Clinical Scorecard: Activation of the Type I Interferon Pathway in Interstitial Lung Disease Linked to Connective Tissue Disorders

At a Glance

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

Related Resources & Content

• Clinical guidelines for ILD management
• IFN-I signaling in lung disease