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.
Sjögren-Gougerot Syndrome; Sicca Syndrome;
Gougerot-Houwer-Sjögren Syndrome; Arthro-Oculosalivary Syndrome.
1 to 3% in general population (primary syndrome). Genetic disorder that mostly affects women (female:male
ratio is 9:1).
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.
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.
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.
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.
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.
Patient receiving chronic therapy must
continue the medication until the morning of surgery. Avoid parasympatholytic and
anticholinergic drugs because of decrease in gland secretions.
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