title: The Differential Diagnosis of Dry Eyes, Dry Mouth, and Parotidomegaly: A Comprehensive Review.
abstract: 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.
authors: Department of Rheumatology, Brest Teaching Hospital, Brest, France.|EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.|LabEx IGO, Brest, France.
authors: Divi
authors: D
authors: Cornec
authors: Department of Rheumatology, Brest Teaching Hospital, Brest, France.|EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.|LabEx IGO, Brest, France.
authors: Alain
authors: A
authors: Saraux
authors: Department of Rheumatology, Brest Teaching Hospital, Brest, France.|EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.|LabEx IGO, Brest, France.
authors: Sandrine
authors: S
authors: Jousse-Joulin
authors: EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.|LabEx IGO, Brest, France.|Department of Odontology, Brest Teaching Hospital, Brest, France.
authors: Jacques-Olivier
authors: JO
authors: Pers
authors: Department of Odontology, Brest Teaching Hospital, Brest, France.
authors: Sylvie
authors: S
authors: Boisramé-Gastrin
authors: EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.|LabEx IGO, Brest, France.|Department of Odontology, Brest Teaching Hospital, Brest, France.|Laboratory of Immunology, Brest Teaching Hospital, Brest, France.
authors: Yves
authors: Y
authors: Renaudineau
authors: Department of Ear, Nose, Throat, Brest Teaching Hospital, Brest, France.
authors: Yves
authors: Y
authors: Gauvin
authors: Department of Dermatology, Brest Teaching Hospital, Brest, France.
authors: Anne-Marie
authors: AM
authors: Roguedas-Contios
authors: Department of Neurological Functional Explorations, Brest Teaching Hospital, Brest, France.
authors: Steeve
authors: S
authors: Genestet
authors: Department of Psychiatry, Brest Teaching Hospital, Brest, France.
authors: Myriam
authors: M
authors: Chastaing
authors: Department of Ophthalmology, Brest Teaching Hospital, Brest, France.
authors: Béatrice
authors: B
authors: Cochener
authors: Department of Rheumatology, Brest Teaching Hospital, Brest, France. valerie.devauchelle-pensec@chu-brest.fr.|EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France. valerie.devauchelle-pensec@chu-brest.fr.|LabEx IGO, Brest, France. valerie.devauchelle-pensec@chu-brest.fr.|Service de Rhumatologie, Hôpital de la Cavale Blanche, BP 824, 29609, Brest Cedex, France. valerie.devauchelle-pensec@chu-brest.fr.
authors: Valérie
authors: V
authors: Devauchelle-Pensec
mesh_terms: D000465:Algorithms; D000818:Animals; D003937:Diagnosis, Differential; D015352:Dry Eye Syndromes / Q000175:diagnosis*; D006801:Humans; D007765:Lacrimal Apparatus / Q000473:pathology*; D012469:Salivary Glands / Q000000981:diagnostic imaging / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000175:diagnosis*; D014463:Ultrasonography; D014987:Xerostomia / Q000175:diagnosis*
keywords: Diagnosis; Salivary gland swelling; Sjögren’s syndrome; Xerophtalmia; Xerostomia