Current issues in the diagnosis and treatment of Sjögrenʼs syndrome

Abstract

Modification of the European Cooperative Group (EEC) criteria for Sjögren's Syndrome (SS) should lead to less confusion in diagnosis and therapeutic trials. The proposed EEC modification will require either a positive minor salivary gland biopsy or a positive autoantibody against Sjögren's-associated A (Ro) or B (La) antigen. This modification will decrease the proportion of women fulfilling EEC criteria from 3-5% to about 0.5%, which is similar to San Diego and San Francisco criteria. Genetic studies have shown increased frequency of alleles for peptide transporter genes TAP1 (0101) and TAP2 (0101) genes as well as tumor necrosis factor microsatellite a2 alleles. Although these markers confer markedly increased risk, they are found in only a small proportion of patients. An increased frequency of drug (antibiotic) allergy and other allergic manifestations appears present in patients with SS and may be linked to HLA-DR3. Hepatitis C as a cause of sicca symptoms, positive anti-nuclear autoantibodies, and mixed cryoglobulinemia is increasingly reported in different parts of the world. Antibodies against muscarinic M3 receptor and expression of costimulatory molecules (CD80 and CD86) by ductal epithelial cells may play a role in pathogenesis. Treatment with pilocarpine is effective in double-blind trials and low dose oral alpha interferon looks promising in initial open studies. In pregnant patients who exhibit evidence of neonatal heart block, treatment with dexamethasone is preferred over prednisone, since the placenta is unable to metabolically activate the latter compound.