Utility of FDG PET/CT in Sjögren’s Syndrome and associated lymphomas; Lymphomagenesis

Document Type : Review article

Authors

1 India Department Of Nuclear Medicine All India Institute of Medical Sciences, Delhi, India

2 Assistant Professor,Department of Nuclear Medicine,, Institute of Liver and Biliary Sciences, New Delhi, Delhi, India.

Abstract

Primary Sjögren syndrome (SS) is an autoimmune disease affecting exocrine glands, with predisposition to development of lymphoma (lymphomagenesis). We report a case of Sjogren’s syndrome and discuss the role of FDG PET/CT in the primary diagnosis of lymphoma transformation in SS. Furthermore, we reviewed the literature regarding the utility of FDG PET/CT to assess systemic disease activity and also its role in the SS associated lymphoma with light into the new PET tracers that can be explored for these indications in the future.  Published data suggest promising role of FDG PET/CT in SS associated lymphomas, and demands larger studies for its establishment.

Keywords

Main Subjects


  1. Mariette X, Criswell LA. Primary Sjögren’s syndrome. N Engl J Med 2018; 378:931-9.
  2. Greenspan JS, Daniels TE, Talal N, Sylvester RA. The histopathology of Sjögren’s syndrome in labial salivary gland biopsies. Oral Surg. Oral Med. Oral Pathol. 1974; 37:217-229.
  3. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Primary Sjögren syndrome. BMJ. 2012; 344: e3821.
  4. Brito-Zerón P, Theander E, Baldini C, Seror R, Retamozo S, Quartuccio L, et al. Early diagnosis of primary Sjögren’s syndrome: EULAR-SS task force clinical recommendations. Expert Rev Clin Immunol. 2016; 12:137-56.
  5. Signore A, Anzola K L, Auletta S, Varani M, Petitti A, Pacilio M, et al. Current status of molecular imaging in inflammatory and autoimmune disorders. Curr Pharm Des. 2018; 24: 743-753.
  6. Garcia-Carrasco M, Ramos-Casals M, Rosas J, Pallares L, Calvo-Alen J, Cervera R, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). 2002; 81(4):270-80.
  7. Goules AV, Tzioufas AG. Lymphomagenesis in Sjögren’s syndrome: Predictive biomarkers towards precision medicine. Autoimmun Rev. 2019; 18: 137-143.
  8. Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T. The Diagnosis and Treatment of Sjögren's Syndrome. Dtsch Arztebl Int. 2017; 114(20):354-361.
  9. Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med. 1978; 89(6): 888-92.
  10. Pariente D, Anaya JM, Combe B, Jorgensen C, Emberger JM, Rossi JF, et al. Non-Hodgkin’s lymphoma associated with primary Sjögren’s syndrome. Eur J Med. 1992; 1(6):337-42.
  11. Tzioufas AG, Voulgarelis M. Update on Sjögren’s syndrome autoimmune epithelitis: from classification to increased neoplasias. Best Pract Res Clin Rheumatol. 2007; 21(6):989-1010.
  12. Routsias JG, Goules JD, Charalampakis G, Tzima S, Papageorgiou A, Voulgarelis M. Malignant lymphoma in primary Sjögren’s syndrome: An update on the pathogenesis and treatment. Semin. Arthritis Rheum. 2013; 43: 178-186.
  13. Royer B, Cazals-Hatem D, Sibilia J, Agbalika F, Cayuela JM, Soussi T,  et al. Lymphomas in patients with Sjogren’s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses. Blood .1997; 90: 766-775.
  14. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome: A multicenter, retrospective, clinical study by the European concerted action on Sjögren’s syndrome. Arthritis Rheum. 1999; 42: 1765-1772.
  15. Seror R, Bowman SJ, Brito-Zeron P, Theander E, Bootsma H, Tzioufas A, et al. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide. RMD Open. 2015; 1(1): e000022.
  16. Cohen C, Mekinian A, Uzunhan Y, Fauchais AL, Dhote R, Pop G, et al. 18F-fluorodeoxyglucose positron emission tomography/computer tomography as an objective tool for assessing disease activity in Sjögren's syndrome. Autoimmun Rev. 2013; 12(11):1109-14.
  17. Jadvar H, Bonyadlou S, Iagaru A, Colletti PM. FDG PET-CT demonstration of Sjogren’s Clin Nucl Med. 2005; 30: 698-699.
  18. Kumar P, Jaco MJ, Pandit AG, Shanmughanandan K, Jain A, Rajeev Ravina M. Miliary sarcoidosis with secondary Sjogren's syndrome. J Assoc Physicians India. 2013; 61(7):505-7.
  19. Sharma P, Chatterjee P. 18F-FDG PET/CT in multisystem Sjögren Syndrome. Clin Nucl Med. 2015; 40: e293-4.
  20. Serizawa I, Inubushi M, Kanegae K, Morita, K, Inoue T, Shiga T, et al. Lymphadenopathy due to amyloidosis secondary to Sjögren syndrome and systemic lupus erythematosus detected by F-18 FDG PET. Clin Nucl Med. 2007; 32: 881-882.
  21. Ma D, Lu H, Qu Y, Wang S, Ying Y, Xiao W. Primary Sjögren’s syndrome accompanied by pleural effusion: A case report and literature review. Int J Clin Exp Pathol. 2015; 8: 15322-15327.
  22. Nakamoto Y, Tatsumi M, Hammoud D, Cohade C, Osman MM, Wahl R L. Normal FDG distribution patterns in the head and neck: PET/CT evaluation. Radiology. 2005; 234: 879-885.
  23. Zincirkeser S, Sahin E, Halac M, Sager S. Standardized uptake values of normal organs on 18F-fluorodeoxyglucose positron emission tomography and computed tomography imaging. J Int Med Res. 2007; 35: 231-236.
  24. Carter KR, Kotlyarov E. Common causes of false positive F18 FDG PET/CT scans in oncology. Braz. Arch. Biol. Technol. 2007; 50: 29-35.
  25. Nicolau J, Sassaki KT. Metabolism of carbohydrate in the major salivary glands of rats. Arch Oral Biol. 1976; 21: 659-661.
  26. Weiler-Sagie M, Bushelev O, Epelbaum R, Dann EJ, Haim N, Avivi I, et al. 18F-FDG avidity in lymphoma readdressed: a study of 766 patients. J Nucl Med. 2010; 51:25-30.
  27. Ghielmini M, Vitolo U, Kimby E, Montoto S, Walewski J, Pfreundschuh M, et al. ESMO guidelines consensus conference on malignant lymphoma 2011 part 1: diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL) and chronic lymphocytic leukemia (CLL). Ann Oncol. 2013; 24:561-76.
  28. Dreyling M, Thieblemont C, Gallamini A, Arcaini L, Campo E, Hermine O, et al. ESMO consensus conferences: guidelines on malignant lymphoma. Part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma. Ann Oncol. 2013; 24: 857-77.
  29. Keraen J, Blanc E, Besson FL, Leguern V, Meyer C, Henry J, et al. Usefulness of 18F-Labeled Fluorodeoxyglucose-Positron Emission Tomography for the Diagnosis of Lymphoma in Primary Sjögren's Syndrome. Arthritis Rheumatol. 2019; 71(7):1147-1157.
  30. Alunno A, Leone MC, Giacomelli R, Gerli R, Carubbi F. Lymphoma and Lymphomagenesis in Primary Sjögren's Syndrome. Front Med (Lausanne). 2018; 5:102.
  31. Perry C, Herishanu Y, Metzer U, Bairey O, Ruchlemer R, Trejo L, et al. Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma. Eur J Haematol. 2007; 79: 205-209.
  32. Albano D, Durmo R, Treglia G, Giubbini R, Bertagna F. 18F-FDG PET/CT or PET Role in MALT Lymphoma: An Open Issue not Yet Solved-A Critical Review. Clin Lymphoma Myeloma Leuk. 2020; 20:137-146.
  33. Cytawa W, Kircher S, Schirbel A, Shirai T, Fukushima K, Buck AK, et al. Chemokine Receptor 4 Expression in Primary Sjögren’s Clin Nucl Med. 2018; 43: 835-836.
  34. Anzola LK, Rivera JN, Dierckx RA, Lauri C, Valabrega S, Galli F, et al. Value of Somatostatin Receptor Scintigraphy with 99mTc-HYNIC-TOC in Patients with Primary Sjögren Syndrome. J Clin Med. 2019; 8(6): 763.