medigraphic.com
Órgano Oficial del Hospital Angeles Health System

2016, Number 2

<< Back Next >>

Acta Med 2016; 14 (2)

Sjögren syndrome. Review with an illustrative case report

Arellano AG, Magaña RJ, Gutiérrez VJL, Domínguez CLG

Language: Spanish
References: 72
Page: 90-100
PDF size: 244.37 Kb.


ABSTRACT

Background: Sjögren syndrome is a chronic, autoimmune, inflammatory diseases characterized by diminished lacrimal and salivary gland function, although the clinical manifestations of SS include both exocrine gland involvement and extraglandular disease features. Clinical case: A 37-year-old female with xerosis, xerophthalmia, xerostomia, vulvovaginal dryness, fatigue and mild sensory neuropathy since 8 months ago. Physical examination: bilateral parotid gland enlargement. She was hospitalized and presented positive Schirmer test, positive salivary scintigraphy, and magnetic resonance with features typical of Sjögren disease; laboratory showed IgG type 1, 19350.0 mg/L; antibodies: anti SSA/Ro, 179.66; AC SSB/La, 114.01, and antinuclear antibodies with speckled pattern reaction 1:5120. After diagnosis, hydroxychloroquine and treatments for dry eye and dry mouth were prescribed. Conclusion: A patient with primary Sjögren’s syndrome and mild extraglandular organ involvement, like sensory neuropathy, without important systemic repercussion.


REFERENCES

  1. Malladi AS, Sack KE, Shiboski SC et al. Primary Sjögren’s syndrome as a systemic disease: a study of participants enrolled in an international Sjögren’s syndrome registry. Arthritis Care Res (Hoboken). 2012; 64 (6): 911-918.

  2. Lockshin MD, Levine AB, Erkan D. Patients with overlap autoimmune disease differ from those with “pure” disease. Lupus Sci Med. 2015; 2:e000084.

  3. Bournia VK, Vlachoyiannopoulos PG. Subgroups of Sjögren syndrome patients according to serological profiles. J Autoimmun. 2012; 39 (1-2): 15-26.

  4. Helmick CG, Felson DT, Lawrence RC et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008; 58 (1): 15-25.

  5. Fox RI. Sjögren’s syndrome. Lancet. 2005; 366 (9482): 321-331.

  6. Gottenberg JE, Busson M, Loiseau P, Cohen-Solal J, Lepage V, Charron D et al. In primary Sjögren’s syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response. Arthritis Rheum. 2003; 48 (8): 2240-2245.

  7. Shiboski CH, Shiboski SC; ACR-EULAR Sjogren’s Syndrome Classification Criteria Working Group. Proposed ACR-EULAR classification criteria for Sjogren’s syndrome: development and validation. Program and abstracts from the 13th International Symposium on Sjogren’s Syndrome; Bergen, Norway. Abstract S1.1. May 19-22, 2015.

  8. Shiboski SC, Shiboski CH, Criswell L et al. American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012; 64 (4): 475-487.

  9. Vissink A, Bootsma H, Spijkervet FK, Hu S, Wong DT, Kallenberg CG. Current and future challenges in primary Sjogren’s syndrome. Curr Pharm Biotechnol. 2012; 13 (10): 2026-2045.

  10. Rasmussen A, Kelly JA, Ice J et al. Characterization of early and progressive autoimmunity in Sjögren’s syndrome: the incomplete Sjogren’s syndrome model. Program and abstracts from the 13th International Symposium on Sjogren’s Syndrome; Bergen, Norway. Abstract S2.10. 2015, pp. 19-22.

  11. Kittridge A, Routhouska SB, Korman NJ. Dermatologic manifestations of Sjögren syndrome. J Cutan Med Surg. 2011; 15 (1): 8-14.

  12. Bernacchi E, Bianchi B, Amato L et al. Xerosis in primary Sjögren syndrome: immunohistochemical and functional investigations. J Dermatol Sci. 2005; 39 (1): 53-55.

  13. Bernacchi E, Amato L, Parodi A et al. Sjögren’s syndrome: a retrospective review of the cutaneous features of 93 patients by the Italian Group of Immunodermatology. Clin Exp Rheumatol. 2004; 22 (1): 55-62.

  14. García-Carrasco M, Sisó A, Ramos-Casals M et al. Raynaud’s phenomenon in primary Sjögren’s syndrome. Prevalence and clinical characteristics in a series of 320 patients. J Rheumatol. 2002; 29 (4): 726-730.

  15. Guellec D, Cornec-Le Gall E, Groh M et al. ANCA-associated vasculitis in patients with primary Sjögren’s syndrome: detailed analysis of 7 new cases and systematic literature review. Autoimmun Rev. 2015; 14 (8): 742-750.

  16. Ramos-Casals M, Anaya JM, García-Carrasco M et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore). 2004; 83 (2): 96-106.

  17. Chapnick SL, Merkel PA. Skin ulcers in a patient with Sjögren’s syndrome. Arthritis Care Res (Hoboken). 2010; 62: 1040-1043.

  18. Brito-Zerón P, Retamozo S, Akasbi M et al. Annular erythema in primary Sjögren’s syndrome: description of 43 non-Asian cases. Lupus. 2014; 23: 166-167.

  19. Roguedas AM, Misery L, Sassolas B et al. Cutaneous manifestations of primary Sjögren’s syndrome are underestimated. Clin Exp Rheumatol. 2004; 22 (5): 632-636.

  20. Mohammed K, Pope J, Le Riche N et al. Association of severe inflammatory polyarthritis in primary Sjögren’s syndrome: clinical, serologic, and HLA analysis. J Rheumatol. 2009; 36 (9): 1937-1942.

  21. Amezcua-Guerra LM, Hofmann F, Vargas A et al. Joint involvement in primary Sjögren’s syndrome: an ultrasound “target area approach to arthritis”. Biomed Res Int. 2013; 2013:640265. doi: 10.1155/2013/640265.

  22. Lindvall B, Bengtsson A, Ernerudh J, Eriksson P. Subclinical myositis is common in primary Sjögren’s syndrome and is not related to muscle pain. J Rheumatol. 2002; 29 (4): 717-725.

  23. Dobloug GC, Antal EA, Sveberg L et al. High prevalence of inclusion body myositis in Norway; a population-based clinical epidemiology study. Eur J Neurol. 2015; 22 (4): 672-e41.

  24. Dass S, Bowman SJ, Vital EM et al. Reduction of fatigue in Sjögren syndrome with rituximab: results of a randomised, double-blind, placebo-controlled pilot study. Ann Rheum Dis. 2008; 67 (11): 1541-1544.

  25. Lu MC, Yin WY, Tsai TY et al. Increased risk of primary Sjögren’s syndrome in female patients with thyroid disorders: a longitudinal population-based study in Taiwan. PLoS One. 2013; 8: e77210.

  26. Kreider M, Highland K. Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 2014; 35: 255-264.

  27. Hatron PY, Tillie-Leblond I, Launay D et al. Pulmonary manifestations of Sjögren’s syndrome. Presse Med. 2011; 40: e49-64.

  28. Gravani F, Papadaki I, Antypa E et al. Subclinical atherosclerosis and impaired bone health in patients with primary Sjogren’s syndrome: prevalence, clinical and laboratory associations. Arthritis Res Ther. 2015; 17: 99.

  29. Bartoloni E, Baldini C, Schillaci G et al. Cardiovascular disease risk burden in primary Sjögren’s syndrome: results of a population-based multicentre cohort study. J Intern Med. 2015; 278: 185-192.

  30. Juarez M, Toms TE, de Pablo P et al. Cardiovascular risk factors in women with primary Sjögren’s syndrome: United Kingdom primary Sjögren’s syndrome registry results. Arthritis Care Res (Hoboken). 2014; 66: 757-764.

  31. Ebert EC. Gastrointestinal and hepatic manifestations of Sjogren syndrome. J Clin Gastroenterol. 2012; 46 (1): 25-30.

  32. Imrich R, Alevizos I, Bebris L et al. Predominant glandular cholinergic dysautonomia in patients with primary Sjögren’s syndrome. Arthritis Rheumatol. 2015; 67: 1345-1352.

  33. Montaño-Loza AJ, Crispín-Acuña JC, Remes-Troche JM, Uribe M. Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren’s syndrome. Ann Hepatol. 2007; 6 (3): 150-155.

  34. Afzelius P, Fallentin EM, Larsen S et al. Pancreatic function and morphology in Sjögren’s syndrome. Scand J Gastroenterol. 2010; 45: 752-758.

  35. Matsuda M, Hamano H, Yoshida T et al. Seronegative Sjögren syndrome with asymptomatic autoimmune sclerosing pancreatitis. Clin Rheumatol. 2007; 26: 117-119.

  36. Lee KL, Chen MY, Yeh JH et al. Lower urinary tract symptoms in female patients with rheumatoid arthritis. Scand J Rheumatol. 2006; 35: 96-101.

  37. Lehrer S, Bogursky E, Yemini M et al. Gynecologic manifestations of Sjögren’s syndrome. Am J Obstet Gynecol. 1994; 170 (3): 835-838.

  38. Mori K, Iijima M, Koike H et al. The wide spectrum of clinical manifestations in Sjögren’s syndrome-associated neuropathy. Brain. 2005; 128 (Pt 11): 2518-2534.

  39. Delalande S, de Seze J, Fauchais AL et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004; 83 (5): 280-291.

  40. Sène D, Jallouli M, Lefaucheur JP et al. Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine (Baltimore). 2011; 90 (2): 133-138.

  41. Kawagashira Y, Koike H, Fujioka Y et al. Differential, size-dependent sensory neuron involvement in the painful and ataxic forms of primary Sjögren’s syndrome-associated neuropathy. J Neurol Sci. 2012; 319 (1-2): 139-146.

  42. Brito-Zerón P, Akasbi M, Bosch X et al. Classification and characterisation of peripheral neuropathies in 102 patients with primary Sjögren’s syndrome. Clin Exp Rheumatol. 2013; 31 (1): 103-110.

  43. Birnbaum J. Peripheral nervous system manifestations of Sjögren syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies. Neurologist. 2010; 16 (5): 287-297.

  44. Scofield RH. Vasculitis in Sjögren’s syndrome. Curr Rheumatol Rep. 2011; 13 (6): 482-488.

  45. Massara A, Bonazza S, Castellino G et al. Central nervous system involvement in Sjögren’s syndrome: unusual, but not unremarkable —clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 2010; 49: 1540-1549.

  46. Theodoridou A, Settas L. Demyelination in rheumatic diseases. J Neurol Neurosurg Psychiatry. 2006; 77 (3): 290-295.

  47. Westhoff G, Dörner T, Zink A. Fatigue and depression predict physician visits and work disability in women with primary Sjögren’s syndrome: results from a cohort study. Rheumatology (Oxford). 2012; 51 (2): 262-269.

  48. Papageorgiou A, Ziogas DC, Mavragani CP et al. Predicting the outcome of Sjogren’s syndrome-associated non-Hodgkin’s lymphoma patients. PLoS One. 2015; 10 (2): e0116189.

  49. Nocturne G, Mariette X. Sjögren syndrome-associated lymphomas: an update on pathogenesis and management. Br J Haematol. 2015; 168 (3): 317-327.

  50. Haga HJ, Gjesdal CG, Koksvik HS et al. Pregnancy outcome in patients with primary Sjögren’s syndrome. A case-control study. J Rheumatol. 2005; 32 (9): 1734-1736.

  51. Ramos-Casals M, Solans R, Rosas J et al. Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008; 87 (4): 210-219.

  52. Ter Borg EJ, Risselada AP, Kelder JC. Relation of systemic autoantibodies to the number of extraglandular manifestations in primary Sjögren’s Syndrome: a retrospective analysis of 65 patients in the Netherlands. Semin Arthritis Rheum. 2011; 40 (6): 547-551.

  53. Menéndez A, Gómez J, Escanlar E et al. Clinical associations of anti-SSA/Ro60 and anti-Ro52/TRIM21 antibodies: diagnostic utility of their separate detection. Autoimmunity. 2013; 46 (1): 32-39.

  54. Kim SM, Park E, Lee JH et al. The clinical significance of anti-cyclic citrullinated peptide antibody in primary Sjögren syndrome. Rheumatol Int. 2012; 32 (12): 3963-3967.

  55. Hu S, Zhao F, Wang Q, Chen WX. The accuracy of the anti-mitochondrial antibody and the M2 subtype test for diagnosis of primary biliary cirrhosis: a meta-analysis. Clin Chem Lab Med. 2014; 52 (11): 1533-1542.

  56. Vinagre F, Santos MJ, Prata A, da Silva JC et al. Assessment of salivary gland function in Sjögren’s syndrome: the role of salivary gland scintigraphy. Autoimmunity Rev. 2009; 8 (8): 672-676.

  57. Guinales DJ, Martorell MV. Síndrome de Sjögren: criterios diagnósticos mediante resonancia magnética. Rev Esp Cir Oral Maxilofac. 2012; 34: 188-191.

  58. Becerra-UR, Pachuca-GD, Alva LL, Menédez ZS et al. Imagen del síndrome de Sjögren en las glándulas salivales. Rev Invest Med Sur Mex. 2014; 21: 31-35.

  59. Shabnum A, Brown J, Ngu R, Daniels T et al. Correlation between ultrasound imaging of major salivary glands and histopathological findings of labial gland biopsy samples in Sjogren’s syndrome. Lancet. 2013; 381: S19-27.

  60. Risselada AP, Kruize AA, Goldschmeding R et al. The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren’s syndrome. Ann Rheum Dis. 2014; 73 (8): 1537-1540.

  61. Foulks GN, Forstot SL, Donshik PC et al. Clinical guidelines for management of dry eye associated with Sjögren disease. Ocul Surf. 2015; 13 (2): 118-122.

  62. Akpek EK, Mathews P, Hahn S et al. Ocular and systemic morbidity in a longitudinal cohort of Sjögren’s syndrome. Ophthalmology. 2015; 122 (1): 56-61.

  63. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A et al. Topical and systemic medications for the treatment of primary Sjögren’s syndrome. Nat Rev Rheumatol. 2012; 8 (7): 399-411.

  64. Jaenen N, Baudouin C, Pouliquen P et al. Ocular symptoms and signs with preserved and preservative-free glaucoma medications. Eur J Ophthalmol. 2007; 17 (3): 341-349.

  65. Roberts CW, Carniglia PE, Brazzo BG. Comparison of topical cyclosporine, punctal occlusion, and a combination for the treatment of dry eye. Cornea. 2007; 26 (7): 805-809.

  66. Wu AJ. Optimizing dry mouth treatment for individuals with Sjögren’s syndrome. Rheum Dis Clin North Am. 2008; 34 (4): 1001-1010.

  67. Katelaris CH. Pilocarpine for dry mouth and dry eye in Sjögren’s syndrome. Curr Allergy Asthma Rep. 2005; 5 (4): 321-326.

  68. Ramos-Casals M, Tzioufas AG, Stone JH et al. Treatment of primary Sjögren syndrome: a systematic review. JAMA. 2010; 304 (4): 452-460.

  69. Reksten TR, Brokstad KA, Jonsson R et al. Implications of long-term medication of oral steroids and antimalarial drugs in primary Sjögren’s syndrome. Int Immunopharmacol. 2011; 11 (12): 2125-2129.

  70. Meijer JM, Meiners PM, Vissink A et al. Effectiveness of rituximab treatment in primary Sjögren’s syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2010; 62 (4): 960-968.

  71. Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 2000; 29 (5): 296-304.

  72. Voulgarelis M, Tzioufas AG, Moutsopoulos HM. Mortality in Sjögren’s syndrome. Clin Exp Rheumatol. 2008; 26 (5 Suppl 51): S66-71.




2020     |     www.medigraphic.com