A5077574387- Systemic Sclerosis and Related Diseases
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Dermatologic Treatments and Research
- Mast cells and histamine
- Inflammatory Myopathies and Dermatomyositis
- Pulmonary Hypertension Research and Treatments
- Autoimmune Bullous Skin Diseases
- Eosinophilic Disorders and Syndromes
- Connective Tissue Growth Factor Research
- Rheumatoid Arthritis Research and Therapies
- Skin Diseases and Diabetes
- Diagnosis and Treatment of Venous Diseases
- IL-33, ST2, and ILC Pathways
- Body Image and Dysmorphia Studies
- Skin and Cellular Biology Research
- Systemic Lupus Erythematosus Research
- Cutaneous lymphoproliferative disorders research
- Urticaria and Related Conditions
- Renal Diseases and Glomerulopathies
- Multiple Sclerosis Research Studies
- Renal and Vascular Pathologies
- Neuroendocrine Tumor Research Advances
- Eosinophilic Esophagitis
- Pancreatic and Hepatic Oncology Research
- Celiac Disease Research and Management
University of Pittsburgh
2016-2025
University of Pittsburgh Medical Center
2016-2025
University of Rhode Island
2023-2024
University of North Carolina at Chapel Hill
2023-2024
AID Atlanta
2023-2024
American College of Rheumatology
2023-2024
Emory University
2023-2024
Convergence
2024
Durham VA Medical Center
2023-2024
eLearning Studios (United Kingdom)
2023
Despite current therapies, diffuse cutaneous systemic sclerosis (scleroderma) often has a devastating outcome. We compared myeloablative CD34+ selected autologous hematopoietic stem-cell transplantation with immunosuppression by means of 12 monthly infusions cyclophosphamide in patients scleroderma.
T cells play a key role in the pathogenesis of early systemic sclerosis. This study was undertaken to assess safety and efficacy abatacept patients with diffuse cutaneous sclerosis (dcSSc).
Abstract Skin and lung fibrosis in systemic sclerosis (SSc) is driven by myofibroblasts, alpha-smooth muscle actin expressing cells. The number of myofibroblasts SSc skin correlates with the modified Rodnan score, most widely used clinical measure disease severity. Murine models indicate that can arise from a variety different cell types, but their origin has remained uncertain. Utilizing single RNA-sequencing, we define dermal fibroblast populations transcriptome changes, comparing to...
Rationale: Systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) is one of the most prevalent and deadly forms PAH. B cells may contribute to SSc pathogenesis. Objectives: We investigated safety efficacy B-cell depletion for SSc-PAH. Methods: In an NIH-sponsored, multicenter, double-blinded, randomized, placebo-controlled, proof-of-concept trial, 57 patients with SSc-PAH on stable-dose standard medical therapy received two infusions 1,000 mg rituximab or placebo administered 2 weeks...
Abstract Background Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial lung disease (ILD) common in SSc, occurring 35–52% of patients and accounting for 20–40% mortality. Evolution therapeutic options has resulted lack consensus on how to manage this condition. This Delphi study was initiated develop recommendations based expert physician insights regarding screening, progression, treatment criteria, monitoring response, the role recent advances with...
We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs).
Objective We provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, Sjögren disease. Methods developed clinically relevant population, intervention, comparator, outcomes questions related to patients SARDs. A systematic literature review was...
Objective We provide evidence‐based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). Methods developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, available evidence rated using Grading Recommendations, Assessment, Development, Evaluation methodology. panel clinicians patients reached consensus on direction strength...
Although there is a wide array of outcome tools for assessing patients with symptomatic ankle arthritis, no disease-specific instrument arthritis has been shown to be reliable and valid. The purpose this study was develop simple, reliable, validated measure the clinical assessment osteoarthritis. We modified Foot Function Index, visual analog-based scale used assess rheumatoid foot problems, patient symptoms functional limitations stemming from osteoarthritis joint. Test-retest reliability...
Abstract Objective Previous case series have examined the relationship between anti–Jo‐1 antibody levels and myositis disease activity, demonstrating equivocal results. Using enzyme‐linked immunosorbent assays (ELISAs) novel measures of current study was undertaken to systematically reexamine association various manifestations myositis. Methods Serum were quantified using 2 independent ELISA methods, while activity retrospectively graded Myositis Disease Activity Assessment Tool, which in 7...
Objective To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers the US. Methods The Pulmonary Hypertension Assessment Recognition Outcomes Scleroderma registry is a prospective high risk or definite diagnosed by right‐sided heart catheterization within 6 months enrollment. Only World Health Organization...
Epidemiology studies suggest that systemic sclerosis (SSc) is more common, occurs at a younger age, and severe in African Americans than Caucasians. However, the scleroderma autoantibody profile very different between these 2 ethnic groups. This study was undertaken to examine demographic disease features, frequency severity of internal organ system involvement, survival American patients compared Caucasian with SSc, giving particular attention their serum profiles.Demographic clinical...
<h3>Objectives</h3> Determine global skin transcriptome patterns of early diffuse systemic sclerosis (SSc) and how they differ from later disease. <h3>Methods</h3> Skin biopsy RNA 48 patients in the Prospective Registry for Early Systemic Sclerosis (PRESS) cohort (mean disease duration 1.3 years) 33 matched healthy controls was examined by next-generation sequencing. Data were analysed cell type-specific signatures compared with similarly obtained data 55 previously biopsied Genetics versus...
Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc African Americans, we established a comprehensive clinical database from largest multicenter cohort American patients assembled to date (the Genome Research Scleroderma Patients (GRASP) cohort). were enrolled retrospectively and prospectively over 30-year period (1987–2016), 18 academic centers throughout United States. The cross-sectional prevalence sociodemographic, clinical,...
To assess the safety and efficacy of lenabasum in diffuse cutaneous systemic sclerosis (dcSSc).A randomized, double-blind, placebo-controlled, phase II study was conducted at 9 SSc clinics US. Adults with dcSSc ≤6 years' duration who were receiving stable standard-of-care treatment randomized to receive (n = 27) or placebo 15). Lenabasum doses 5 mg once daily, 20 twice daily for 4 weeks, followed by 8 weeks. Safety assessed weeks 4, 8, 12, 16.Adverse events (AEs) occurred 63% group 60%...
In this study, we sought a comprehensive understanding of myeloid cell types driving fibrosis in diffuse cutaneous systemic sclerosis (dcSSc) skin.We analyzed the transcriptomes 2,465 cells from skin biopsy specimens 12 dcSSc patients and 10 healthy control subjects using single-cell RNA sequencing. Monocyte-derived dendritic (mo-DCs) were assessed immunohistochemical staining immunofluorescence analyses targeting ficolin-1 (FCN-1).A t-distributed stochastic neighbor embedding analysis...
Although T cells have been implicated in the pathogenesis of systemic sclerosis (SSc), a comprehensive study T-cell-mediated immune responses affected skin patients with progressive SSc is lacking. Droplet-based single-cell transcriptome analysis biopsies opens avenues for dissecting patient-specific T-cell heterogeneity, providing basis identifying novel gene expression related to functional pathways associated severity disease.Single-cell RNA sequencing was performed by droplet-based (10x...
Objective We provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, Sjögren disease. Methods developed clinically relevant population, intervention, comparator, outcomes questions related to patients SARDs. A systematic literature review was...
To characterize a new serum autoantibody in patients with systemic sclerosis (SSc) directed against U11/U12 RNP and to identify the clinical features associated this autoantibody.We identified autoantibodies complex sera of SSc confirmed antibody specificity by immunoprecipitation, reverse transcriptase-polymerase chain reaction, Southern blotting. We determined prevalence these antibodies their for SSc. compared anti-U11/U12 autoantibody-positive negative on demographic, disease...
Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It associated with increased vascular permeability, activation coagulation cascade, and renin secretion, which may lead to the acute failure typically accelerated hypertension. The histologic picture SRC that a thrombotic microangiopathy process prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies...
Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pressures (21-24 are "at risk" of developing hypertension (PH). The objectives this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) mPAP explore long-term outcomes SSc patients versus haemodynamics.PHAROS is a multicentre prospective longitudinal cohort or recently diagnosed resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, function...