Exploratory clinical subgroup clustering in systemic sclerosis : Results from the Indian Progressive Systemic Sclerosis Registry
© The Author(s) 2023..
Objective: To conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry.
Methods: Patients satisfying American College of Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared.
Results: Of the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 (n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 (n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 (n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 (n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters.
Conclusion: With exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:9 |
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| Enthalten in: |
Journal of scleroderma and related disorders - 9(2024), 1 vom: 13. Feb., Seite 29-37 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Philip, Shery Susan [VerfasserIn] |
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| Links: |
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| Themen: |
Anti-centromere antibody |
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| Anmerkungen: |
Date Revised 10.02.2024 published: Print-Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1177/23971983231215470 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM368230163 |
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| 100 | 1 | |a Philip, Shery Susan |e verfasserin |4 aut | |
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| 500 | |a published: Print-Electronic | ||
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| 520 | |a © The Author(s) 2023. | ||
| 520 | |a Objective: To conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry | ||
| 520 | |a Methods: Patients satisfying American College of Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared | ||
| 520 | |a Results: Of the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 (n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 (n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 (n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 (n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters | ||
| 520 | |a Conclusion: With exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Systemic sclerosis | |
| 650 | 4 | |a anti-centromere antibody | |
| 650 | 4 | |a anti-topoisomerase I | |
| 650 | 4 | |a cluster analysis | |
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| 700 | 1 | |a Bairwa, Devender |e verfasserin |4 aut | |
| 700 | 1 | |a Sircar, Geetabali |e verfasserin |4 aut | |
| 700 | 1 | |a Ghosh, Parasar |e verfasserin |4 aut | |
| 700 | 1 | |a Wakhlu, Anupam |e verfasserin |4 aut | |
| 700 | 1 | |a Selvam, Sumithra |e verfasserin |4 aut | |
| 700 | 1 | |a Khanna, Dinesh |e verfasserin |4 aut | |
| 700 | 1 | |a Shobha, Vineeta |e verfasserin |4 aut | |
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