Clinical manifestations of Sjögren's syndrome: Exocrine gland disease
- Robert Fox, MD, PhD
Robert Fox, MD, PhD
- Section Editor — Sjögren's syndrome
- Member, Rheumatology Clinic
- Scripps Memorial Hospital and Ximed Medical Group
Sjögren’s syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function. SS occurs in a primary form not associated with other diseases and in a secondary form that complicates other rheumatic conditions. The most common disease associated with secondary SS is rheumatoid arthritis.
In primary or secondary SS, decreased exocrine gland function leads to the “sicca complex,” a combination of dry eyes (keratoconjunctivitis sicca [KCS]) and dry mouth (xerostomia) [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 .
A retrospective study of 80 patients with primary SS followed for a median of 7.5 years reported the following frequencies of clinical manifestations :
●KCS and/or xerostomia occurred in all patients and were the only disease manifestation in 31 percent
●Extraglandular involvement occurred in 25 percent
- 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.
- 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 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.
- 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.
- 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.
- 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.
- Behrens A, Doyle JJ, Stern L, et al. Dysfunctional tear syndrome: a Delphi approach to treatment recommendations. Cornea 2006; 25:900.
- 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.