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Frequent autosomal recessive disorder characterized by dysfunction and destruction of exocrine glands. Neurological and immunological function could be involved. Humidification of mucous membrane must be rigorous during anesthetic procedure.

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Sjögren-Gougerot Syndrome; Sicca Syndrome; Gougerot-Houwer-Sjögren Syndrome; Arthro-Oculosalivary Syndrome.

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1 to 3% in general population (primary syndrome). Genetic disorder that mostly affects women (female:male ratio is 9:1).

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Autosomal recessive.

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Strong association with HLA-Dw3 and HLA-Dw4. Because of the significant infiltration of the lacrymal and salivary glands with lymphocytes, acetylcholine activation does not lead to eccrine secretion.

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Sjögren syndrome is characterized by dysfunction and destruction of the exocrine glands, associated with lymphocytes infiltrates and immunological hyperreactivity. These include the affirmation of subjective complaints about oral and/or eye dryness, the objective evidence of salivary gland and/or ocular involvement (lymphocytic infiltrative lesions in the salivary glands), as well as of autoimmune reactivity. Presence of serum autoantibodies against Ro and La.

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Generally, the syndrome has an indolent or slowly progressive course with the disease confined to the exocrine glands. In 30% of patients, general signs occur, which can involve neurological function (abnormal gait, autonomic dysfunction, seizures, movement disorder, ataxia/incoordination, insensitivity to pain, hyporeflexia, and even paraparesis or quadriparesis), immunological disorder (immune system anomalies, dysfunction autoimmunity), pharyngeal abnormality, respiratory tract modification (interstitial-like disease), chronic atrophic gastritis, celiac-like disease, distal renal tubular acidosis, Raynaud phenomenon (35% of cases), and other skin anomalies. Lymphoma and mortality are higher in these patients. All of these general signs are usually moderate. Secondary Sjögren syndrome occurs in 10 to 20% of patients with Rheumatoid Arthritis, Systemic Lupus Erythematosus, and Scleroderma. Various other diseases can imitate this syndrome, including Sarcoidosis, Lipoproteinemia, and Amyloidosis.

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A complete medical history, i.e., evolution of the disease and symptomatology, as well as a thorough physical examination is important. An abdominal and renal echography, chest radiograph, and laboratory investigation may be indicated.

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Prevent a keratoconjunctivitis with strict ocular protection and regular corneal humidification. Avoid respiratory dryness using humidified fresh gases. The administration of anesthetic agents, hypnotics and local anesthetics must be done slowly and in presence of blood pressure monitoring because of the possibility of autonomic nervous system dysfunction.

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Patient receiving chronic therapy must continue the medication until the morning of surgery. Avoid parasympatholytic and anticholinergic drugs because of decrease in gland secretions.

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Mikulicz Syndrome (Mikulicz-Radecki Syndrome; Mikulicz-Sjögren Syndrome; von Mikulicz Syndrome; Mikulicz-Gougerot-Sjögren Syndrome) presents with hypertrophic salivary and lacrimal glands, especially the parotids. The association of lacrimal gland and parotid gland enlargements, dry mouth, and dry eyes are the classic signs of this condition. The tonsils and other glands in the soft tissue of the face and neck may also be involved. It always occurs in association with another underlying disorder such as tuberculosis, leukemia, syphilis, Hodgkin disease, lymphosarcoma, Sjögren syndrome, or lupus erythematous. The incidence for lymphoma is significantly higher in the individuals affected ...

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