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Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: A longitudinal EUSTAR study
- Source :
- PLoS ONE, Vol 11, Iss 10, p e0163894 (2016), PLoS ONE
- Publication Year :
- 2016
- Publisher :
- Public Library of Science, 2016.
-
Abstract
- Objective Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. Methods In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. Results Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. Conclusion In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to ‘widen' the still very narrow ‘window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.
- Subjects :
- Male
Genetics and Molecular Biology (all)
Pulmonology
Blood Pressure
Vascular Medicine
Biochemistry
Scleroderma
Diagnostic Radiology
0302 clinical medicine
Retrospective Studie
Longitudinal Studies
lcsh:Science
integumentary system
Incidence
Arteries
Systolic Pressure
3. Good health
Echocardiography
Cohort
Regression Analysis
Human
medicine.medical_specialty
Imaging Techniques
Gastrointestinal Disease
Immunology
1100 General Agricultural and Biological Sciences
Skin Diseases
03 medical and health sciences
FEV1/FVC ratio
Rheumatology
1300 General Biochemistry, Genetics and Molecular Biology
Humans
Autoantibodies
Retrospective Studies
Aged
1000 Multidisciplinary
Biochemistry, Genetics and Molecular Biology (all)
Proportional hazards model
lcsh:R
Biology and Life Sciences
Proteins
medicine.disease
Agricultural and Biological Sciences (all)
Blood Vessels
lcsh:Q
Clinical Medicine
Ejection Fraction
Lung Diseases
Kidney Disease
Physiology
Gastrointestinal Diseases
lcsh:Medicine
Longitudinal Studie
Kaplan-Meier Estimate
Medicine (all)
Lung Disease
Pericardial effusion
Risk Factors
Medizinische Fakultät
Immune Physiology
Ultrasound Imaging
Medicine and Health Sciences
030212 general & internal medicine
Pulmonary Arteries
Connective Tissue Diseases
Immune System Proteins
Pulmonary Hypertension
Multidisciplinary
Ejection fraction
Radiology and Imaging
Incidence (epidemiology)
10051 Rheumatology Clinic and Institute of Physical Medicine
Middle Aged
Autoantibodie
Heart Disease
Kidney Diseases
Female
Anatomy
Research Article
Adult
Heart Diseases
Cardiology
610 Medicine & health
Research and Analysis Methods
Antibodies
Regression Analysi
Autoimmune Diseases
Diagnostic Medicine
Internal medicine
medicine
ddc:610
030203 arthritis & rheumatology
Scleroderma, Systemic
business.industry
Risk Factor
Skin Disease
Raynaud Disease
Retrospective cohort study
Surgery
Cardiovascular Anatomy
Clinical Immunology
business
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- PLoS ONE, Vol 11, Iss 10, p e0163894 (2016), PLoS ONE
- Accession number :
- edsair.doi.dedup.....ddaf64db61b7068f2891952ddf048fc1