Severe extra-glandular involvement and pleural effusions complicating primary Sjogren's syndrome: a case report.

Autor: Abou Ziki MD; Department of Medicine, Yale University School of Medicine, 300 George St, New Haven, CT, 06520, USA. maen.abouziki@yale.edu., Taoutel R; Department of Medicine, Lankenau Medical Center, Wynnewood, PA, 19096, USA., Hong JC; Department of Medicine, Yale University School of Medicine, 300 George St, New Haven, CT, 06520, USA., Podell DN; Department of Medicine, Yale University School of Medicine, 300 George St, New Haven, CT, 06520, USA.
Jazyk: angličtina
Zdroj: Journal of medical case reports [J Med Case Rep] 2022 Oct 18; Vol. 16 (1), pp. 374. Date of Electronic Publication: 2022 Oct 18.
DOI: 10.1186/s13256-022-03557-7
Abstrakt: Background: Sjogren's syndrome, an autoimmune disease of the exocrine glands, results in keratoconjunctivitis sicca, xerostomia, and dental caries. It is often overlooked, considered by clinicians to be a benign disease. However, it can cause life-threatening extra-glandular complications that affect multiple organ systems.
Case Presentation: Here we present a 78-year-old Caucasian woman with a history of primary Sjogren's syndrome (pSS) whose symptoms of keratoconjunctivitis sicca were managed managed conservatively. She was evaluated for sub-acute shortness of breath. Imaging showed severe bronchiectasis with features of lymphocytic interstitial pneumonia. She also had exudative bilateral pleural effusions and skin ulcers, likely vasculitic in origin. The workup was significant for anti-Ro antibody, pancytopenia, hypocomplementia, cryoglobulinemia and monoclonal gammopathy, all of which reflect disease severity. Although there was no evidence of malignancy, she developed B-cell non-Hodgkin lymphoma during follow-up.
Conclusions: Primary Sjogren's syndrome can result in severe multi-organ disease. Pleural effusions are a rare complication of pSS, with only ten cases reported in the literature over the last 30 years, and tend to respond well to steroids. Prognostic biomarkers for disease severity include hypocomplementia, cryoglobulinemia, monoclonal gammopathy, and hypergammaglobulinemia. In this report we review the literature and the management of the disease.
(© 2022. The Author(s).)
Databáze: MEDLINE
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