Clinical Report: Low DLCO/SB Linked to Faster SSc Progression
Overview
Revise to specify the study design and clarify the association between DLCO/SB and disease progression.
Background
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis and vasculopathy, leading to significant morbidity and mortality. Early identification of disease progression is crucial for timely intervention and management. Understanding the role of pulmonary function metrics, such as DLCO/SB, can aid in predicting disease trajectory in SSc patients.
Data Highlights
| DLCO/SB Category | Progression Rate | Time to Transition (Years) |
|---|---|---|
| Below 70% | 33% | 7.7 |
| 70% or Higher | 8% | 10.2 |
Key Findings
- 12% of patients progressed to definite SSc over a median follow-up of 5 years.
- Patients with DLCO/SB below 70% had a significantly higher progression rate (33% vs 8%).
- All patients who progressed were positive for SSc-specific antibodies.
- Baseline DLCO/SB below 70% is suggested as a potential marker of disease progression.
- 24% of antibody-positive patients experienced a decline in DLCO/SB of at least 15% compared to 6% of antibody-negative patients.
- Declines in forced vital capacity of at least 10% were more common in antibody-positive patients (88% vs 64%).
Clinical Implications
Highlight the necessity for further research on DLCO/SB as a progression predictor.
Conclusion
The findings underscore the importance of DLCO/SB as a potential indicator of disease progression in systemic sclerosis, warranting further investigation and monitoring in clinical practice.
References
- American College of Rheumatology, ACR Open Rheumatology, 2023 -- Low DLCO/SB Linked to Faster SSc Progression
- Clinical Rheumatology, 2023 -- Interstitial Lung Disease Associated with Systemic Sclerosis: A Retrospective Cross-Sectional Analysis from Taiwan
- Clinical Rheumatology, 2019 -- Interstitial Lung Disease with Progressive Fibrosis Linked to Systemic Autoimmune Disorders
- Bone Marrow Transplantation — Evaluation of Cardiopulmonary Function in Systemic Sclerosis Patients Prior to Hematopoietic Stem Cell Transplantation: Guidelines from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Group and Associated Collaborators
- EUSTAR/VEDOSS Analysis on DLCO in SSc
- 2025 ERS/EULAR Guidelines for CTD-ILD
- Effect of Nintedanib on Progression of Systemic Sclerosis‐Associated Interstitial Lung Disease Over 100 Weeks: Data From a Randomized Controlled Trial - PMC
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