Related Experiment Video
Updated: May 6, 2026
14:27
Identification of Disease-related Spatial Covariance Patterns using Neuroimaging Data
Published on: June 26, 2013
15.6K
Phenotypes and prognosis of systemic sclerosis: A cluster analysis
Stefan Nowak1, Dany Jaffuel1, Jacques Morel2
1Department of Respiratory Disease, PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR 9214, CHU Montpellier, Montpellier, France.
Respiratory Medicine and Research
|April 4, 2025
Summary
Systemic sclerosis patient groups were identified, revealing distinct clinical presentations and prognoses. Pulmonary hypertension significantly impacted survival rates across these identified systemic sclerosis cohorts.
Area of Science:
- Rheumatology
- Immunology
- Pulmonology
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease with high morbidity and mortality, largely driven by pulmonary complications like diffuse interstitial lung disease (ILD) and pulmonary hypertension (PH).
- The heterogeneous presentation of SSc makes disease course prediction challenging.
- This study focused on creating homogeneous patient cohorts within severe systemic sclerosis to better understand prognosis.
Purpose of the Study:
- To stratify patients with severe systemic sclerosis into homogeneous subgroups.
- To analyze the clinical characteristics and prognostic factors within each subgroup.
- To investigate the impact of specific complications, such as pulmonary hypertension and interstitial lung disease, on patient outcomes.
Main Methods:
- A retrospective observational study was conducted on 198 patients with systemic sclerosis.
- Bottom-up hierarchical clustering was employed to group patients based on factors associated with poor prognosis.
- Patient data included clinical presentation, autoantibody profiles, organ involvement, and occupational exposure.
Main Results:
- Four distinct patient clusters emerged: Cluster 1 (n=25) with high prevalence of PH and ILD; Cluster 2 (n=11) with scleroderma renal crisis and some PH; Cluster 3 (n=109) predominantly female patients with limited cutaneous SSc and anticentromere antibodies, without severe pulmonary disease; Cluster 4 (n=53) with occupational exposure, ILD, and anti-topoisomerase I antibodies.
- Survival rates were significantly lower in Cluster 1 (p < 0.001).
- Pulmonary hypertension was a key factor associated with adverse prognosis across clusters.
Conclusions:
- Systemic sclerosis exhibits significant heterogeneity in clinical phenotypes and disease trajectories.
- Hierarchical clustering effectively identified distinct patient subgroups with varying prognoses.
- The development of pulmonary hypertension is a critical determinant of poor prognosis in systemic sclerosis patients.
Related Concept Videos
01:29Psychological and Sociocultural Causes of Schizophrenia
984
Schizophrenia, a complex psychiatric disorder, has been historically misunderstood. Early psychological theories attributed its origins to childhood trauma and unresponsive parenting. However, contemporary research largely rejects these notions, favoring the vulnerability-stress hypothesis. This model proposes that individuals with a genetic predisposition to schizophrenia may develop the disorder following exposure to significant environmental stressors. Notably, studies on high-risk...
984
01:19Multiple Sclerosis l: Introduction
31
Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
31