open access

Vol 5, No 4 (2019)
Case report
Published online: 2020-02-05
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Primary Sjögren syndrome complicated by vasculitis and non-Hodgkin lymphoma

Mateusz Moskal1, Jolanta Osieleniec1, Piotr Krawiec1, Małgorzata Pukal1, Bogdan Batko1
DOI: 10.5603/R.2019.0021
·
Forum Reumatol 2019;5(4):197-199.
Affiliations
  1. KCM, Kopernika 32, 31-501 Krakow, Poland

open access

Vol 5, No 4 (2019)
Case report
Published online: 2020-02-05

Abstract

Sjögren syndrome (SS) is a chronic inflammatory disease of autoimmune origin. During its course, lymphocytic infiltrates within the exocrine glands (primarily lacrimal and salivary glands) cause damage to these glands and impairment of their functions, leading to, among others, symptoms of dryness typical for SS. In approx. 70–80% of patients, glandular lesions
may be accompanied by involvement of, and subsequent damage to, other organs and systems. Some of the most serious, life-threatening organ complications include vasculitis, nervous system involvement and development of non-Hodgkin lymphomas ( NHLs). This article describes a case of a 64-year-old patient with primary Sjögren syndrome, in whom the course of the disease led to deterioration of health with symptoms of vasculitis and peripheral nervous system involvement, eventually ending in diagnosis of a non-Hodgkin lymphoma (NHL). The authors point out the significant heterogeneity of symptoms in the course of SS and emphasize the need to identify and closely monitor patients who are at high risk of developing a lymphoproliferative process.

Forum Reumatol. 2019, tom 5, nr 4: 197–199

Abstract

Sjögren syndrome (SS) is a chronic inflammatory disease of autoimmune origin. During its course, lymphocytic infiltrates within the exocrine glands (primarily lacrimal and salivary glands) cause damage to these glands and impairment of their functions, leading to, among others, symptoms of dryness typical for SS. In approx. 70–80% of patients, glandular lesions
may be accompanied by involvement of, and subsequent damage to, other organs and systems. Some of the most serious, life-threatening organ complications include vasculitis, nervous system involvement and development of non-Hodgkin lymphomas ( NHLs). This article describes a case of a 64-year-old patient with primary Sjögren syndrome, in whom the course of the disease led to deterioration of health with symptoms of vasculitis and peripheral nervous system involvement, eventually ending in diagnosis of a non-Hodgkin lymphoma (NHL). The authors point out the significant heterogeneity of symptoms in the course of SS and emphasize the need to identify and closely monitor patients who are at high risk of developing a lymphoproliferative process.

Forum Reumatol. 2019, tom 5, nr 4: 197–199

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Keywords

Sjögren syndrome; lymphoma; cryoglobulins; vasculitis

About this article
Title

Primary Sjögren syndrome complicated by vasculitis and non-Hodgkin lymphoma

Journal

Rheumatology Forum

Issue

Vol 5, No 4 (2019)

Article type

Case report

Pages

197-199

Published online

2020-02-05

Page views

482

Article views/downloads

716

DOI

10.5603/R.2019.0021

Bibliographic record

Forum Reumatol 2019;5(4):197-199.

Keywords

Sjögren syndrome
lymphoma
cryoglobulins
vasculitis

Authors

Mateusz Moskal
Jolanta Osieleniec
Piotr Krawiec
Małgorzata Pukal
Bogdan Batko

References (15)
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  13. Brito-Zerón P, Kostov B, Solans R, et al. SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI). Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis. 2016; 75(2): 348–355.
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