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

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.

Sjögren syndrome associated with protein-losing enteropathy: case-based review.

The association between Sjögren's syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ's pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient's ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9-3.4 g/dL which increased to 2.8-4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient's deterioration, complications, and reducing morbidity and potential mortality.