Περίληψη:
Sjogren’s syndrome is a chronic autoimmune disorder, characterized by
lymphocytic infiltration and malfunction of the exocrine glands,
resulting in dry mouth and eyes. The syndrome can present either alone
(primary Sjogren’s syndrome) or in the context of an underlying
connective tissue disease (secondary Sjogren’s syndrome). Systemic
features, resulting from cutaneous, respiratory, renal, hepatic,
neurologic, and vascular involvement, often occur. Two types of primary
Sjogren’s syndrome are currently recognized: a benign disease that
affects quality of life, and a systemic syndrome associated with
increased morbidity and mortality owing to a high risk of malignant
transformation, and that requires close follow-up. Ocular involvement,
manifested as keratoconjunctivitis sicca, is managed with local and
systemic stimulators of tear secretion and supportive surgical
procedures. Treatment of oral manifestations includes intense oral
hygiene, prevention and treatment of oral infections, use of saliva
substitutes, and local and systematic stimulation of salivary secretion.
Cholinergic agents, such as pilocarpine and cevimeline, are helpful in
patients with residual salivary function, and ciclosporin ocular drops
seem to be of some benefit. Systemic immunosuppressives are reserved for
treatment of severe extraglandular manifestations of Sjogren’s syndrome.
Anti-B-cell therapy is a new potential therapy for the glandular and
extraglandular manifestations, such as glomerulonephritis or vasculitis,
in addition to the management of lymphoma associated with Sjogren’s
syndrome. Induction of oral tolerance and gene-transfer modalities were
recently attempted in animal models, with promising results.
Συγγραφείς:
Mavragani, CP
Moutsopoulos, NM
Moutsopoulos, HM
