Sjögren Syndrome

At a glance

Frequent autoimmune disorder characterized by dysfunction and destruction of exocrine glands (dry eyes and dry mouth) affecting mostly middle-aged women. Humidification of mucous membrane must be rigorous during anesthetic procedure.

Synonyms

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

Incidence

0.3 to 2.7% in general population (primary syndrome). Disorder that mostly affects women (female:male ratio is 9:1).

Genetic inheritance

Unknown.

Pathophysiology

Strong association with HLA-Dw3 and HLADw4. Because of the significant infiltration of the lacrimal and salivary glands with lymphocytes, acetylcholine activation does not lead to eccrine secretion.

Diagnosis

Primary Sjögren Syndrome requires the presence of four of six criteria: (1) ocular symptoms, (2) oral symptoms, (3) objective evidence of dry eyes, (4) objective evidence of salivary gland involvement, (5) histology, and (6) autoantibodies. Secondary Sjögren Syndrome requires an established connective tissue disease in addition to criteria above. Exclude previous radiotherapy, lymphoma, sarcoidosis, graft versus host disease, anticholinergics, and viral infections. 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.

Clinical aspects

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.

Precautions before anesthesia

A complete medical history, ie, 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.

Anesthetic considerations

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. Underlying ...