A subset of patients presenting with sicca features suggestive of primary Sjögren's syndrome (pSS) do not fulfill diagnostic or histopathological criteria. This presentation was previously designated as dry eyes and mouth syndrome (DEMS) or sicca asthenia polyalgia syndrome (SAPS). We sought to define the underlying clinical, laboratory, and histological features of these patients.
The study population consisted of 27 consecutive patients with DEMS/SAPS; 54 patients with pSS served as controls. Medical charts were retrospectively evaluated for clinical and serological data and frozen sera were tested for the presence of antibodies against HIV, hepatitis C virus, and thyroid antigens. Immunohistochemical analysis of paraffin embedded tissues was also performed.
Sicca symptoms and nonspecific musculoskeletal pain were the commonest clinical features of patients with DEMS/SAPS; positive titers of antibodies against thyroid peroxidase was the main underlying abnormality found in 16 out of 27 (59.2%) of patients with DEMS/SAPS compared to 11 out of 54 (20.4%) of pSS controls (p = 0.0009). Histological analysis of the minor salivary gland (MSG) biopsies of patients with DEMS/SAPS disclosed a mild inflammatory infiltration of the interstitial tissue with a predominantly perivascular distribution.
Patients with DEMS/SAPS present with sicca features and nonspecific musculoskeletal complaints, have high prevalence of antithyroid antibodies, and their MSG biopsies demonstrate a mild interstitial lymphocytic infiltration with a predominantly perivascular distribution. In the setting of clinical practice, we propose that in the presence of DEMS/SAPS testing for antithyroid antibody should be performed.