathic inflammatory myopathy, systemic lupus erythematosus, systemic vasculitis, SjÓ§gren's syndrome and antiphospholipid syndrome. A further 48 articles were identified pertaining to the validation of these damage indices. The methodological process for the development of these indices included expert consensus, item reduction and item weighting methods. The level of validation that these indices have achieved is variable, with only 2 damage indices fulfilling all criteria of the OMERACT filter. CONCLUSIONS: To date, there have been 7 composite disease damage indices created in a variety of rheumatic diseases, with the exception of systemic sclerosis (SSc). This review has informed methodology for the development of a disease damage index in SSc.


Subject(s)
Rheumatic Diseases/pathology , Humans , Outcome Assessment, Health Care , Psychometrics , Rheumatic Diseases/diagnosis , Rheumatology , Severity of Illness Index
12.
Semin Arthritis Rheum ; 48(4): 728-735, 2019 02.
Article in English | MEDLINE | ID: mdl-29706242

ABSTRACT

OBJECTIVE: This study aimed to review and compare the analytical and clinical performance of automated indirect immunofluorescence (AIIF) and manual indirect immunofluorescence (MIIF) as anti-nuclear antibody screening assays for patients with systemic rheumatic diseases (SRDs), such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). METHODS: A systematic literature search was performed in the Medline, Embase, Cochrane, Web of Science, and Scopus databases for studies published before August 2017. A bivariate random effects model was used to calculate the summary diagnostic values. RESULTS: Twenty-two studies involving 6913 positive and 1818 negative samples of MIIF, as well as 524 combined SRD, 132 SLE, and 104 SSc patients, and 520 controls were available for meta-analysis. The summary positive concordance (PC) of qualitative result between AIIF and MIIF was 93.7%, whereas PCs of total pattern (68.5%; homogeneous, 52.3%; speckled, 56.5%; nucleolar, 52.7%; centromere, 51.4%; nuclear dot, 11.7%) and titer (77.8%) exhibited significantly lower values. The summary clinical sensitivities of AIIF vs. MIIF were 84.7% vs 78.2% for combined SRDs, 95.5% vs. 93.9% for SLE, and 86.5% vs. 83.7% for SSc, respectively. Meanwhile, the summary specificities of AIIF vs. MIIF were 75.6% vs. 79.6% for combined SRDs, 74.2% vs. 83.3% for SLE, and 74.2% vs. 83.3% for SSc, respectively. Although the differences in sensitivity and specificity between AIIF and MIIF were not significant in most subgroups, the summary specificity of SLE and SSc showed statistically significant changes. CONCLUSIONS: Our systematic meta-analysis demonstrates that AIIF is comparable to MIIF in distinguishing between the positive and negative results, and screening SRDs based on clinical sensitivities and standardization. However, improvements in the pattern and titer recognition and clinical specificities are necessary.


Subject(s)
Antibodies, Antinuclear/analysis , Fluorescent Antibody Technique, Indirect/methods , Rheumatic Diseases/diagnosis , Humans , Rheumatic Diseases/immunology , Sensitivity and Specificity
13.
Rheumatol Int ; 38(8): 1345-1354, 2018 Aug.
Article in English | MEDLINE | ID: mdl-29869008

ABSTRACT

Inflammasomes are large intracellular complexes that induce inflammation in response to exogenous and endogenous damage signals. They regulate production and release of the proinflammatory cytokines IL-1ß and IL-18, playing a defensive role against infections. Inflammasomes have also been involved in the pathogenesis of a wide range of autoinflammatory conditions that are caused by dysregulation of the IL-1 pathway, such as cryopyrinopathies and hereditary periodic fever syndromes. On top of that, research in recent years suggests that defects in inflammasome regulation and signaling associate with a number of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others. In this review, we describe the inflammasome and mechanisms that trigger it, provide a brief review of autoinflammatory disorders and discuss the current understanding and emerging data from experimental and clinical studies for the role of the innate immune system and inflammasomes in the biology and pathogenesis of systemic autoimmune diseases.


Subject(s)
Autoimmune Diseases/metabolism , Inflammasomes/physiology , Autoimmune Diseases/etiology , Cryopyrin-Associated Periodic Syndromes , Hereditary Autoinflammatory Diseases , Host-Pathogen Interactions/immunology , Humans , Inflammasomes/metabolism , Inflammation , Rheumatic Diseases
14.
Semin Arthritis Rheum ; 48(2): 334-342, 2018 10.
Article in English | MEDLINE | ID: mdl-29609799

ABSTRACT

OBJECTIVE: This study aimed to review and compare the diagnostic accuracy of the screening enzyme immunoassay (SEIA) and indirect immunofluorescence (IIF) as anti-nuclear antibody (ANA) screening assays for patients with systemic rheumatic diseases (SRDs), including systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), and systemic sclerosis (SSc). METHODS: A systematic literature search was conducted in the Medline, Embase, Cochrane, Web of Science, and Scopus databases for articles published before August 2017. A bivariate random effects model was used to calculate pooled diagnostic values. RESULTS: Thirty-three studies including 3976 combined SRDs, 2839 SLE, 610 SS, and 1002 SSc patients and 11,716 non-healthy and 8408 healthy controls were available for the meta-analysis. The summary sensitivities of SEIA vs. IIF were 87.4% vs 88.4% for combined SRDs, 89.4% vs. 95.2% for SLE, 88.7% vs. 88.4% for SS, and 85.4% vs. 93.6% for SSc, respectively. Meanwhile, the summary specificities of SEIA vs. IIF were 79.7% vs.78.9% for combined SRDs, 89.1% vs. 83.3% for SLE, 89.9% vs. 86.8% for SS, and 92.8% vs. 84.2% for SSc, respectively. Although the differences in sensitivity and specificity between SEIA and IIF were not significant in most subgroups, the summary sensitivity of SLE presented statistically significant changes. CONCLUSIONS: Our systematic meta-analysis demonstrates that both SEIA and IIF are useful to detect ANAs for SRDs. Between the two assays, IIF is a more sensitive screening assay than SEIA, particularly in patients with SLE. SEIA is comparable to IIF, considering the specificity and standardization.


Subject(s)
Antibodies, Antinuclear/analysis , Autoimmune Diseases/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Rheumatic Diseases/diagnosis , Scleroderma, Systemic/diagnosis , Sjogren's Syndrome/diagnosis , Autoimmune Diseases/immunology , Fluorescent Antibody Technique, Indirect , Humans , Immunoenzyme Techniques , Lupus Erythematosus, Systemic/immunology , Rheumatic Diseases/immunology , Scleroderma, Systemic/immunology , Sensitivity and Specificity , Sjogren's Syndrome/immunology
15.
Autoimmun Rev ; 17(3): 201-214, 2018 Mar.
Article in English | MEDLINE | ID: mdl-29353099

ABSTRACT

Systemic autoimmune diseases can affect various kinds of organs including the kidney, the skin, soft tissue and the bone. Among others, cardiovascular involvement in rheumatic diseases has been shown to affect myocardium, pericardium, cardiac vessels, conduction system and valves, eventually leading to increased mortality. In general, underlying chronic inflammation leads to premature atherosclerosis, but also other manifestations such as arrhythmia and heart failure may have a 'silent' progress. Traditional cardiovascular risk factors play a secondary role, while disease-specific factors (i.e. disease duration, severity, antibody positivity, persistent disease activity) can directly influence the cardiovascular system. Therefore, early diagnosis is critical to optimize management and to control inflammatory activity and recent data suggest that risk factors (i.e. hypercholesterolemia and hypertension) need intensive treatment as well. With the advent of immunosuppressive agents, most rheumatic diseases are well controlled on treatment, but information related to their cardioprotective efficacy is not well-defined. In this review, we focus on cardiovascular involvement in rheumatic diseases and highlight current evidence which should be of help for the treating physicians. Moreover, cardiotoxicity of immunosuppressive drugs is a rare issue and such potential adverse events will be briefly discussed.


Subject(s)
Cardiovascular Diseases/etiology , Physicians/standards , Rheumatic Diseases/complications , Cardiovascular Diseases/pathology , Humans , Rheumatic Diseases/pathology , Risk Factors
16.
Bone Rep ; 6: 3-8, 2017 Jun.
Article in English | MEDLINE | ID: mdl-28377975

ABSTRACT

Purpose To evaluate the incidence of fragility fractures associated with high-dose glucocorticoid therapy in patients with systemic rheumatic disease. METHODS: A retrospective study of patients who were treated with high-dose prednisolone (> 0.8 mg/kg) for systemic rheumatic disease at Kobe University Hospital from April 1988 to March 2012. The primary outcome was a major osteoporotic fracture (defined as a clinical vertebral, hip, forearm, or proximal humerus fracture) after high-dose glucocorticoid therapy. For postmenopausal women and men over 40 of age, the patient's fracture risk at the beginning of high-dose glucocorticoid therapy was assessed by the World Health Organization's Fracture Risk Assessment Tool (FRAX®). Results Of 229 patients (median age: 49 years), 57 suffered a fragility fracture during the observation period (median observation period: 1558 days). Of 84 premenopausal patients, 5 suffered a fracture. In contrast, of 86 postmenopausal female, 36 suffered a fracture. Fragility fractures were far more frequent than predicted by the FRAX® score. Patients with FRAX® scores over 8.3% had a particularly high risk of fracture. Conclusions Fragility fractures associated with high-dose glucocorticoid therapy are common among postmenopausal women. Extreme care should be taken especially for postmenopausal women when high-dose glucocorticoid therapy is required.

17.
Curr Rheumatol Rev ; 13(3): 162-169, 2017.
Article in English | MEDLINE | ID: mdl-28317489

ABSTRACT

BACKGROUND: Ankylosing Spondylitis (AS) is the prototype of a group of systemic rheumatic diseases collectively referred to as Spondylarthitides (SpA). It has now become clear that AS is not as rare as previously thought and, although it has an early onset in life affecting patients in their reproductive years, it has not been proved to adversely affect fertility in females. OBJECTIVE: The aim of this review is to summarize all the recent data on AS and pregnancy in terms of fertility, disease course and pregnancy outcome from a clinical perspective. METHOD: A literature research was conducted based on the following medical databases: Pubmed/ Medline and the Cochrane Library. We searched for randomized controlled studies, casecontrol studies, cohort studies, patient and drug registers in relation to pregnancy and AS. RESULTS: The existing data do not support a causal relationship between AS and infertility. The state of pregnancy is not associated with reduced disease activity in patients with AS. Additionally, AS tends to adversely affect health-related quality of life during pregnancy, in comparison with normal population and patients with rheumatoid arthritis. As far as the obstetrical outcome is concerned, there is no consensus on the significant association between AS and specific pregnancy, delivery and fetal complications. CONCLUSION: Previous studies are highly heterogenous and mainly retrospective and thus, the existing data are controversial and inconclusive. Subsequent studies are required to enlighten our knowledge on the interaction between AS and pregnancy.


Subject(s)
Pregnancy Complications , Spondylitis, Ankylosing , Female , Humans , Pregnancy , Pregnancy Outcome
18.
Laboratory Medicine Online ; : 153-157, 2011.
Article in Korean | WPRIM (Western Pacific) | ID: wpr-89630

ABSTRACT

BACKGROUND: Anti-streptolysin O (ASO) test is usually used to diagnose group A streptococcal infection-related diseases, such as rheumatic fever, reactive arthritis, and various infectious diseases. Despite the recent declining incidence of these diseases, ASO test is still frequently performed as a screening test to diagnose rheumatic diseases. This study re-evaluated the clinical usefulness of ASO test in systemic rheumatic diseases (SRD). METHODS: ASO tests was performed in 825 patients between April and October in 2010. ASO levels were compared between SRD and non-SRD groups of patients. The results of ASO, C-reactive protein (CRP), and rheumatoid factor (RF) were compared among 6 subgroups of SRD: rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, Behcet disease, Sjogren's syndrome and others. RESULTS: Positive results in ASO test (>200 IU/mL) were observed in 15.3% (126/825) of the patients tested. None of the ASO positive patients was, however, diagnosed with rheumatic fever or reactive arthritis. There were no statistically significant differences in the mean value (P=0.688) or positive rate (P=0.835) of ASO test between SRD and non-SRD groups. Positive rates of ASO test were also not statistically significant different among six subgroups of SRD patients (all P>0.05), whereas those of CRP and RF tests were significantly different. CONCLUSIONS: The usefulness of ASO test is very low for diagnosing SRD, although it is frequently carried out as a screening test. We suggest that ASO test must be performed selectively when diseases from group A streptococcal infection are suspected.


Subject(s)
Humans , Arthritis, Reactive , Arthritis, Rheumatoid , Behcet Syndrome , C-Reactive Protein , Communicable Diseases , Incidence , Lupus Erythematosus, Systemic , Mass Screening , Rheumatic Diseases , Rheumatic Fever , Rheumatoid Factor , Sjogren's Syndrome , Spondylitis, Ankylosing , Streptococcal Infections
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