The EUSTAR database collects, among other things, information on the development of interstitial lung disease in patients with systemic sclerosis. Characteristics of the development of interstitial lung disease among systemic sclerosis patients have been published in the Annals of Rheumatic Diseases.

A total of 826 patient records were analyzed. Within one year, 27% of interstitial lung disease developed as expressed by a decrease in forced vital capacity (FVC) of at least 5%. In the subgroup of patients who studied for an average period of more than 5 years, 67% had a progression of ILD. However, the majority (85%) showed a slow decrease in forced vital capacity, while 8% showed rapid disease progression. In the remaining 33%, no long-term decrease in the forced vital capacity value was observed. Decrease in forced vital capacity over 5 years was associated with male type, elevated Rudnan’s modified skin lesion score, and presence of reflux or dysphagia symptoms.

Interstitial lung disease in patients with systemic sclerosis is not uniform, and most patients have a progressive deterioration in lung function, and in every third patient the lung function does not change significantly.

Prepared by: Lik. med. Mikołaj Kamiski

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