Systemic Rheumatic Diseases Patients Research Articles

Outcomes of COVID-19 re-infections: a single-center cohort of 167 patients with systemic rheumatic diseases.

Data on COVID-19 re-infections in patients with systemic rheumatic diseases (SRDs) are lacking. We aimed to describe the course and outcomes of COVID-19 re-infections in these patients versus controls. In this single-center retrospective study, we included 167 consecutive SRD patients with at least one COVID-19 re-infection (mean age 47.3 years, females 70.7%). SRD patients were compared in terms of patient-perceived COVID-19 re-infection severity and hospitalizations/deaths with 167 age/sex-matched non-SRD controls. Logistic regression analysis was performed to assess potential milder re-infection versus primary infection severity, adjusting for study group, demographics (age, sex), vaccination status, body mass index, smoking, and comorbidities. 23 and 7 out of 167 re-infected SRD patients experienced two and three re-infections, respectively, which were comparable to the re-infection rates in controls (two: 32; and three: 2) who also had comparable COVID-19 vaccination history (89% and 95% vaccinated, respectively). In the initial infection, patients with SRDs were hospitalized (7.2% versus 1.8%, p = 0.017), and had received antiviral treatment (16.1% versus 4.7%, p < 0.001) more frequently than controls. However, hospitalizations (1.8% vs 0.6%) and antiviral treatment (7.8% vs 3.5%) did not differ (p > 0.05) between patients and controls at the first re-infection, as well as during the second and third re-infection; no deaths were recorded. Perceived severity of re-infections was also comparable between patients and controls (p = 0.847) and among those on biologic DMARDs or not (p = 0.482). In multivariable analysis, neither SRDs presence nor demographics or comorbidities were associated with COVID-19 re-infection severity. COVID-19 re-infection severity (patient-perceived/hospitalizations/deaths) did not differ between SRDs and controls.

In-ICU-acquired infections in flare-up systemic rheumatic disease patients receiving immunosuppressant.

Systemic rheumatic diseases (SRDs) are a group of inflammatory disorders that can need intensive care unit (ICU) admission during a flare-up, requiring administration of immunosuppressants. We undertook this study to determine the frequency, outcome, and occurrence associated factors of infections in flare-up SRD patients receiving immunosuppressant. Monocenter, a retrospective study including SRD patients admitted to ICU for a flare-up requiring immunosuppressant from 2004 to 2019. The primary endpoint was in-ICU-acquired infections. Ninety-eight patients (female/male ratio: 1.6; mean age at admission: 39.5 ± 17.4 years) were admitted to the ICU for a SRD flare-up, inaugural in 61.2% cases. A specific treatment was given to every patient: corticosteroids 100%, cyclophosphamide 45.9%, plasma exchange 46.9%. Ninety-five infections occurred in 35 (36%) patients mainly pneumonias. The overall in-hospital mortality was 17.3%, and 46% of patients with a nosocomial infection died during their ICU stay. The logistic regression multivariable model retained renal replacement therapy and mechanical ventilation as independent predictors of infection. In-ICU-acquired infection in SRD flare-up is a frequent event associated with organ failures but not with in-ICU use of immunosuppressants. These data suggest that the fear of infection should not withhold a careful in-ICU use of immunosuppressive drugs. Key Points • In-ICU infections are frequent in flare-up systemic rheumatic disease patients. • Infections are associated with increased mortality. • Cyclophosphamide given in ICU was not independently associated with infection. • Severe neutropenia occurred in 27% of patients receiving cyclophosphamide in ICU.

Different systemic rheumatic diseases as risk factors for COVID-19-related mortality.

COVID-19 has been associated with increased morbidity and mortality, globally. Whether COVID-19-related mortality is increased in patients with systemic rheumatic diseases (SRDs) is still debatable. Although results are somewhat conflicting, there are a handful of nationwide studies published indicating that, in individuals with SRD, there is signal for increased adverse COVID-19-related outcomes and higher mortality. It appears that there are differences in COVID-19-related mortality across various SRDs. Besides, certain disease-specific (disease activity, disease duration, medication received) and/or other features (e.g. comorbidities) seem to also affect COVID-19-related mortality in SRD patients. Herein, we wanted to highlight that a more individualized approach taking into consideration the effect of the aforementioned factors into the risk calculation for COVID-19 adverse outcomes, including mortality, in SRD patients is warranted. A multinational study based on nationwide data, examining all common SRDs and stratifying accordingly, would be of interest, toward this direction.Key Points• It is still debatable whether Covid-19-related mortality is increased in patients with sytemic rheumatic diseases (SRD).• Disease-specific risk factors (e.g. type of SRD, disease activity) should be taken into account in risk assessment for Covid-19-releted outcomes in SRD patients.

Short- and long-term prognosis of acute critically ill patients with systemic rheumatic diseases: A retrospective multicentre study.

Patients with systemic rheumatic disease (SRD) share the risks of multi-organ flare-up, cardiovascular diseases, and immunosuppression. Such situations can lead to an acute critical illness. The present study describes the clinical features of SRD patients admitted to the intensive care unit (ICU) and their short- and long- term mortality.We performed a multicentre retrospective study in 10 French ICU in Lyon, France. Inclusion criteria were SRD diagnosis and admission for an acute organ failure. The primary endpoint was ICU mortality.A total of 271 patients were included. SRD included systemic lupus erythematosus (23.2% of included patients), vasculitis (10.7%), systemic sclerosis (10.7%), idiopathic inflammatory myopathy (6.3%), and other connective tissue disorders (rheumatoid arthritis, Sjögren and Sharp syndromes; 50.9%). Initial organ failure(s) were shock (43.5% of included patients), acute kidney injury (30.5%), and acute respiratory failure (23.2%). The cause(s) of ICU admission included sepsis (61.6%), cardiovascular events (33.9%), SRD-flare up (32.8%), and decompensations related to comorbidities (28%). The ICU mortality reached 14.3%. The factors associated with ICU mortality were chronic cardiac failure, invasive ventilation and admission in ICU for another reason than sepsis or SRD flare-up. The median follow-up after ICU discharge was 33.6 months. During follow-up, 109 patients died. The factors associated with long-term mortality included age, Charlson comorbidity index, and ICU admission for sepsis or SRD flare-up.The ICU mortality of patients with SRD was low. Sepsis was the first cause of admission. Cardiovascular events and comorbidities negatively impacted ICU mortality. Admission for sepsis or SRD flare-up exerted a negative effect on the long-term outcome.

Evaluation of pulmonary involvement in systemic rheumatic diseases with high resolution computed tomography and pulmonary function test: A single-center experience

The objective of this study was to investigate the clinical and radiological features and pulmonary function tests (PFTs) in patients with the pulmonary involvement of systemic rheumatic diseases (SRDs). This study was conducted as a retrospective and single-center study. Patients diagnosed with an SRD and admitted/referred to the department of chest diseases of our hospital between January 2015 and June 2019 were enrolled. All patients were evaluated using High Resolution Computed Tomography (HRCT) and PFT. This study included 68 patients (15 males, 53 females) with a mean age of 62.38 ± 12.4 years. Forty-one (60.2%) patients had diagnosis of rheumatoid arthritis (RA), 10 (14.7%) patients had sjögren's syndrome (SS), 8 (11.7%) patients had systemic lupus erythematosus (SLE), 6 (8.8%) patients had systemic sclerosis (SSc), and 3 (4.4%) patients had mixed connective tissue disease (MCTD). While RA, SLE, MCTD patients were more commonly symptomatic, most of the SS patients were asymptomatic. Overall, 30 (44.1%) patients had normal PFT. Although 30 (%44.1) patients were asymptomatic and 30 (%44.1) patients had normal PFTs, at least one imaging finding was found in all patients according to HRCT imaging. "Bronchiectasis" was the most common HRCT finding in RA, followed by "chronic fibrotic changes" and "peribronchial thickening". "Chronic fibrotic changes" and "peribronchial thickening" were the most common changes in SS. Similarly, "peribronchial thickening" was the most common radiologic finding in SLE. As for SSc, "chronic fibrotic changes", "interlobular septal thickening", and "pleural effusion" were the most common radiologic findings. Pulmonary involvement in systemic rheumatic diseases can occur with various radiological images even in asymptomatic patients. PFTs can be normal as well as an obstructive, restrictive or mixed pattern can be seen. Heterogeneous and combined HRCT findings can be seen in SRD patients.

Outcomes of Severe Systemic Rheumatic Disease Patients Requiring Extracorporeal Membrane Oxygenation

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), whose may need a VA- or VV-ECMO support. Data focusing on ECMO in SRD patients are scarce and many questions persist. We undertook this study to determine the outcomes and unfavorable outcome-associated factors of severely ill SRD patients requiring ECMO support. 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. The secondary outcomes included patient's characteristics (laboratory findings, in-ICU organ-failure treatment(s), SRD-specific manifestations and treatment(s), complications). Were compared the primary and secondary outcomes for the entire population and in the following subgroups : flare-/infection-related admission and VA/VV-ECMO. 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). The flow chart reports patients’ outcomes according to the reason for admission and ECMO hook-up. 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 ; day-0 SAPS II score ≥ 70, ventilator-associated pneumonia and arterial lactate ≥ 7.5 mmol/L for VV-ECMO–treated patients whereas vasculitis independently predicts hospital survey. The main analysis considered VA- and VV-ECMO patients jointly. The reasons for ICU admissions and ECMO canulation, and the characteristics, management and outcomes of these patients obviously differ. However, the analysis aimed to present a comprehensive, real-life picture of ECMO treatment of SRD patients, with separate analyses of VA- and VV-ECMO subgroups thereafter. ECMO support appears to be relevant for critically ill SRD patients, with 49% survival at hospital discharge. Herein, we report the largest series of ECMO-treated, severely ill SRD patients. 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.

Interstitial Lung Disease Worsens Short- and Long-Term Outcomes of Systemic Rheumatic Disease Patients Admitted to the ICU: A Multicenter Study.

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.

Outcomes of severe systemic rheumatic disease patients requiring extracorporeal membrane oxygenation

BackgroundSystemic 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.MethodsThis 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.ResultsNinety 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.ConclusionsECMO 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.

Levels of anti-nuclear antibodies by Fana and Ana-Lia in systemic rheumatic diseases patients

Objetivo: Correlacionar los niveles de anticuerpos antinucleares obtenidos por las técnicas de inmunofluorescencia indirecta (FANA) e inmunoensayo lineal (ANA-LIA) en pacientes diagnosticados o con sospecha clínica de enfermedad autoinmune. Métodos: Se incluyeron 100 pacientes que dieron su consentimiento informado para participar en el estudio. Los ensayos e interpretación de los resultados de las pruebas de FANA y ANA-LIA se realizaron siguiendo las recomendaciones del fabricante. Resultados: el 97,4% de los pacientes estudiados eran de sexo femenino con edad promedio de 42 años, siendo la Artritis reumatoide, Poliartritis y Lupus Eritematoso Sistémico las enfermedades que con mayor frecuencia acompañaron la solicitud médica. Se observó que ambos métodos fueron positivos en el 21% de los casos y que el indice de correlaciòn de Kappa entre las pruebas fue moderado (k= 0,51; p&lt; 0,05), la sensibilidad y especificidad de los métodos fue del 71,4% y 84,8% respectivamente, se observó también que para un determinado patrón fluorescente puede haber positividad de más de un antígeno de la prueba de ANA-LIA y viceversa. Conclusiones: FANA es el método de tamizaje aceptado en la práctica clínica para orientar hacia un diagnóstico clínico de enfermedad autoinmune más probable, debido a la subjetividad en la interpretación de sus resultados y necesidad de entrenamiento en la identificación de los patrones fluorescentes, se requiere del apoyo de otros métodos de laboratorio que permitan identificar con mayor precisión los antígenos reconocidos por los autoanticuerpos, ANA-LIA es una herramienta de laboratorio costo-efectiva de elevada sensibilidad y especificidad que se ajusta a este requerimiento.

Recommendations for the management of patients with systemic rheumatic diseases during the coronavirus disease pandemic.

Patients with systemic rheumatic diseases (SRD) are vulnerable for coronavirus disease (COVID-19). The Korean College of Rheumatology recognized the urgent need to develop recommendations for rheumatologists and other physicians to manage patients with SRD during the COVID-19 pandemic. The working group was organized and was responsible for selecting key health questions, searching and reviewing the available literature, and formulating statements. The appropriateness of the statements was evaluated by voting panels using the modified Delphi method. Four general principles and thirteen individual recommendations were finalized through expert consensus based on the available evidence. The recommendations included preventive measures against COVID-19, medicinal treatment for stable or active SRD patients without COVID-19, medicinal treatment for SRD patients with COVID-19, and patient evaluation and monitoring. Medicinal treatments were categorized according to the status with respect to both COVID-19 and SRD. These recommendations should serve as a reference for individualized treatment for patients with SRD. As new evidence is emerging, an immediate update will be required.

Safety of the Zoster Vaccine Recombinant Adjuvanted in Rheumatoid Arthritis and Other Systemic Rheumatic Disease Patients: A Single Center's Experience With 400 Patients.

ObjectivePatients with rheumatoid arthritis (RA) and other systemic rheumatic diseases (SRDs) are at increased risk of developing herpes zoster (HZ). Zoster recombinant adjuvanted (ZRA) is a recombinant vaccine approved by the Food and Drug Administration in 2018. Concern has been raised that the ZRA may trigger disease flares in rheumatology patients who are immunocompromised. We investigated the impact of the ZRA vaccine in patients with RA and SRD and measured the incidence of flares and side effects.MethodsA flare was defined as occurring within 12 weeks of vaccine administration by either 1) documentation of RA flare in office notes, telephone encounter, or patient portal communication or 2) new or increased dose of corticosteroids.ResultsWe identified 403 patients (239 patients with RA and 164 patients with SRD) who received the ZRA vaccine from February 1, 2018, to February 1, 2019. We measured a 6.7% (n = 27) incidence of flare. Side effects occurred in 12.7% (n = 51) of patients. All flares and side effects were regarded as mild. Three cases of HZ were reported as occurring 2, 10, and 11 months after the vaccination.ConclusionIn 403 patients who received the ZRA vaccine, the incidence of disease flares was 7% or less and that of side effects was 13% or less, both of which are less than the incidence rates observed in the pivotal trials.

Etiologies and outcomes of rheumatology patients with acute respiratory failure requiring intensive care: a single-center medical records review study of 259 patients.

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. 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. 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 < 100mmHg (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. 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.

Profile of common inflammatory markers in treatment-naïve patients with systemic rheumatic diseases.

To evaluate and compare the clinical implications of common inflammatory markers in systemic rheumatic diseases (SRDs). We investigated the profiles of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and white blood cell (WBC) count in treatment-naïve patients with SRDs, osteoarthritis and pneumonia diagnosed at Seoul National University Hospital during 2004-2016. SRDs included rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), ankylosing spondylitis (AS), systemic sclerosis (SSc), idiopathic inflammatory myopathy (IIM) and adult-onset Still's disease (AOSD). Associations between inflammatory markers were evaluated using Pearson's correlation and regression analysis. ROC curve analysis was performed to examine the predictive value of inflammatory markers for SRD diagnosis. We identified a total of 1191 patients. Leukocytosis was present in < 20% SRD patients. There was marked variability in ESR and CRP levels among different SRDs. The highest mean CRP levels (mean ± SD, mg/dL) were observed in AOSD (11.3 ± 7.9), followed by RA (2.0 ± 3.3), IIM (1.8 ± 3.5), SLE (1.5 ± 3.1), SSc (0.6 ± 1.3) and AS (0.08 ± 0.1). Mean ESR (mm/h) was also highest in AOSD (71.2 ± 31.0), followed by SLE (47.3 ± 34.2), RA (45.5 ± 30.6), IIM (40.8 ± 24.8) and SSc (27.8 ± 26.0). All SRDs showed significant positive correlations between ESR and CRP: greatest in RA (r = 0.53, p < 0.001) and weakest in SLE (r = 0.20, p = 0.03). WBC correlated weakly with CRP but not with ESR in most SRDs. While the AUC for WBC count was less than that of ESR or CRP, the AUC for ESR and CRP were similar in SRD. The optimal cuff-off values for inflammatory markers predicting SRD were within or slightly above the normal limit. ESR, CRP and WBC are not always elevated in treatment-naïve patients with SRD. Individual SRDs have a unique profile of inflammatory markers. However, routine inflammatory markers should still be interpreted with caution when diagnosing and assessing disease activity in those with SRD. Key Points •Leukocytosis and elevation of ESR and CRP are not always present in all systemic rheumatic diseases. •Inflammatory markers are often dissociated and they are not specific for disease diagnosis. •Better biomarkers, which measure disease-specific local and systemic inflammation, are needed.

The Prevalence of Systemic Rheumatic Diseases Among Breast Cancer Patients and Its Relationship With Survival.

This study aims to investigate the prevalence of systemic rheumatic diseases (SRDs) among patients with breast cancer (BC) and to identify the clinicopathological characteristics of these patients. A total of 3,744 female patients with BC (mean age 49±11.7 years; range, 18 to 92 years) followed in Hacettepe University Faculty of Medicine, Medical Oncology Department between January 2006 and December 2015 were retrospectively assessed. Patients with or without SRD were compared in terms of clinicopathological features including age, menopausal state, smoking status, Body Mass Index (BMI), age of menarche, age at first labor, and number of children. The groups were also evaluated regarding tumor grade, stage, estrogen receptor and progesterone receptor expression, human epidermal growth factor receptor 2 overexpression, and survival. Of the patients analyzed, 68 (1.81%) had concomitant SRD. Among these patients, 33 (48.6%) had rheumatoid arthritis, eight (11.8%) had familial Mediterranean fever, eight (11.8%) had Behçet's disease, four (5.8%) had Sjögren's syndrome, four (5.8%) had systemic lupus erythematosus, six (8.8%) had ankylosing spondylitis, three (4.4%) had systemic sclerosis, one (1.4%) had polymyositis, and one (1.4%) had temporal arteritis. The groups with or without SRDs were similar in terms of age, smoking status, BMI, menopausal state, breast feeding duration, age at menarche and first birth. Stage 1 and 2 BC was more prevalent in SRD patients (74.6% vs. 64.5%, p=0.018). The rate to receive chemotherapy was significantly lower in patients with SRD. However, there was no significant difference in five-year overall survival rates between patients with or without SRD. Among patients with BC, 1.81% had concomitant SRD. These patients were diagnosed at early stages and given chemotherapy less frequently. However, they had similar survival rates compared to those without SRDs.

Elevated serum globulin gap as a highly reliable marker of elevated erythrocyte sedimentation rate in patients with systemic rheumatic diseases

ObjectiveSerum 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. MethodsThe 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. ResultsIn 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. ConclusionIn 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.

Determination of Specificity and Pattern of Antinuclear Antibodies (ANA) in Systemic Rheumatic Disease Patients Positive for ANA Testing.

To determine probability of finding antinuclear antibodies (ANA) and anti extractable nuclear antigens (ENA) positive samples and associating ANA patterns with anti-ENA reactivities among a consecutive cohort of samples of systemic rheumatic disease patients referred for ANA testing. Prospective cohort study. Immunology Department, Armed Forces Institute of Pathology, Rawalpindi, Pakistan, from January to June 2016. All the samples referred for ANA testing with clinical suspicion of systemic rheumatic disease were included. After screening, ANA positive samples were subjected to anti-ENA antibodies testing (including anti-SSA, antiSSB, anti-Sm, anti-RNP, anti-SCL-70 and anti-Jo-1 antibodies) and ANA pattern and titer determination. Of 4,347 samples received, 397 were positive for ANA (9%). Of 397, 96 (24%) samples positive on ENA screen were tested for anti-ENA reactivity. Anti-SSA antibodies were found in 59 samples. Commonest ANA patterns were coarse and fine speckled (43 and 22 samples of 81 tested), while majority of samples carried ANA in titers of 1:40 and 1:80 (22 and 18 samples of 81 tested). No specific ANA pattern was associated with any particular anti-ENA reactivity. Among samples/patients referred for investigations of autoimmune disorders, probability of finding positive ANA is approximately 9%. Of these 9%, about 24% also show reactivity against ENA. Commonest ANA pattern is coarse speckled and majority of such patients carry ANA in titers ranging from 1:40 to 1:80. Commonest ENA reactivity was against SSA.

The fact not to ignore: Mean blood pressure is the main predictor of increased arterial stiffness in patients with systemic rheumatic diseases

PurposeWe aimed to evaluate the association between carotid-radial pulse wave velocity (PWV), augmentation index (AIx), and flow-mediated dilatation (FMD) of the brachial artery and factors potentially influencing them in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Material and methods316 patients diagnosed with RA (32%), SLE (20%), SSc (16%) and 156 controls (32%) were included in the study. Parameters of arterial stiffness AIx and PWV were obtained using applanation tonometry. FMD reflecting endothelial function was measured by ultrasound. ResultsAIx was increased in all three diseases (p<0.0001), but no differences were found between rheumatic diseases. In most of the RA cases PWV values were abnormal (on average by 0.52m/sec higher than in controls), while in SSc patients FMD values were diminished (p=0.006). Mean blood pressure (MBP) was the most consistent predictive factor in all three diseases, influencing both PWV and AIx, although patient age was also important in variation of AIx. The disease activity score (DAS28) was relevant only in RA patients. Furthermore, SLE disease activity index in SLE or Rodnan skin thickness score had no statistical significance in SSc and inflammatory markers. ConclusionsBoth, PWV and AIx are dependent on MBP and age DAS28 may affect AIx in RA patients, while other disease or inflammatory markers are unlikely to have any effect. MBP is one of the main cardiovascular risk factors affecting the arterial stiffness in RA, SLE and SSc patients therefore controlling MBP in systemic rheumatic disease patients is mandatory.

SAT0469 Ineffective Fracture Prevention by Bisphosphonate in Patients Undergoing High Dose Glucocorticoid Therapy with A Frax Ten Year Probability Greater than 5.8%

BackgroundHigh dose glucocorticoid (GC) therapy (PSL>0.8mg/kg/day) is often provided to patients with systemic rheumatic disease. GC induced osteoporosis (GIO) is a common complication of high dose GC therapy and bisphosphonate...

Outcomes and organ dysfunctions of critically ill patients with systemic lupus erythematosus and other systemic rheumatic diseases

Our objective was to compare the pattern of organ dysfunctions and outcomes of critically ill patients with systemic lupus erythematosus (SLE) with patients with other systemic rheumatic diseases (SRD). We studied 116 critically ill SRD patients, 59 SLE and 57 other-SRD patients. The SLE group was younger and included more women. Respiratory failure (61%) and shock (39%) were the most common causes of ICU admission for other-SRD and SLE groups, respectively. ICU length-of-stay was similar for the two groups. The 60-day survival adjusted for the groups' baseline imbalances was not different (P = 0.792). Total SOFA scores were equal for the two groups at admission and during ICU stay, although respiratory function was worse in the other-SRD group at admission and renal and hematological functions were worse in the SLE group at admission. The incidence of severe respiratory dysfunction (respiratory SOFA >2) at admission was higher in the other-SRD group, whereas severe hematological dysfunction (hematological SOFA >2) during ICU stay was higher in the SLE group. SLE patients were younger and displayed a decreased incidence of respiratory failure compared to patients with other-SRDs. However, the incidences of renal and hematological failure and the presence of shock at admission were higher in the SLE group. The 60-day survival rates were similar.

Outcome of systemic rheumatic disease patients admitted in intensive care unit

Outcome of systemic rheumatic disease patients admitted in intensive care unit