Clinical Reviews in Allergy & Immunology

, Volume 49, Issue 3, pp 278–287 | Cite as

The Differential Diagnosis of Dry Eyes, Dry Mouth, and Parotidomegaly: A Comprehensive Review

  • Divi Cornec
  • Alain Saraux
  • Sandrine Jousse-Joulin
  • Jacques-Olivier Pers
  • Sylvie Boisramé-Gastrin
  • Yves Renaudineau
  • Yves Gauvin
  • Anne-Marie Roguedas-Contios
  • Steeve Genestet
  • Myriam Chastaing
  • Béatrice Cochener
  • Valérie Devauchelle-Pensec


Primary Sjögren’s syndrome (pSS) is a frequent autoimmune systemic disease, clinically characterized by eyes and mouth dryness in all patients, salivary gland swelling or extraglandular systemic manifestations in half of the patients, and development of lymphoma in 5 to 10 % of the patients. However, patients presenting with sicca symptoms or salivary gland swelling may have a variety of conditions that may require very different investigations, treatments, or follow-up. Eye and/or mouth dryness is a frequent complaint in clinical setting, and its frequency increases with age. When evaluating a patient with suspected pSS, the first step is to rule out its differential diagnoses, before looking for positive arguments for the disease. Knowledge of normal and abnormal lachrymal and salivary gland physiology allows the clinician to prescribe the most adapted procedures for evaluating their function and structure. New tests have been developed in recent years for evaluating these patients, notably new ocular surface staining scores or salivary gland ultrasonography. We describe the different diagnoses performed in our monocentric cohort of 240 patients with suspected pSS. The most frequent diagnoses are pSS, other systemic autoimmune diseases, idiopathic sicca syndrome and drug-induced sicca syndrome. However, other diseases are important to rule out due to their specific management, such as sarcoidosis, granulomatosis with polyangeitis, IgG4-related disease, chronic hepatitis C virus or human immunodeficiency virus infections, graft-versus-host disease, and head and neck radiation therapy. At the light of these data, we propose a core of minimal investigations to be performed when evaluating a patient with suspected pSS.


Sjögren’s syndrome Xerostomia Xerophtalmia Salivary gland swelling Diagnosis 



American-European Consensus Group


Break-up time


Graft-versus-host disease


Hepatitis C virus


Human immunodeficiency virus


Ocular staining score


Primary Sjögren’s syndrome


Salivary gland ultrasonography


Unstimulated whole salivary flow


Conflict of Interest

The authors have no conflict of interest to declare.




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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Divi Cornec
    • 1
    • 2
    • 3
  • Alain Saraux
    • 1
    • 2
    • 3
  • Sandrine Jousse-Joulin
    • 1
    • 2
    • 3
  • Jacques-Olivier Pers
    • 2
    • 3
    • 4
  • Sylvie Boisramé-Gastrin
    • 4
  • Yves Renaudineau
    • 2
    • 3
    • 4
    • 5
  • Yves Gauvin
    • 6
  • Anne-Marie Roguedas-Contios
    • 7
  • Steeve Genestet
    • 8
  • Myriam Chastaing
    • 9
  • Béatrice Cochener
    • 10
  • Valérie Devauchelle-Pensec
    • 1
    • 2
    • 3
    • 11
  1. 1.Department of RheumatologyBrest Teaching HospitalBrestFrance
  2. 2.EA2216, INSERM ESPRI, ERI29Université de BrestBrestFrance
  3. 3.LabEx IGOBrestFrance
  4. 4.Department of OdontologyBrest Teaching HospitalBrestFrance
  5. 5.Laboratory of ImmunologyBrest Teaching HospitalBrestFrance
  6. 6.Department of Ear, Nose, ThroatBrest Teaching HospitalBrestFrance
  7. 7.Department of DermatologyBrest Teaching HospitalBrestFrance
  8. 8.Department of Neurological Functional ExplorationsBrest Teaching HospitalBrestFrance
  9. 9.Department of PsychiatryBrest Teaching HospitalBrestFrance
  10. 10.Department of OphthalmologyBrest Teaching HospitalBrestFrance
  11. 11.Service de RhumatologieHôpital de la Cavale BlancheBrest CedexFrance

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