Clinical manifestations of Sjögren's syndrome: Exocrine gland disease
- Alan N Baer, MD, FACP
Alan N Baer, MD, FACP
- Associate Professor of Medicine
- Director, Jerome L. Greene Sjogren’s Syndrome Center
- Johns Hopkins University School of Medicine
Sjögren’s syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function. SS may occur alone (primary form) or in association with a second well-defined rheumatic disease (secondary form). The most common disease associated with secondary SS is rheumatoid arthritis.
In primary or secondary SS, decreased exocrine gland function leads to dry eye and dry mouth [1,2]. In addition, a wide variety of other disease manifestations can occur in SS. The clinical manifestations of SS are divided into the exocrine gland features and the extraglandular disease features .
The clinical manifestations of exocrine gland disease in SS will be reviewed here. The extraglandular manifestations, the classification and diagnosis, and the treatment of both sicca and extraglandular manifestations are discussed separately. (See "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Diagnosis and classification of Sjögren's syndrome" and "Treatment of dry eye in Sjögren's syndrome: General principles and initial therapy" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Overview of the management and prognosis of Sjögren's syndrome".)
Major features — The exocrine gland involvement is primarily dry eye disease and salivary hypofunction, but other manifestations may also be seen. The exocrine manifestations include:
●Dry eye disease – The ocular symptoms of dry eyes are irritation, grittiness, and a foreign body sensation. Signs include diminished tear flow as measured by the Schirmer test, ocular surface staining with vital dyes, and increased tear osmolarity.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64:347.
- Pertovaara M, Korpela M, Uusitalo H, et al. Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis 1999; 58:423.
- Asmussen K, Andersen V, Bendixen G, et al. A new model for classification of disease manifestations in primary Sjögren's syndrome: evaluation in a retrospective long-term study. J Intern Med 1996; 239:475.
- https://www.dentalhealth.org/uploads/download/resourcefiles/download_68_1_The%20Challacombe%20Scale.pdf (Accessed on March 07, 2017).
- Schiødt M, Christensen LB, Petersen PE, Thorn JJ. Periodontal disease in primary Sjögren's syndrome. Oral Dis 2001; 7:106.
- Wu AJ. The oral component of Sjögren's syndrome: pass the scalpel and check the water. Curr Rheumatol Rep 2003; 5:304.
- Soto-Rojas AE, Villa AR, Sifuentes-Osornio J, et al. Oral candidiasis and Sjögren's syndrome. J Rheumatol 1998; 25:911.
- Roguedas AM, Misery L, Sassolas B, et al. Cutaneous manifestations of primary Sjögren's syndrome are underestimated. Clin Exp Rheumatol 2004; 22:632.
- Provost TT, Watson R. Cutaneous manifestations of Sjögren's syndrome. Rheum Dis Clin North Am 1992; 18:609.
- Bernacchi E, Bianchi B, Amato L, et al. Xerosis in primary Sjögren syndrome: immunohistochemical and functional investigations. J Dermatol Sci 2005; 39:53.
- Skopouli FN, Papanikolaou S, Malamou-Mitsi V, et al. Obstetric and gynaecological profile in patients with primary Sjögren's syndrome. Ann Rheum Dis 1994; 53:569.
- Lehrer S, Bogursky E, Yemini M, et al. Gynecologic manifestations of Sjögren's syndrome. Am J Obstet Gynecol 1994; 170:835.
- Marchesoni D, Mozzanega B, De Sandre P, et al. Gynaecological aspects of primary Sjogren's syndrome. Eur J Obstet Gynecol Reprod Biol 1995; 63:49.
- Mulherin DM, Sheeran TP, Kumararatne DS, et al. Sjögren's syndrome in women presenting with chronic dyspareunia. Br J Obstet Gynaecol 1997; 104:1019.
- Jonsson MV, Skarstein K, Jonsson R, Brun JG. Serological implications of germinal center-like structures in primary Sjögren's syndrome. J Rheumatol 2007; 34:2044.
- Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002; 61:554.
- Vitali C. Classification criteria for Sjögren's syndrome. Ann Rheum Dis 2003; 62:94.
- Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 2017; 76:9.
- Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol 2017; 69:35.
- 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; 64:475.
- 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; 64:911.
- Shimazaki J, Goto E, Ono M, et al. Meibomian gland dysfunction in patients with Sjögren syndrome. Ophthalmology 1998; 105:1485.
- Goto E, Matsumoto Y, Kamoi M, et al. Tear evaporation rates in Sjögren syndrome and non-Sjögren dry eye patients. Am J Ophthalmol 2007; 144:81.
- Schein OD, Hochberg MC, Muñoz B, et al. Dry eye and dry mouth in the elderly: a population-based assessment. Arch Intern Med 1999; 159:1359.
- Hochberg MC, Tielsch J, Munoz B, et al. Prevalence of symptoms of dry mouth and their relationship to saliva production in community dwelling elderly: the SEE project. Salisbury Eye Evaluation. J Rheumatol 1998; 25:486.
- Daniels TE, Silverman S Jr, Michalski JP, et al. The oral component of Sjögren's syndrome. Oral Surg Oral Med Oral Pathol 1975; 39:875.
- Rhodus NL, Michalowicz BS. Periodontal status and sulcular Candida albicans colonization in patients with primary Sjögren's Syndrome. Quintessence Int 2005; 36:228.
- van der Reijden WA, Vissink A, Veerman EC, Amerongen AV. Treatment of oral dryness related complaints (xerostomia) in Sjögren's syndrome. Ann Rheum Dis 1999; 58:465.
- Rhodus NL, Bloomquist C, Liljemark W, Bereuter J. Prevalence, density, and manifestations of oral Candida albicans in patients with Sjögren's syndrome. J Otolaryngol 1997; 26:300.
- Tapper-Jones L, Aldred M, Walker DM. Prevalence and intraoral distribution of Candida albicans in Sjögren's syndrome. J Clin Pathol 1980; 33:282.
- Belafsky PC, Postma GN. The laryngeal and esophageal manifestations of Sjögren's syndrome. Curr Rheumatol Rep 2003; 5:297.
- Postma GN, Belafsky PC, Aviv JE, Koufman JA. Laryngopharyngeal reflux testing. Ear Nose Throat J 2002; 81:14.
- Soto-Rojas AE, Villa AR, Sifuentes-Osornio J, et al. Oral manifestations in patients with Sjögren's syndrome. J Rheumatol 1998; 25:906.
- Hernandez YL, Daniels TE. Oral candidiasis in Sjögren's syndrome: prevalence, clinical correlations, and treatment. Oral Surg Oral Med Oral Pathol 1989; 68:324.
- Haga HJ, Gjesdal CG, Irgens LM, Ostensen M. Reproduction and gynaecological manifestations in women with primary Sjögren's syndrome: a case-control study. Scand J Rheumatol 2005; 34:45.
- Fukaya E, Watanabe H, Kobayashi H, et al. A case of interstitial cystitis accompanying Sjögren's syndrome. Mod Rheumatol 2005; 15:73.
- van de Merwe JP, Yamada T, Sakamoto Y. Systemic aspects of interstitial cystitis, immunology and linkage with autoimmune disorders. Int J Urol 2003; 10 Suppl:S35.
- Lee KL, Chen MY, Yeh JH, et al. Lower urinary tract symptoms in female patients with rheumatoid arthritis. Scand J Rheumatol 2006; 35:96.
- Van de Merwe J, Kamerling R, Arendsen E, et al. Sjögren's syndrome in patients with interstitial cystitis. J Rheumatol 1993; 20:962.
- Freeman SR, Sheehan PZ, Thorpe MA, Rutka JA. Ear, nose, and throat manifestations of Sjögren's syndrome: retrospective review of a multidisciplinary clinic. J Otolaryngol 2005; 34:20.
- Nocturne G, Mariette X. Sjögren Syndrome-associated lymphomas: an update on pathogenesis and management. Br J Haematol 2015; 168:317.
- Major features
- Pathogenesis and histopathology of exocrine disease
- DRY EYE
- DRY MOUTH
- Clinical and diagnostic features
- - Oral candidiasis
- SALIVARY AND LACRIMAL GLAND ENLARGEMENT
- GENITOURINARY MANIFESTATIONS IN WOMEN
- UPPER AIRWAYS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS