Primary Sjögren’s syndrome without ocular manifestation: a case report

Abstract

Introduction and importance: Sjögren’s Syndrome is a systemic immune disorder, manifested in dry eyes and mouth. Primary Sjögren’s syndrome without ocular manifestation is seldom mentioned in the literature. Case presentation: The authors report a case of a 48-year-old female who complained of dryness of mouth and dysphagia for 6 months. Physical examinations showed dry lips with angular cheilitis, an erythematous tongue, and dry buccal mucosa, with multiple carious teeth. The salivary flow was scanty from the Stenson’s and Wharton’s ducts on both sides. Her ophthalmological examination was normal. Laboratory tests revealed leukopenia, anemia, thrombocytopenia, elevated levels of C-reactive protein and erythrocyte sedimentation rate, a strongly positive antinuclear antibody, anti-SS-A, anti-SS-, and rheumatic factor. Hyperechoic nodules in both parotids were shown by Ultrasonography. Salivary gland biopsy showed lymphocytic infiltration. Diagnosis of primary Sjögren’s syndrome was made. She was treated with Pilocarpine 5 mg for 3 months, Vitamin C, and artificial saliva for oral dryness. She is under continuous follow-up with 50–60% relief, without any systemic complications Discussion: Sjögrenʼs Syndrome affects the exocrine glands causing dry mouth and eyes, and can cause systemic symptoms, including fatigue and joint pain. The incidence of ocular involvement among the reported cases is 86.1%, whereas our patient did not have any ocular involvement, and this represents a rare condition. The differential diagnosis included diabetes mellitus, hypothyroidism, chronic virology infection, and some medications that cause dryness, which were very much ruled out. Treatment of sicca symptoms involves artificial tears and medications that stimulate saliva flow while treatment of systemic disease includes corticosteroids, and various DMARDs, Rituximab. this disease has an increased relative risk for the development of B-cell non-Hodgkinʼs lymphoma. Therefore, patients need to be monitored, especially in the presence of risk factors. Conclusion: It is very important to diagnose this disorder early, using the various diagnostic criteria.