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.

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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References

  1. Alunno A, Leone MC, Giacomelli R, et al. Lymphoma and Lymphomagenesis in Primary Sjögren's Syndrome. Front Med (Lausanne). 2018; 5: 102.
  2. Johnsen SJ, Brun JG, Gøransson LG, et al. Risk of non-Hodgkin's lymphoma in primary Sjögren's syndrome: a population-based study. Arthritis Care Res (Hoboken). 2013; 65(5): 816–821.
  3. Rischmueller M, Tieu J, Lester S. Primary Sjögren's syndrome. Best Pract Res Clin Rheumatol. 2016; 30(1): 189–220.
  4. Kaplan JG, Rosenberg R, Reinitz E, et al. Invited review: peripheral neuropathy in Sjogren's syndrome. Muscle Nerve. 1990; 13(7): 570–579.
  5. Domżalska M, Wiglusz M, Chylińska M, et al. Zajęcie układu nerwowego w zespole Sjögrena. Neurological involvement in Sjögren’s syndrome . Neurol. Prakt. 2015; 15(6): 6–15.
  6. Ejma M, Madetko N, Waliszewska-Prosół M. Neurologiczne powikłania zespołu Sjögrena. Varia Medica. 2018; 2(1): 35–45.
  7. Baldini C, Pepe P, Quartuccio L, et al. Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford). 2014; 53(5): 839–844.
  8. Quartuccio L, Isola M, Baldini C, et al. Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome: results of a large multicentre study. Scand J Rheumatol. 2015; 44(1): 36–41.
  9. Martel C, Gondran G, Launay D, et al. Active immunological profile is associated with systemic Sjögren's syndrome. J Clin Immunol. 2011; 31(5): 840–847.
  10. Sebastian A, Misterska-Skóra M, Wiland P. Cryoglobulinemia in the course of a primary Sjögren’s syndrome. Forum Reumatologiczne. 2017; 3(1): 35–38.
  11. Seror R, Bowman SJ, Brito-Zeron P, et al. EULAR Sjogren's syndrome disease activity index (ESSDAI): a user guide. RMD Open. 2015; 1(1): e000022–e000022.
  12. Theander E, Vasaitis L, Baecklund E, et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren's syndrome. Ann Rheum Dis. 2011; 70(8): 1363–1368.
  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.
  14. Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use. Medicine (Baltimore). 2016; 95(25): e3766.
  15. De Vita S, Gandolfo S. Predicting lymphoma development in patients with Sjögren's syndrome. Expert Rev Clin Immunol. 2019; 15(9): 929–938.