Sjögren's syndrome: An underdiagnosed condition in mixed connective tissue disease

Abstract

OBJECTIVE:To determine the prevalence of sicca symptoms, dry eye, and secondary Sjögren's syndrome and to evaluate the severity of dry eye in patients with mixed connective tissue disease.METHODS:In total, 44 consecutive patients with mixed connective tissue disease (Kasukawa's criteria) and 41 healthy controls underwent Schirmer's test, a tear film breakup time test, and ocular surface staining to investigate dry eye. In addition, the dry eye severity was graded. Ocular and oral symptoms were assessed using a structured questionnaire. Salivary gland scintigraphy was performed in all patients. Classification of secondary Sjögren's syndrome was assessed according to the American-European Consensus Group criteria.RESULTS:The patients and controls had comparable ages (44.7±12.4 vs. 47.2±12.2 years) and frequencies of female gender (93 vs. 95%) and Caucasian ethnicity (71.4 vs. 85%). Ocular symptoms (47.7 vs. 24.4%) and oral symptoms (52.3 vs. 9.7%) were significantly more frequent in patients than in controls. Fourteen (31.8%) patients fulfilled Sjögren's syndrome criteria, seven of whom (50%) did not have this diagnosis prior to study inclusion. A further comparison of patients with mixed connective tissue disease with or without Sjögren's syndrome revealed that the former presented significantly lower frequencies of polyarthritis and cutaneous involvement than did the patients without Sjögren's syndrome. Moderate to severe dry eye was found in 13 of 14 patients with mixed connective tissue disease and Sjögren's syndrome (92.8%).CONCLUSIONS:Sjögren's syndrome, particularly with moderate to severe dry eye, is frequent in patients with mixed connective tissue disease. These findings alert the physician regarding the importance of the appropriate diagnosis of this syndrome in such patients.

The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren’s Syndrome Diagnosis

“…Data show that patients have symptoms for an average of 3.9 years before being diagnosed with SS [9], and it has been estimated that more than half of adults with SS are undiagnosed [7,10,30]. There are many factors that add to the difficulty of diagnosing SS, including variable expression of ocular and non-ocular symptoms, symptoms that do not always present at the same time, and slow disease progression [7,10,31,32].…”

Diffusional kurtosis imaging of parotid glands in Sjögren's syndrome: Initial findings

“…Sjögren's syndrome (SS) is a chronic autoimmune systematic disease with clinical manifestations such as salivary gland enlargement, xerostomia, and xerophthalmia . The salivary and lacrimal glands are the principal targets of the proposed T‐cell‐mediated chronic inflammation process in SS, and the parotid glands are most frequently involved . Histopathologically, the parotid glands suffer from persistent and progressive focal infiltration of mononuclear cells, and undergo glandular cell apoptosis, parenchymal atrophy, and fibrosis proliferation, resulting in parotid functional impairment …”

Use of T1ρMR imaging in Sjögren's syndrome with normal appearing parotid glands: Initial findings

“…The average T1ρ value of bilateral parotid glands in SS patients without morphological changes of parotid glands was significantly higher than that in healthy volunteers, with a mean increase rate of 14.51%. SS is an autoimmune disease which is characterized by lymphocytic infiltration of the salivary and lachrymal glands . Disruption of the acinar and ductal structures and significant changes in the expression and activation of the matrix metalloproteinases of ECM are important characteristics of this disease .…”

“…Sjögren's Syndrome (SS) is a chronic autoimmune systematic disease characterized by injury of exocrine glands with clinical manifestations that include salivary gland enlargement, xerostomia, and xerophthalmia . The parotid glands are the most frequently involved glands in SS .…”