Type I interferon pathway activation in connective tissue disease associated interstitial lung disease
By
Tobias M. Defesche
Thomas Koudstaal
Marjan A. Versnel
Odilia B.J. Corneth
Zana Brkic
June 11, 2026
Clinical Scorecard: Activation of the Type I Interferon Pathway in Interstitial Lung Disease Linked to Connective Tissue Disorders
At a Glance
Category Detail
Condition Key Mechanisms Type I interferon (IFN-I) signaling is implicated in the pathophysiology of CTD-ILD. Target Population Care Setting
Key Highlights
Up to 40% of ILDs are progressive, marked by lung function decline and increased symptoms. IFN-I activation correlates with pulmonary decline in CTD-ILD. Anifrolumab, an IFN-I blocking agent, is under clinical investigation for CTD-ILD. Guideline-Based Recommendations
Diagnosis
Progression of ILD in CTD is defined by ≥ 10% relative decline in forced vital capacity (FVC) or radiological progression. Management
Immunosuppression is standard therapy for ILD; antifibrotics like nintedanib are used in progressive fibrotic cases. Monitoring & Follow-up
Serial pulmonary function testing is essential to assess progression of ILD. Risks
Up to 40% of patients with CTD-ILD develop progressive pulmonary fibrosis (PPF). Patient & Prescribing Data
Patients with connective tissue diseases experiencing interstitial lung disease.
Many patients continue to progress despite treatment, highlighting the need for biomarkers and targeted interventions.
Clinical Best Practices
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