Lung Involvement in Connective Tissue Disease: Diagnosis and Management

Dear Colleagues,

Interstitial lung disease (ILD) is a frequent complication of connective tissue disease (CTD) with a significant impact on morbidity and mortality. Although ILD is reported in all CTDs, there is substantial variability in the prevalence and pattern of ILD subtypes.

The complex etiology of CTD-ILD includes genetic risks, epigenetic changes, and dysregulated immunity, which interact. CTD-ILD exhibits a broad spectrum of clinical manifestations; the disease course features remissions and relapses. It can range from stability or slow progression over several years to rapid deterioration.

Significant work has been undertaken to better characterise ILD prevalence, risk factors, and frequency of specific CT patterns and establish treatment guidelines. Still, rheumatologists are faced with several challenges: (a) the precise mechanisms that drive CTD-ILD remain unclear, (b) it is easy to miss ILD diagnosis as symptoms are frequently masked by the rheumatic condition, (c) except for systemic sclerosis, there is no standard protocol for screening and assessing disease progression, there are no effective methods or biomarkers to determine whether the patient’s deterioration is due to the progression of ILD or other reasons, such as infection or drug-induced causes; (d) CTD-ILD is difficult to treat, requiring vigorous effort and a multidisciplinary team.

Dr. Laura Groșeanu
Guest Editor

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