LeRoy Ec

Bio: LeRoy Ec is an academic researcher from Medical University of South Carolina. The author has contributed to research in topics: Scleroderma & Vascular disease. The author has an hindex of 10, co-authored 11 publications receiving 5967 citations.

Criteria for the classification of early systemic sclerosis.

Abstract: We propose criteria for the early diagnosis and classification of systemic sclerosis that reflect the vascular and serological advances of the last 2 decades.

A disease severity scale for systemic sclerosis: development and testing.

Abstract: Objective To develop and test a severity scale for individual organ involvements in systemic sclerosis (SSc, scleroderma). Methods An international study group completed the following tasks: (1) developed a glossary of terms including all pertinent variables for 9 potentially affected organ systems; (2) collected prospective data to determine the feasibility and practicality of each proposed variable; (3) revised the initial list of variables; (4) determined the association of each variable with mortality (a proxy for morbidity) using 579 patients in an existing comprehensive longitudinal scleroderma databank; (5) developed a severity grading scale for each organ system by discussion and consensus; and (6) externally validated the scale using an independent group of 680 patients from the same databank. Results Nine organ-specific severity scales were developed from 0 (no documented involvement) to 4 (endstage disease). The data required for scale completion are relatively easy and practical for all physicians to obtain. Conclusion This preliminary severity scale will be useful for assessing disease severity status in individual patients both at one point in time and longitudinally. The severity scale will assist in the design and conduct of clinical trials and the comparison of study populations with one another. The scale will serve as a framework for developing a scleroderma disease activity index.

Raynaud's phenomenon: a proposal for classification.

Abstract: Major confusion exists with regard to the definition of patients with Raynaud's phenomenon; defining the patient and the phenomenon are reasonably straightforward, but variations in the definition of its primary and secondary forms have created a situation in which the same patient might be classified as primary by one group and secondary by another. The present essay is a proposal for the strict definition of Primary Raynaud's Phenomenon (PRP) formulated as a hypothesis amenable to experimental testing. This hypothesis is tested retrospectively on a group of 240 patients with Raynaud's phenomenon. The proposed criteria permit classification in 215 of 240 cases or 89%, leaving 25 patients difficult to classify at initial evaluation. Further testing of the hypothesis is encouraged.

SYSTEMIC SCLEROSIS : A Vascular Perspective

Abstract: The evidence that generalized scleroderma (systemic sclerosis, SSc) is a vascular disease is compelling. Raynaud's phenomenon and subcutaneous edema are early manifestations, whereas vascular insufficiency from both arterial intimal scarring and microvessel obliteration in many organs compromises critical organ function, determines mortality and morbidity in the individual patient, and in association with fibrosis provides in the skin the fundamental pathogenetic rationale for dividing SSc into subsets that can empirically be shown to be prognostically meaningful. Consider the extent of the clinical problem. Between 5% and 30% of adults worldwide can be shown to have the cold- and stress-induced exaggerated yet intermittent vasospasm called Raynaud's phenomenon (RP). Now well-established microscopic (nailfold capillaroscopy) and serologic (indirect immunofluorescence with rapidly dividing human cells such as HEp-2) laboratory determinations can select a small proportion of RP subjects (estimated to be 10% of all RP-positive and up to 50% of clinically referred RP-positive subjects) who, when capillary- and serology-positive, join the mild end of the spectrum of SSc with limited cutaneous systemic sclerosis. Thus, in the definable portion of adults who are Raynaud's-positive, and either capillary-positive or serology-positive, we have a group of subjects in which to study the natural history of at least limited cutaneous SSc and to interrupt the pathogenetic process when we understand how to intervene safely. 7,24,28,44 It follows, therefore, that a detailed understanding of the vascular pathogenesis and its relationship to the fibrosis characteristic of SSc could directly influence our management and therapy of this difficult disease. Such a premise is the basis of this article.

2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative

Abstract: OBJECTIVE: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. METHODS: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. RESULTS: It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. CONCLUSION: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.

Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States

Abstract: Objective To provide a single source for the best available estimates of the national prevalence of arthritis in general and of selected musculoskeletal disorders (osteoarthritis, rheumatoid arthritis, juvenile rheumatoid arthritis, the spondylarthropathies, systemic lupus erythematosus, scleroderma, polymyalgia rheumatica/giant cell arteritis, gout, fibromyalgia, and low back pain). Methods The National Arthritis Data Workgroup reviewed data from available surveys, such as the National Health and Nutrition Examination Survey series. For overall national estimates, we used surveys based on representative samples. Because data based on national population samples are unavailable for most specific musculoskeletal conditions, we derived data from various smaller survey samples from defined populations. Prevalence estimates from these surveys were linked to 1990 US Bureau of the Census population data to calculate national estimates. We also estimated the expected frequency of arthritis in the year 2020. Results Current national estimates are provided, with important caveats regarding their interpretation, for self-reported arthritis and selected conditions. An estimated 15% (40 million) of Americans had some form or arthritis in 1995. By the year 2020, an estimated 18.2% (59.4 million) will be affected. Conclusion Given the limitations of the data on which they are based, this report provides the best available prevalence estimates for arthritis and other rheumatic conditions overall, and for selected musculoskeletal disorders, in the US population.

2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative

Abstract: Objective The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. Methods Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by (1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and (2) validating against the combined view of a group of experts on a set of cases with or without SSc. Results It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, seven additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud9s phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. Conclusions The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.

Criteria for the classification of early systemic sclerosis.

Abstract: We propose criteria for the early diagnosis and classification of systemic sclerosis that reflect the vascular and serological advances of the last 2 decades.

Systemic sclerosis: a prototypic multisystem fibrotic disorder.

Abstract: A unique feature of systemic sclerosis (SSc) that distinguishes it from other fibrotic disorders is that autoimmunity and vasculopathy characteristically precede fibrosis. Moreover, fibrosis in SSc is not restricted to a single organ, but rather affects many organs and accounts for much of the morbidity and mortality associated with this disease. Although immunomodulatory drugs have been used extensively in the treatment of SSc, no therapy to date has been able to reverse or slow the progression of tissue fibrosis or substantially modify the natural progression of the disease. In this Review, we highlight recent studies that shed light on the cellular and molecular mechanisms underlying the fibrotic process in SSc and that identify cellular processes and intra- and extracellular proteins as potential novel targets for therapy in this prototypic multisystemic fibrotic disease.