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Interstitial lung diseases in rheumatological diseases
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DOI：10.1055/a-2477-3126.png)
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Abstract
En 中文
The prevalence of interstitial lung disease (ILD) in the general population is low, at approximately 0.07%. However, the presence of an underlying rheumatologic disease significantly increases the risk. ILD associated with rheumatic disorders is linked to substantial morbidity and mortality, underscoring the importance of early recognition and prompt therapeutic intervention. Clinical presentation is highly heterogeneous, ranging from asymptomatic or subclinical disease to acute hypoxemic respiratory deterioration, which often delays diagnosis. Typical symptoms include exertional dyspnea and chronic non-productive cough, while advanced stages may manifest with digital clubbing, cachexia, and cyanosis. Pulmonary function testing, particularly body plethysmography and measurement of diffusing capacity for carbon monoxide (DLCO), together with high-resolution computed tomography (HRCT), represent central diagnostic tools. Characteristic radiologic patterns such as usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) allow for disease stratification and prognostic assessment. Multidisciplinary discussion within an ILD board involving rheumatologists, pulmonologists, and radiologists is essential for accurate classification and individualized treatment planning. High-dose glucocorticoids are reserved for acute or subacute inflammatory presentations, whereas long-term steroid therapy should be minimized due to adverse effects. Current management strategies combine immunomodulatory agents, including conventional and biologic DMARDs, with antifibrotic therapies in progressive fibrosing phenotypes. Close interdisciplinary follow-up is crucial to detect progression early and optimize patient outcomes.
Keywords:
HR-CT pattern
lung function
progressive fibrosing ILD
antifibrotic therapy
immunomodulation
HR-CT pattern
lung function
progressive fibrosing ILD
antifibrotic therapy
immunomodulation
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