Title:Lymphocytic Interstitial Pneumonia Associated with Sjögren Syndrome: Case-Based Review
Volume: 19
Issue: 1
Author(s): Caio Marco Gonçalves, Luísa Rodrigues Cordeiro, Carlos Augusto Ferreira de Andrade and Jozélio Freire de Carvalho*
Affiliation:
- Department of Medicine, Institute for Health Sciences, Federal University of Bahia, Salvador, Bahia, Brazil
Keywords:
Sjögren syndrome, pneumonitis, lymphocytic interstitial pneumonia, lungs, bronchiolitis obliterans, pulmonary fibrosis.
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.
