Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma: outcomes from a multicenter US-based systemic sclerosis registry

Shanmugam, Victoria K.; Frech, Tracy; Steen, Virginia D.; Hummers, Laura K.; Shah, Ami A.; Bernstein, Elana J.; Khanna, Dinesh; Gordon, Jessica; Castelino, Flavia V.; Chung, Lorinda; Hant, Faye N.; Startup, Emily; VanBuren, John M.; Evnin, Luke; Assassi, Shervin

doi:10.1007/s10067-019-04792-y

Cited by 12 publications

(10 citation statements)

References 19 publications

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“…The main marker antibodies for SSc are anti-RNA polymerase III antibodies (a-RNA Pol III), anti-topoisomerase I antibodies (a-topo I) and anti-centromere antibodies (ACAs). They are associated with a typical subset and clinical manifestations of the disease [1,2,3]. Furthermore, they are listed among the ACR/EULAR classification criteria for systemic sclerosis [4].…”

Section: Introductionmentioning

confidence: 99%

The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis

Wielosz¹,

Dryglewska²,

Majdan³

2020

Ann Agric Environ Med.

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Introduction. Anti-PM/Scl (a-PM/Scl) antibodies are found in different systemic autoimmune diseases such as polymyositis, dermatomyositis, systemic sclerosis (SSc) and overlap syndromes. According to literature, they are detected in approx. 2% of SSc patients, but their presence is more common in SSc with myositis overlap. Objective. The aim of the study was to assess the prevalence of a-PM/Scl in patients with SSc and to identify differences in the clinical profile of the disease in patients with the presence of a-PM/Scl. Materials and method. The study was performed on 126 European Caucasian SSc patients (98 females and 28 males) hospitalized consecutively in the Department of Rheumatology and Connective Tissue Diseases. The study group was analyzed for the potential presence of a-PM/Scl using a commercial test-EUROLINE Systemic Sclerosis Profile. The detection and interpretation were carried out electronically using the specific Euroimmun-EUROLineScan programme. The subtype of SSc, incidence of internal organ involvement and serological profile were determined in the entire group. Due to the presence of a-PM/Scl, patients were divided into two groups: a-PM/Scl (+) SSc-22 patients and a-PM/Scl (-) SSc-104 patients. Results. A-PM/Scl was detected in 22/126 patients with SSc (17.5%). A strong correlation was found between a-PM/Scl and myalgia or myositis (p = 0.0379), hand joints contractures (p = 0.0002) and the prevalence of overlap syndrome (p = 0.0142). There were no relationships between the presence of a-PM/Scl and subtypes of SSc, other organ involvement, digital ulcers or calcinosis. Conclusions. Anti-PM/Scl antibodies are fairly common in patients with systemic sclerosis. In SSc, anti-PM/Scl antibodies are frequently associated with myalgia or myositis, hand joint contractures and an overlap syndrome.

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Section: Introductionmentioning

confidence: 99%

The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis

Wielosz¹,

Dryglewska²,

Majdan³

2020

Ann Agric Environ Med.

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“…The female predominance with a female-to-male ratio of 16:3 is much lower than reported in regional cohorts such as Al-Adhadh and Al-Sayed[ 9 ] (8.3:1) and Ghosh et al[ 10 ] (8.3:1). Cohorts from the other continents, however, have shown similar female predominance, as shown by Carreira et al,[ 11 ] Coi et al,[ 12 ] Shanmugam et al,[ 13 ] and Walker et al[ 14 ] from the European Scleroderma Trials and Research (EUSTAR) group. Besides, our cohort had a higher percentage of patients suffering from diffuse scleroderma, which is not consistent with the reported literature, showing a higher percentage suffering from limited disease.…”

Section: Discussionmentioning

confidence: 54%

Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan

Asif¹,

Khan²,

Faiq³

et al. 2021

Arch Rheumatol

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Objectives: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. Patients and methods: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. Results: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). Conclusion: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity.

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“…We performed a prospective cohort study of patients with SSc enrolled in the Collaborative National Quality and Efficacy Registry (CONQUER) at 13 sites in the United States between June 6, 2018, and February 1, 2020. CONQUER is a US-based, prospective, multicenter cohort of adults 18 years of age or older with SSc who meet 2013 American College of Rheumatology/ European League Against Rheumatism Classification Criteria for SSc (7) and have a disease duration of 5 years or less from the first non-Raynaud's symptom at enrollment (8,9). Participants' medical records were reviewed to determine whether HRCT was ever performed, either at a CONQUER baseline or follow-up visit or prior to enrolling in CONQUER.…”

Section: Methodsmentioning

confidence: 99%

Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients With Systemic Sclerosis at Expert Scleroderma Centers in the United States

Bernstein

Assassi

Castelino

et al. 2022

ACR Open Rheumatology

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Objective Although a high‐resolution computed tomography (HRCT) scan of the chest is the gold standard test for the detection of interstitial lung disease (ILD), there is no consensus among rheumatologists regarding the use of HRCT to screen for ILD in their patients with systemic sclerosis (SSc). The aims of this study were to describe the HRCT ordering practices at SSc centers in the United States and to determine which patient characteristics are associated with HRCT performance. Methods We performed a prospective cohort study of patients with SSc enrolled in the US‐based Collaborative National Quality and Efficacy Registry (CONQUER). We performed univariate logistic regression followed by multivariable logistic regression to determine which patient characteristics were associated with HRCT performance. Results Of the 356 patients with SSc enrolled in CONQUER, 286 (80.3%) underwent HRCT at some point during their disease course. On multivariable analyses, missing total lung capacity percent predicted (odds ratio [OR] 3.26, 95% confidence interval [CI]: 1.53‐7.41, P = 0.007) was positively associated with ever having undergone HRCT, whereas a positive anti‐centromere antibody (OR 0.27, 95% CI: 0.12‐0.61, P = 0.008) and missing forced vital capacity percent predicted (OR 0.29, 95% CI: 0.10‐0.80, P = 0.005) were negatively associated with ever having undergone HRCT. There was a trend toward a positive association between crackles on pulmonary exam and ever having undergone HRCT (OR 2.28, 95% CI: 0.97‐6.05, P = 0.058), although this relationship did not reach statistical significance. Conclusion The majority of patients with SSc enrolled in CONQUER underwent HRCT. A positive anti‐centromere antibody was the key clinical variable inversely associated with performance of HRCT.

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Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma: outcomes from a multicenter US-based systemic sclerosis registry

Cited by 12 publications

References 19 publications

The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis

The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis

Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan

Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients With Systemic Sclerosis at Expert Scleroderma Centers in the United States

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