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1.
Clin Rheumatol ; 2019 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-31667644

RESUMO

The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5 years of first non-Raynaud's symptom. The data collection methodology incorporates successful models from other SSc registries. The cohort is designed to provide linked bio-specimen and clinical outcomes data on a longitudinal cohort of SSc patients for validation of hypothesis-driven research and to provide a platform for studying patient-reported outcomes in scleroderma. The CONQUER registry was developed using the guidelines of the International Society for Biological Repositories, and was an iterative process between physicians with an expertise in SSc, patient stakeholders, and information technology experts. Enrollment commenced in June 2018. During the first 6 months of the CONQUER Scleroderma study, 151 SSc patients with less than 5 years of disease duration (from first non-Raynaud's symptom) have been recruited. The mean age is 51 ± 14 years, 83% are female, and 60% of patients have diffuse disease. Survey completion rates are above 88% for all patient-reported outcome surveys. Bio-specimen collection rates are over 97%, and disease severity score completion rates are over 98%. Pulmonary function test data is available on 91% of patients, and echocardiography is available 80%. The CONQUER scleroderma study provides a unique and growing resource for studying scleroderma in a longitudinal, US-based population. KEY POINTS : • The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5 years of first non-Raynaud's symptom. • The CONQUER scleroderma study provides a unique and growing resource for studying scleroderma in a longitudinal, US-based population. • CONQUER is innovative in its design in that it is focused on prospective collection of paired clinical and patient outcome data with bio-specimens.

2.
Arthritis Res Ther ; 21(1): 202, 2019 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-31481106

RESUMO

BACKGROUND: To determine the effect of riociguat, an oral, selective soluble guanylate cyclase stimulator, on the net digital ulcer (DU) burden in systemic sclerosis (SSc). METHODS: Participants with SSc-related active or painful indeterminate DUs were recruited in a multicenter, double-blind, randomized, placebo-controlled, proof-of-concept trial. Eligible participants were required to have at least one visible, active ischemic DU or painful indeterminate DU at screening, located at or distal to the proximal interphalangeal joint and that developed or worsened within 8 weeks prior to screening. Participants were randomized 1:1 to placebo or riociguat in individualized doses (maximum of 2.5 mg three times daily) during an 8-week titration period, followed by an 8-week stable dosing period. This was followed by an optional 16-week open-label extension phase for participants with active DU/reoccurrence of DUs within 1 month of the end of the main treatment phase. The primary endpoint was the change from baseline to week 16 in net ulcer burden (NUB), analyzed using ANCOVA. Other endpoints included plasma biomarkers and proportion of participants with treatment-emergent adverse events (AEs). RESULTS: Seventeen participants (eight placebo, nine riociguat) were randomized at five centers. Six participants in each group transitioned to the open-label extension. Baseline characteristics were comparable between the treatment groups, except participants randomized to placebo were older and had longer disease duration (p < 0.05). At baseline, the mean (SD) NUB was 2.5 (2.0) in the placebo and 2.4 (1.4) in the riociguat. No significant treatment difference was observed in the change from baseline to 16 weeks in NUB (adjusted mean treatment difference - 0.24, 95% CI (- 1.46, 0.99), p = 0.70). Four participants experienced five serious AE (four in riociguat and one in placebo); none was considered related to study medication. Statistically significant elevation of cGMP was observed at 16 weeks in the riociguat group (p = 0.05); no other biomarkers showed significant changes. In the open-label extension, participants in the riociguat-riociguat arm had complete healing of their DUs. CONCLUSION: In participants with SSc-DU, treatment with riociguat did not reduce the number of DU net burden compared with placebo at 16 weeks. Open-label extension suggests that longer duration is needed to promote DU healing, which needs to be confirmed in a new trial. TRIAL REGISTRATION: ClinicalTrials.gov, NCT02915835 . Registered on September 27, 2016.

3.
Arthritis Rheumatol ; 2019 Jul 24.
Artigo em Inglês | MEDLINE | ID: mdl-31342624

RESUMO

OBJECTIVES: T cells play a key role in the pathogenesis of early systemic sclerosis. This study assessed the safety and efficacy of abatacept in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: A 12-month, randomized, double-blind, placebo-controlled trial with participants randomized in a 1:1 ratio to either abatacept 125 mg subcutaneous or matching placebo, stratified by duration of dcSSc. Escape therapy was allowed at six months for worsening disease. The co-primary end points were change in modified Rodnan skin score (mRSS) and safety over 12 months. Treatment differences in longitudinal outcomes were assessed using linear mixed models, with outcomes censored after initiation of escape therapy. Baseline skin tissue was classified into intrinsic gene expression subsets. RESULTS: Among 88 participants, the adjusted mean change in mRSS at 12 months was -6.24 units in the abatacept and -4.49 units in the placebo, with adjusted mean treatment difference of -1.75 units (p=0.28). Two secondary outcome measures (HAQ-DI and a composite measure) were clinically and statistically significant favoring abatacept. A larger proportion of placebo subjects required escape therapy relative to abatacept (36% vs. 16%). Decline in mRSS over 12 months was clinically and significantly higher in abatacept vs. placebo for the Inflammatory (p<0.001) and Normal-like skin gene expression subsets (p=0.03). 35 participants in the abatacept versus 40 in the placebo had adverse events (AEs), including two and one deaths, respectively. CONCLUSIONS: In this Phase 2 trial, abatacept was well tolerated, but change in mRSS was not statistically significant. Secondary outcome measures, including gene expression subsets, showed some evidence in favor of abatacept. These data should be confirmed in a Phase 3 trial. This article is protected by copyright. All rights reserved.

5.
Arthritis Rheumatol ; 71(10): 1691-1700, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31066998

RESUMO

OBJECTIVE: A prognostic equation and risk score calculator derived from the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) are being used to predict 1-year survival in patients with pulmonary arterial hypertension (PAH), but little is known about the performance of these REVEAL survival prediction tools in systemic sclerosis (SSc)-associated PAH (SSc-PAH). METHODS: Prospectively gathered data from the Johns Hopkins Pulmonary Hypertension Program and Pulmonary Hypertension Assessment and Recognition of Outcome in Scleroderma Registries were used to evaluate the predictive accuracy of the REVEAL models for predicting the probability of 1-year survival in patients with SSc-PAH. Model discrimination was assessed by comparison of the Harrell's C-index, model fit was assessed using multivariable regression techniques, and model calibration was assessed by comparing predicted to observed survival estimates. RESULTS: The validation cohort consisted of 292 SSc-PAH patients with a 1-year survival rate of 87.4%. The C-index for predictive accuracy of the REVEAL prognostic equation (0.734, 95% confidence interval [95% CI] 0.652-0.816) and for the risk score (0.743, 95% CI 0.663-0.823) demonstrated good discrimination, comparable to that in the model development cohort. The calibration slope was 0.707 (95% CI 0.400-1.014), indicating that the overall model fit was marginal (P = 0.06). The magnitude of risk assigned to low distance on the 6-minute walk test (6MWD) and elevated serum levels of brain natriuretic peptide (BNP) was lower in the validation cohort than was originally seen in the REVEAL derivation cohort. Model calibration was poor, particularly for the highest risk groups. CONCLUSION: In predicting 1-year survival in patients newly diagnosed as having SSc-PAH, the REVEAL prognostic equation and risk score provide very good discrimination but poor calibration. REVEAL prediction scores should be interpreted with caution in newly diagnosed SSc-PAH patients, particularly those with higher predicted risk of poor 1-year survival resulting from a low 6MWD or a high BNP serum level.

6.
Arthritis Care Res (Hoboken) ; 71(9): 1154-1163, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30821906

RESUMO

OBJECTIVE: African Americans with scleroderma have more severe disease and higher mortality than non-African Americans. Differences in rates of diffuse disease, autoantibody status, or socioeconomic status have not completely explained this phenomenon. Our study evaluates these risks at our site. METHODS: A retrospective study comparing African American and non-African American patients with scleroderma seen from 2008 to 2016 was performed. Groups were matched by sex, age at first visit, date of first visit, disease duration at first visit, and limited versus diffuse cutaneous disease. Demographic, serologic, and clinical features were compared. Mortality risks were assessed by a Cox proportional hazards model with covariates of race, marital status, education, employment, insurance, and imputed household income. RESULTS: African Americans comprised 202 of 402 patients. They demonstrated reduced forced vital capacity and diffusing capacity for carbon monoxide, more severe lung fibrosis, a higher prevalence of pulmonary hypertension, and more severe cardiac involvement. The autoantibody profile statistically differed between the 2 groups. Death during follow-up was 21% in African Americans versus 11% in non-African Americans (P = 0.005). African American race demonstrated an unadjusted hazard ratio for death during follow-up of 2.061 (P = 0.006) that declined with adjustment for socioeconomic covariates to 1.256 (P = 0.633). The only significant covariate was median income in tens of thousands of dollars by zip code (hazard ratio 0.845; P = 0.033). CONCLUSION: African American patients with scleroderma have more severe pulmonary disease and higher unadjusted mortality than matched non-African Americans. Following adjustment for socioeconomic factors, African American race was not a significant risk factor for mortality; however, independent of race, a lower median household income predicted increased mortality.

7.
Chest ; 155(3): 565-586, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30660783

RESUMO

BACKGROUND: Pulmonary arterial hypertension (PAH) carries a poor prognosis if not promptly diagnosed and appropriately treated. The development and approval of 14 medications over the last several decades have led to a rapidly evolving approach to therapy, and have necessitated periodic updating of evidence-based treatment guidelines. This guideline statement, which now includes a visual algorithm to enhance its clinical utility, represents the fourth iteration of the American College of Chest Physicians Guideline and Expert Panel Report on Pharmacotherapy for PAH. METHODS: The guideline panel conducted an updated systematic review to identify studies published after those included in the 2014 guideline. A systematic literature search was conducted using MEDLINE via PubMed and the Cochrane Library. The quality of the body of evidence was assessed for each critical or important outcome of interest using the Grading of Recommendations Assessment, Development and Evaluation approach. Graded recommendations and ungraded consensus-based statements were developed and voted on using a modified Delphi technique to achieve consensus. RESULTS: Two new recommendations on combination therapy and two ungraded consensus-based statements on palliative care were developed. An evidence-based and consensus-driven treatment algorithm was created to guide the clinician through an organized approach to management, and to direct readers to the appropriate area of the document for more detailed information. CONCLUSIONS: Therapeutic options for the patient with PAH continue to expand through basic discovery, translational science, and clinical trials. Optimal use of new treatment options requires prompt evaluation at an expert center, utilization of current evidence-based guidelines, and collaborative care using sound clinical judgment.

8.
Arthritis Rheumatol ; 71(2): 182-195, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30604506
10.
J Rheumatol ; 2018 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-30275260

RESUMO

OBJECTIVE: We sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for pulmonary hypertension (PH) and enrolled in PHAROS, a prospective cohort study to investigate the natural history of PH in systemic sclerosis (SSc). METHODS: The at-risk population for PH was defined by the following entry criteria: echocardiogram systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing. Baseline clinical measures were evaluated as predictors of hospitalization and death between 2005 and 2014. Cox proportional hazards models were censored at date of PH onset or latest study visit and adjusted for age, sex, race, and disease duration. RESULTS: Of the 236 at-risk subjects who were followed for a median of 4 years (range 0.4-8.5 yrs), 35 developed PH after entering PHAROS (reclassified as PH group). In the at-risk group, higher mortality was strongly associated with male sex, low %DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea scores, and baseline pericardial effusion. Risks for cardiopulmonary hospitalization were associated with increased dyspnea and pericardial effusions, although PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations. CONCLUSION: Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, including male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. This group should undergo right heart catheterization and receive appropriate intervention if PH is confirmed.

11.
Arthritis Rheumatol ; 70(10): 1654-1660, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29732714

RESUMO

OBJECTIVE: Whole-exome sequencing (WES) studies in systemic sclerosis (SSc) patients of European American (EA) ancestry have identified variants in the ATP8B4 gene and enrichment of variants in genes in the extracellular matrix (ECM)-related pathway that increase SSc susceptibility. This study was undertaken to evaluate the association of the ATP8B4 gene and the ECM-related pathway with SSc in a cohort of African American (AA) patients. METHODS: SSc patients of AA ancestry were enrolled from 23 academic centers across the US under the Genome Research in African American Scleroderma Patients consortium. Unrelated AA individuals without serologic evidence of autoimmunity who were enrolled in the Howard University Family Study were used as unaffected controls. Functional variants in genes reported in the 2 WES studies in EA patients with SSc were selected for gene association testing using the optimized sequence kernel association test (SKAT-O) and pathway analysis by Ingenuity Pathway Analysis in 379 patients and 411 controls. RESULTS: Principal components analysis demonstrated that the patients and controls had similar ancestral backgrounds, with roughly equal proportions of mean European admixture. Using SKAT-O, we examined the association of individual genes that were previously reported in EA patients and none remained significant, including ATP8B4 (P = 0.98). However, we confirmed the previously reported association of the ECM-related pathway with enrichment of variants within the COL13A1, COL18A1, COL22A1, COL4A3, COL4A4, COL5A2, PROK1, and SERPINE1 genes (corrected P = 1.95 × 10-4 ). CONCLUSION: In the largest genetic study in AA patients with SSc to date, our findings corroborate the role of functional variants that aggregate in a fibrotic pathway and increase SSc susceptibility.

13.
Pulm Circ ; 8(2): 2045893218757404, 2018 Apr-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29468935

RESUMO

Group classification of pulmonary hypertension (PH) is based on pulmonary artery wedge pressure (PAWP) on right heart catheterization (RHC). How hemodynamics, particularly PAWP, change over time in systemic sclerosis (SSc)-PH patients is unknown. SSc-PH patients enrolled in the prospective observational PHAROS registry who had > 1 RHC (n = 120) were included in this analysis. Patients were considered to have a "PAWP class change" if they had a PAWP ≤ 15 mmHg on RHC-1 and then a PAWP > 15 on RHC-2 or had a PAWP > 15 on RHC-1 and then PAWP ≤ 15 on RHC-2. There was a median time of 1.4 years between RHC-1 and RHC-2 and 75% of patients had a PH medication added after their initial RHC. PAWP increased significantly (11 ± 5 versus 13 ± 6 mmHg, P = 0.01) between RHC-1 and RHC-2, particularly for patients who were started on PH medications. Overall, 30% of patients who had a repeat RHC experienced a PAWP class change between their initial and follow-up RHC, independent of whether a PH medication was added. Patients initially classified as World Health Organization group 2 PH were most likely to change PAWP class over time. In conclusion, PAWP values commonly change to a significant degree in SSc-PH, which highlights the challenges in using a single time-point PAWP to define clinical classification groups.

14.
PLoS One ; 13(1): e0189498, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29293537

RESUMO

Gene-level analysis of ImmunoChip or genome-wide association studies (GWAS) data has not been previously reported for systemic sclerosis (SSc, scleroderma). The objective of this study was to analyze genetic susceptibility loci in SSc at the gene level and to determine if the detected associations were shared in African-American and White populations, using data from ImmunoChip and GWAS genotyping studies. The White sample included 1833 cases and 3466 controls (956 cases and 2741 controls from the US and 877 cases and 725 controls from Spain) and the African American sample, 291 cases and 260 controls. In both Whites and African Americans, we performed a gene-level analysis that integrates association statistics in a gene possibly harboring multiple SNPs with weak effect on disease risk, using Versatile Gene-based Association Study (VEGAS) software. The SNP-level analysis was performed using PLINK v.1.07. We identified 4 novel candidate genes (STAT1, FCGR2C, NIPSNAP3B, and SCT) significantly associated and 4 genes (SERBP1, PINX1, TMEM175 and EXOC2) suggestively associated with SSc in the gene level analysis in White patients. As an exploratory analysis we compared the results on Whites with those from African Americans. Of previously established susceptibility genes identified in Whites, only TNFAIP3 was significant at the nominal level (p = 6.13x10-3) in African Americans in the gene-level analysis of the ImmunoChip data. Among the top suggestive novel genes identified in Whites based on the ImmunoChip data, FCGR2C and PINX1 were only nominally significant in African Americans (p = 0.016 and p = 0.028, respectively), while among the top novel genes identified in the gene-level analysis in African Americans, UNC5C (p = 5.57x10-4) and CLEC16A (p = 0.0463) were also nominally significant in Whites. We also present the gene-level analysis of SSc clinical and autoantibody phenotypes among Whites. Our findings need to be validated by independent studies, particularly due to the limited sample size of African Americans.


Assuntos
Grupo com Ancestrais do Continente Africano/genética , Grupo com Ancestrais do Continente Europeu/genética , Estudo de Associação Genômica Ampla , Escleroderma Sistêmico/genética , Humanos , Polimorfismo de Nucleotídeo Único
15.
Arthritis Care Res (Hoboken) ; 70(10): 1517-1524, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29316366

RESUMO

OBJECTIVE: We examined systemic sclerosis (SSc) patients with breast cancer to identify the prevalence of radiation complications and to examine outcomes in SSc patients who received radiation therapy as part of their cancer treatment. METHODS: Patients with SSc and breast cancer were identified from the Johns Hopkins and University of Pittsburgh Scleroderma Center databases. We examined whether erythema, blistering, ulceration, or thickening of the skin developed in the radiation therapy port. Changes in modified Rodnan skin thickness score (mRSS) and forced vital capacity percent predicted (FVC%) at 12 and 24 months post-cancer diagnosis were compared between patients who did and those who did not receive radiation therapy. RESULTS: A total of 43 of 116 breast cancer patients at Johns Hopkins and 26 of 37 patients at the University of Pittsburgh received breast radiation therapy. At Johns Hopkins, 4 of 30 (13.3%) patients with available data developed erythema, none had blistering, 1 of 30 (3.3%) developed ulceration, and 15 of 31 (48.4%) had skin thickening in the radiation port. At the University of Pittsburgh, 7 of 11 patients (63.6%) with available data developed erythema, 2 of 11 (18.2%) had blistering, none developed ulceration, and 6 of 11 (54.6%) had skin thickening in the radiation port. In a limited sample, there were no significant changes in the mRSS or FVC% between patients who did and those who did not receive radiation therapy. CONCLUSION: These data suggest that radiation injury causing local tissue fibrosis is not inevitable in SSc patients with breast cancer, occurring in approximately 50% of patients without evidence of lung or generalized skin disease flare. Therefore, the use of radiation therapy for breast cancer is considered an option based on the informed patient's preference.

16.
J Rheumatol ; 45(3): 405-410, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29093152

RESUMO

OBJECTIVE: To evaluate the safety and efficacy of pomalidomide (POM) on forced vital capacity (FVC), modified Rodnan skin score (mRSS), and gastrointestinal (GI) symptomatology over 52 weeks of treatment in patients with interstitial lung disease due to systemic sclerosis (SSc). METHODS: Twenty-three adult patients diagnosed with SSc were randomized 1:1 POM:placebo (PBO). RESULTS: Mean change at Week 52 from baseline in predicted FVC% -5.2 and -2.8; mRSS -2.7 and -3.7; and UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract (SCTC GIT 2.0) score 0.1 and 0.0, with POM and PBO, respectively. Statistical significance was not achieved for any of these 3 primary endpoints at 52 weeks. CONCLUSION: Because of recruitment challenges, subject enrollment was discontinued early. In an interim analysis, the study did not meet its Week 52 primary endpoints. Therefore, a decision was made to terminate all study phases. POM was generally well tolerated, with an adverse event profile consistent with the known safety and tolerability profile of POM in other diseases. Study results were neither positive nor negative because too few subjects were enrolled to make meaningful conclusions. Clinical Trials number: NCT01559129.

17.
Autoimmun Rev ; 16(10): 1029-1035, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28778706

RESUMO

The pathophysiology of SSc-mediated organ damage is complex and not well understood. Hallmarks of the disease include skin thickening, vasculopathy and gastrointestinal dysmotility. Diverse anti-nuclear antibodies can be used as biomarkers for classification and prognosis, but their role in producing tissue pathology/organ dysfunction is not established. In contrast, antibodies against cell surface receptors for platelet derived growth factor, angiotensin II, endothelin A, ICAM-1, and type 3 muscarinic acetyl choline receptors may play a major role in skin thickening, vasoconstriction/pulmonary and renal hypertension, ischemia and gastrointestinal dysmotility, respectively. In addition, antibodies to an inhibitory B-lymphocyte surface molecule, CD 22, may allow increased production of other autoantibodies. Each of these types of antibodies have been reported in some SSc patients, and laboratory studies suggest signaling pathways and mechanisms by which they may contribute to disease activity. However, we are far from a consensus on their importance. Additional epidemiologic, mechanistic and physiologic studies are needed. Confirmation of the roles of anti-receptor antibodies and identification of the signaling pathways by which they alter cellular functions would have major implications for treatment of SSc, both in terms of targeting autoantibodies and the cells that produce them, and in the use of small molecules which inhibit their pernicious effects.


Assuntos
Autoanticorpos/imunologia , Escleroderma Sistêmico/genética , Humanos , Escleroderma Sistêmico/fisiopatologia
18.
Respirology ; 22(7): 1386-1392, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28500695

RESUMO

BACKGROUND AND OBJECTIVE: Systemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP. METHODS: Patients from the PHAROS registry (a prospective observational cohort of SSc-PH patients) who had mean PAP ≥ 25 and PAWP > 15 on right heart catheterization were stratified based on diastolic pressure gradient (DPG). Kaplan-Meier analysis was performed to compare survival and PH-related hospitalization. Baseline factors were compared between patients dying and those who survived using Cox regression analysis. RESULTS: A total of 59 patients were included, of whom 21 (36%) patients were classified as combined PH and 38 (64%) had post-capillary PH. No baseline characteristics were significantly different between the two groups. There were no differences in survival or PH-related hospitalization between the groups. The only baseline factor independently associated with death was lower 6-min walk distance (6MWD) (hazard ratio (HR): 1.33 per 25 m decrease, 95% CI: 1.11-1.59, P = 0.002). PH-specific medications were started during follow-up in significantly more patients in the combined PH group compared with the post-capillary group (86% vs 50%, P = 0.01). CONCLUSION: Outcomes were similar between SSc patients with post-capillary PH and combined pre- and post-capillary PH. 6MWD at baseline can predict risk for death in SSc patients with PH and an elevated PAWP. More patients with combined PH were started on PH-specific medications, and the clinical benefit of treating this subgroup specifically in SSc patients needs further exploration.


Assuntos
Progressão da Doença , Hipertensão Pulmonar/fisiopatologia , Pressão Propulsora Pulmonar/fisiologia , Escleroderma Sistêmico/fisiopatologia , Cateterismo Cardíaco , Estudos de Coortes , Feminino , Hospitalização , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/mortalidade , Análise de Sobrevida
19.
J Rheumatol ; 44(6): 791-794, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28298560

RESUMO

OBJECTIVE: To determine the inter/intraobserver reliability of the tender and swollen joint counts (TJC, SJC) and the modified Rodnan Skin Score (mRSS) in diffuse cutaneous systemic sclerosis (dcSSc) and to assess content validity of the TJC/SJC. METHODS: Ten rheumatologists completed the SJC, TJC, and mRSS on 7 patients. Musculoskeletal ultrasound (MSUS) was performed. RESULTS: Interobserver and intraobserver reliability for the TJC was 0.97 and 0.99, for the SJC was 0.24 and 0.71, and for the mRSS was 0.81 and 0.94, respectively. MSUS abnormalities did not correspond with SJC/TJC. CONCLUSION: We demonstrate excellent inter- and intraobserver reliability for the mRSS and TJC in dcSSc. However, the SJC and TJC did not correspond to MSUS.


Assuntos
Articulações/patologia , Esclerodermia Difusa/diagnóstico , Pele/patologia , Adulto , Avaliação da Deficiência , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Físico , Estudos Prospectivos , Sistema de Registros , Reprodutibilidade dos Testes , Esclerodermia Difusa/patologia , Índice de Gravidade de Doença , Adulto Jovem
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