Clinical rheumatology
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Clinical rheumatology · Feb 2021
Characteristics of primary Sjögren's syndrome related lymphocytic interstitial pneumonia.
This paper is aimed at investigating the clinical characteristics of primary Sjogren's syndrome (pSS) with lymphocytic interstitial pneumonia (LIP). ⋯ To the patients of pSS-LIP, female were more common than male, and the onset of LIP was usually more insidious. Hyperglobulinemia and anti-SSA antibody were more prominent in patients with pSS-LIP. Pulmonary function showed the higher rate of obstructive ventilation dysfunction and the lower rate of diffusion dysfunction. The appearance of ground-glass opacities in pSS-LIP patients suggests that the infiltration of inflammatory cells increases, which may cause airway compression, the expansion of terminal bronchioles, and the formation of cysts. The more ground-glass opacities appear earlier, and the more appearance of new cysts later. Therapy with glucocorticoid may be effective on the ground-glass opacity during acute stage, and therapy with cyclophosphamide may be effective on the cysts during chronic stage. The heavier ground-glass opacity is at baseline, the more likely it will recur during maintenance treatment. So follow-up closely is needed. Key Points • It is the first clinical study with more cases of patients with pSS-LIP. • Female and hyperglobulinemia and anti-SSA antibody were more prominent in patients with pSS-LIP. • Pulmonary function showed the higher rate of obstructive ventilation dysfunction and the lower rate of diffusion dysfunction. • Therapy with glucocorticoid may be effective on the ground-glass opacity during acute stage, and therapy with cyclophosphamide may be effective on the cysts during chronic stage.