Interstitial lung disease in primary Sjögren's syndrome

Abstract

Interstitial lung disease is a common complication of Sjögren's syndrome that can occur at diagnosis or during follow-up. To detect it, complete pulmonary function studies should be performed, including spirometry, measurement of lung volumes, and DLCO, with the latter being the most sensitive parameter for detecting the presence of the disease. High-resolution computed tomography is essential for the study. Sixty percent of patients present a single tomographic pattern, with non-specific interstitial pneumonia being the most frequent pattern, followed by usual interstitial pneumonia pattern. Mortality is high, being higher in those with lower forced vital capacity, lower DLCO, and higher fibrosis score on chest computed tomography. Currently, there are two international guidelines for the treatment of pulmonary manifestations of Sjögren, but recommendations are based on low-quality scientific evidence. A stepwise approach is suggested, initially with glucocorticoids, then immunosuppressants, and in refractory or severe cases, considering other agents such as rituximab. The use of antifibrotic medication is recommended in patients who develop progressive pulmonary fibrosis as defined by current criteria. It is important to bear in mind that although non-specific interstitial pneumonia is considered a pattern where inflammation predominates, there may be progression to progressive pulmonary fibrosis in some cases. Lung transplantation and oxygen therapy may be options for selected patients. The relevance of an interdisciplinary team approach to achieve adequate diagnosis and treatment of patients is highlighted.