[Interstitial lung disease in connective tissue diseases]

Area of Science:

• Pulmonology
• Rheumatology
• Radiology

Background:

• Interstitial lung disease (ILD) is a serious complication of connective tissue diseases, significantly affecting patient survival.
• Diagnosis relies on clinical findings like lung crackles and imaging (high-resolution CT) showing basal opacities.

Purpose of the Study:

• To review the diagnosis, clinical presentation, and histopathology of interstitial lung disease in various connective tissue diseases.
• To summarize current knowledge on the treatment and outcomes of ILD in these conditions.

Main Methods:

• Review of diagnostic criteria including auscultation, high-resolution computed tomography (HRCT), and bronchoalveolar lavage.
• Analysis of ILD prevalence and histopathological patterns in systemic sclerosis, dermatopolymyositis, rheumatoid arthritis, and Sjögren syndrome.

Main Results:

• ILD affects 25% of systemic sclerosis, 7-30% of dermatopolymyositis (associated with anti-Jo-1 antibodies), and 5% of rheumatoid arthritis patients.
• Nonspecific interstitial pneumonia is common in systemic sclerosis/dermatopolymyositis; usual interstitial pneumonia is frequent in rheumatoid arthritis.
• Cyclophosphamide showed modest short-term benefit in systemic sclerosis ILD, but efficacy waned at 2 years.

Conclusions:

• ILD is a significant comorbidity in connective tissue diseases with distinct patterns.
• Further research is needed to establish effective and sustained treatments for ILD in these patient populations.