Lymphocytic Interstitial Pneumonia Associated with Sjögren Syndrome: Case-Based Review

Abstract

Background: Few studies have described lymphocytic interstitial pneumonia (LIP) in Sjögren's syndrome (SS). We aimed to analyze the clinical, therapeutic, and outcome of patients with SS and LIP.

Methods: We searched for articles in PubMed/MEDLINE, LILACS, SciELO, and Cochrane from 1966 to 2020, in English, Spanish, French, Chinese, and Japanese literature had an English summary about LIP and SS. The keywords were "Sjögren syndrome" and "Lymphocytic interstitial pneumonia." Additionally, we report a patient with SS and LIP.

Results: 27 patients with SS and LIP were found. The age range ranged from 14 to 73 years old, with only 3 male patients, with a predominance of LIP cases in patients with primary SS (22/27). In the following case, the LIP preceded SS by 2 years; in the other 26 patients, SS preceded it. The majority presented dyspnea, mainly on exertion, followed by a dry cough. Lung biopsy was performed in 10 studies. Therapy varied from the use of clinical observation, corticosteroids alone, or associated with immunosuppressants. Most studies have shown improvement or stabilization of the pulmonary condition after therapy (13/16 studies).

Conclusion: This article reviews cases of lymphocytic interstitial pneumonia associated with Sjögren syndrome and shows a good outcome with adequate treatment. It emphasizes that early LIP diagnosis in patients with Sjogren Syndrome may be determined using lung computed tomography.

Keywords: Sjögren syndrome; bronchiolitis obliterans; lungs; lymphocytic interstitial pneumonia; pneumonitis; pulmonary fibrosis.