Thoracic Involvement in Systemic Autoimmune Rheumatic Diseases: Pathogenesis and Management

Area of Science:

• Rheumatology
• Pulmonology
• Immunology

Background:

• Thoracic involvement significantly impacts morbidity and mortality in autoimmune rheumatic diseases (ARDs).
• Interstitial lung disease (ILD) is the most frequent pulmonary complication, especially in systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIMs), and rheumatoid arthritis (RA).
• Other manifestations include pulmonary arterial hypertension (PAH), airway disease, and pleural involvement, varying by ARD type.

Purpose of the Study:

• To review and critically discuss current knowledge on thoracic involvement in ARDs.
• To emphasize disease pathogenesis and management strategies for thoracic complications in ARDs.
• To highlight challenges and future research directions in managing ARDs with thoracic manifestations.

Main Methods:

• Comprehensive literature review of thoracic manifestations in ARDs.
• Critical discussion of disease pathogenesis, clinical presentation, and management.
• Analysis of current therapeutic approaches, focusing on immunosuppression for ARDs-ILD.

Main Results:

• Thoracic involvement presents diversely across ARDs, with ILD being most common.
• Pulmonary arterial hypertension (PAH), airway disease, and pleural disease are other significant manifestations.
• Immunosuppression is a key therapy for ARDs-ILD, but requires careful patient selection based on disease activity and progression risk.

Conclusions:

• Optimizing patient management necessitates thorough, multidisciplinary assessment of disease activity and impairment.
• Managing thoracic involvement, particularly ILD, is challenging due to heterogeneous pathogenesis and limited clinical trial data.
• Further research into pathogenesis is crucial for developing more effective therapies for thoracic complications in ARDs.