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  • 1
    Online Resource
    Online Resource
    Validation of the RIM Score-COVID in the Spanish SEMI-COVID-19 Registry
    Springer Science and Business Media LLC ; 2023
    In:  Internal and Emergency Medicine Vol. 18, No. 3 ( 2023-04), p. 907-915
    Details
    In: Internal and Emergency Medicine, Springer Science and Business Media LLC, Vol. 18, No. 3 ( 2023-04), p. 907-915
    Type of Medium: Online Resource
    ISSN: 1828-0447 , 1970-9366
    URL: Article
    DOI: 10.1007/s11739-023-03200-3
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2378342-4
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  • 2
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    Mortality reduction in older COVID-19-patients hospitalized in Spain during the second pandemic wave from the SEMI-COVID-19 Registry
    Springer Science and Business Media LLC ; 2023
    In:  Scientific Reports Vol. 13, No. 1 ( 2023-10-18)
    Details
    In: Scientific Reports, Springer Science and Business Media LLC, Vol. 13, No. 1 ( 2023-10-18)
    Abstract: In 2020, the COVID-19 pandemic followed a two-wave pattern in most countries. Hospital admission for COVID-19 in one wave or another could have affected mortality, especially among the older persons. The objective of this study was to evaluate whether the admission of older patients during the different waves, before SARS-CoV-2 vaccination was available, was associated with a different mortality. We compared the mortality rates of patients hospitalized during 2020 before (first wave) and after (second wave) July 7, 2020, included in the SEMI-COVID-19 Registry, a large, multicenter, retrospective cohort of patients admitted to 126 Spanish hospitals for COVID-19. A multivariate logistic regression analysis was performed to control for changes in either the patient or disease profile. As of December 26, 2022, 22,494 patients had been included (17,784 from the first wave and 4710 from the second one). Overall mortality was 20.4% in the first wave and 17.2% in the second wave (risk difference (RD) − 3.2%; 95% confidence interval (95% CI) − 4.4 to − 2.0). Only patients aged 70 and older (10,973 patients: 8571 in the first wave and 2386 in the second wave) had a significant reduction in mortality (RD − 7.6%; 95% CI − 9.7 to − 5.5) (unadjusted relative risk reduction: 21.6%). After adjusting for age, comorbidities, variables related to the severity of the disease, and treatment received, admission during the second wave remained a protective factor. In Spain, patients aged 70 years and older admitted during the second wave of the COVID-19 pandemic had a significantly lower risk of mortality, except in severely dependent persons in need of corticosteroid treatment. This effect is independent of patient characteristics, disease severity, or treatment received. This suggests a protective effect of a better standard of care, greater clinical expertise, or a lesser degree of healthcare system overload.
    Type of Medium: Online Resource
    ISSN: 2045-2322
    URL: Article
    DOI: 10.1038/s41598-023-42735-5
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2615211-3
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  • 3
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    3233 Von Willebrand Factor is Localized in the Extravascular tissue of Patients with Juvenile Scleroderma
    Cambridge University Press (CUP) ; 2019
    In:  Journal of Clinical and Translational Science Vol. 3, No. s1 ( 2019-03), p. 158-159
    Details
    In: Journal of Clinical and Translational Science, Cambridge University Press (CUP), Vol. 3, No. s1 ( 2019-03), p. 158-159
    Abstract: OBJECTIVES/SPECIFIC AIMS: To further explore the role of vWF in the pathogenesis of scleroderma by identifying its location within the tissue of sample biopsies obtained as part of routine diagnosis with the use of immuno-histochemical staining. METHODS/STUDY POPULATION: We examined 8 skin biopsies from 2 patients with systemic sclerosis (SSc), 2 with localized scleroderma (LS) and 4 with JDM. Double immunofluorescence staining was performed in each tissue with antibodies against vWF and collagens type I and III. DAPI (4′, 6-diamidino-2-phenylindole) was also used for counterstaining of inflammatory cells. Tissue staining patterns were compared between groups. RESULTS/ANTICIPATED RESULTS: Biopsies were obtained from the upper extremity of 7 females and the lower extremity of 1 male. Median age, symptom duration, and serum levels of vWF antigen around the time of biopsy was 8 years (IQR 4.5-11), 5.5 months (IQR 2.5-7), and 245% (IQR 203-302 for 7 patients), respectively. All but 1 biopsy was performed prior to initiation of immunosuppressive therapy. Immunofluorescence staining showed a superficial and deep perivascular inflammatory cell infiltrate that co-localized with vWF in all tissues. There was expression of vWF in the extravascular tissue of patients with JScl co-localizing with collagen III in the reticular dermis (Figures 1 and 2). In comparison, vWF expression was restricted to the endothelium and did not co-localize with collagen in the dermis of patients with JDM (Figure 3). Patients with SSc had higher expression of vWF as compared to patients with LS. DISCUSSION/SIGNIFICANCE OF IMPACT: vWF may participate in the pathogenesis of cutaneous inflammatory conditions. We have demonstrated that vWF co-localizes with cellular inflammatory infiltrates in the perivascular areas and in the dermis of patients with JScl and JDM. We additionally speculate that vWF may participate in the pathogenesis of fibrosing skin diseases based on evidence of increased extravascular expression in the tissue of patients with JScl (vs. JDM), and its co-localization with collagen. vWF expression intensity in the dermis of JScl patients may relate to disease extension (SSc vs. LS).
    Type of Medium: Online Resource
    ISSN: 2059-8661
    URL: Article
    DOI: 10.1017/cts.2019.363
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2019
    detail.hit.zdb_id: 2898186-8
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  • 4
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    Impact of SARS-CoV-2 on the clinical presentation of juvenile idiopathic inflammatory myopathies
    Springer Science and Business Media LLC ; 2023
    In:  Pediatric Rheumatology Vol. 21, No. 1 ( 2023-08-11)
    Details
    In: Pediatric Rheumatology, Springer Science and Business Media LLC, Vol. 21, No. 1 ( 2023-08-11)
    Abstract: Growing evidence suggests that infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may trigger idiopathic inflammatory myopathies (IIM). Few studies have described individual juvenile IIM (JIIM) cases following SARS-CoV-2 infection, and none explored its potential effects on JIIM clinical presentation. We aim to investigate the impact of SARS-CoV-2 on JIIM in patients diagnosed before and after the onset of the Coronavirus Disease 2019 (COVID-19) pandemic. Methods Patients diagnosed with JIIM before age 19 at The Children’s Hospital at Montefiore were included. Demographics, clinical and laboratory data, and evidence of SARS-CoV-2 exposure were collected retrospectively. Patients were grouped by pre-COVID-19 (before January 1, 2020) and post-COVID-19 (January 1, 2020, or later). Descriptive statistics were used to summarize each variable. Non-parametric testing was performed using Fischer’s exact test and Mann-Whitney U test. Results Fifty-one patients were included, 13 (25%) diagnosed in the post-COVID-19 era. Of these, 10 (77%) had onset of JIIM symptoms after January 1, 2020; 6 (60%) with known or suspected SARS-CoV-2 exposure. Though not statistically significant, post-pandemic patients tended to be older, female, and have non-specific cutaneous manifestations. Despite reported delays in care for other pediatric diagnoses during the pandemic, fewer post-pandemic patients had delays in JIIM diagnosis. Conclusions This is the first study to explore the effects of SARS-CoV-2 on JIIM clinical presentation. While our exploratory single-center study did not find significant differences in JIIM diagnosed pre- and post-pandemic, larger prospective multicenter studies are warranted to evaluate this association and to explore clinical variances over time.
    Type of Medium: Online Resource
    ISSN: 1546-0096
    URL: Article
    DOI: 10.1186/s12969-023-00861-4
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2279468-2
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  • 5
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    Juvenile Localized Scleroderma
    Elsevier BV ; 2021
    In:  Rheumatic Disease Clinics of North America Vol. 47, No. 4 ( 2021-11), p. 737-755
    Details
    In: Rheumatic Disease Clinics of North America, Elsevier BV, Vol. 47, No. 4 ( 2021-11), p. 737-755
    Type of Medium: Online Resource
    ISSN: 0889-857X
    URL: Article
    DOI: 10.1016/j.rdc.2021.07.014
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2021
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  • 6
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    Rapid Implementation of a Multidisciplinary COVID‐19 Cytokine Storm Syndrome Task Force
    Wiley ; 2021
    In:  ACR Open Rheumatology Vol. 3, No. 3 ( 2021-03), p. 133-137
    Details
    In: ACR Open Rheumatology, Wiley, Vol. 3, No. 3 ( 2021-03), p. 133-137
    Abstract: Patients with coronavirus disease 2019 (COVID‐19) can progress to a state of unregulated inflammation called cytokine storm syndrome (CSS). We describe formation and operation of a COVID‐19 multidisciplinary consultation service that was allowed to individualize treatment for critically ill patients with COVID‐19 during the pandemic. Methods Institutional experts from different subspecialties formed a COVID‐19 CSS task force at Montefiore Medical Center, Bronx, NY. They agreed on a set of four clinical and six laboratory parameters that can help early identify COVID‐19 CSS. We describe the formation and implementation of the COVID‐19 task force. The case series description of the COVID‐19 CSS consultation cohort highlights consultation volume, baseline characteristics, clinical and laboratory parameters, and how biologic treatments were allocated to these patients. Results Between April 4,2020, and May 7,2020, the COVID‐19 CSS task force was formed, consisting of adult and pediatric rheumatologists and allergy and immunology physicians. The task force evaluated a total of 288 patients, of whom 197 (68%) were male, the median (interquartile range [IQR]) age was 62 (51‐70) years, 122 (42%) were Hispanic, and 88 (31%) were Black or African American. The common presenting symptoms in all referred patients were dyspnea (85%) and diarrhea (80%). Thirty‐one patients who received biologic therapy were younger, with a median (IQR) age of 53 (32‐63) years, as opposed to 62.5 (52‐70) years in the nonbiologic group ( P = 0.008). A higher proportion receiving biologics was in the critical care setting (26 [84%] vs 151 [59%] ; P = 0.006). Conclusion To the best of our knowledge, this is the first multidisciplinary collaborative effort to provide individualized patient recommendations for evaluation and treatment of patients with COVID‐19 who may have CSS. This working model helped to devise an approach that may have identified patients who were most likely to benefit from biologic therapy in the absence of evidence‐based guidelines.
    Type of Medium: Online Resource
    ISSN: 2578-5745 , 2578-5745
    URL: Issue
    URL: Article
    DOI: 10.1002/acr2.v3.3
    DOI: 10.1002/acr2.11220
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2971160-5
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  • 7
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    Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort
    SAGE Publications ; 2023
    In:  Journal of Scleroderma and Related Disorders Vol. 8, No. 2 ( 2023-06), p. 120-130
    Details
    In: Journal of Scleroderma and Related Disorders, SAGE Publications, Vol. 8, No. 2 ( 2023-06), p. 120-130
    Abstract: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. Conclusion: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.
    Type of Medium: Online Resource
    ISSN: 2397-1983 , 2397-1991
    URL: Article
    DOI: 10.1177/23971983221143244
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2023
    detail.hit.zdb_id: 2964332-6
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  • 8
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    Diagnostic and prognostic tests in systemic lupus erythematosus
    Elsevier BV ; 2017
    In:  Best Practice & Research Clinical Rheumatology Vol. 31, No. 3 ( 2017-06), p. 351-363
    Details
    In: Best Practice & Research Clinical Rheumatology, Elsevier BV, Vol. 31, No. 3 ( 2017-06), p. 351-363
    Type of Medium: Online Resource
    ISSN: 1521-6942
    URL: Article
    DOI: 10.1016/j.berh.2017.10.002
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2017
    detail.hit.zdb_id: 2028861-X
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  • 9
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    Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort
    Wiley ; 2022
    In:  Arthritis Care & Research Vol. 74, No. 10 ( 2022-10), p. 1575-1584
    Details
    In: Arthritis Care & Research, Wiley, Vol. 74, No. 10 ( 2022-10), p. 1575-1584
    Abstract: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. Methods A cross‐sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient‐ and physician‐reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi‐square and Mann‐Whitney U tests. Results At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6‐minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician‐rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. Conclusion Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    URL: Article
    DOI: 10.1002/acr.v74.10
    DOI: 10.1002/acr.24609
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2016713-1
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  • 10
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    Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis
    Wiley ; 2022
    In:  Arthritis Care & Research Vol. 74, No. 3 ( 2022-03), p. 364-370
    Details
    In: Arthritis Care & Research, Wiley, Vol. 74, No. 3 ( 2022-03), p. 364-370
    Abstract: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DL co ) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high‐resolution computed tomography (HRCT) in juvenile SSc. Methods The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DL co in detecting ILD. Results Eighty‐six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison. Using findings on HRCT as the standard measure of ILD presence, the sensitivity of FVC in detecting ILD in juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty‐eight juvenile SSc patients had both CT imaging and DL co values for comparison. The sensitivity of DL co in detecting ILD was 76%, the specificity was 70%, and AUC was 0.73. Conclusion The performance of PFTs in juvenile SSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss ~60% of children who had ILD changes on their accompanying HRCT. The DL co was more sensitive in detecting potential abnormalities on HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in juvenile SSc.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    URL: Article
    DOI: 10.1002/acr.v74.3
    DOI: 10.1002/acr.24499
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2016713-1
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