Acute dacryoadenitis as atypical presentation in Sjögren Syndrome

Purpose To present a case report of a patient with acute dacryoadenitis as an atypical clinical presentation of Sjogren Syndrome. Methods A 37-year-old female developed acute left dacryoadenitis with poor response to oral corticosteroids. She had no history of any other clinical symptoms. During the following weeks she developed cervical lymphadenopathies, arthralgia and mild asthenia. Complete blood analysis, imaging diagnostic techniques, C-reactive protein (CRP) and autoimmunity tests showed normal results, but high levels of anti-Ro antibodies were found, suggesting Sjogren Syndrome (SS) as the etiopathological cause. Results All symptoms and clinical findings improved after successive treatment with intravenous corticosteroids, hydroxychloroquine and azathioprine. At the present moment the patient remains stable using maintenance treatment with immunosuppressive drugs. Conclusion Chronic destruction of the lacrimal gland is the most common cause of dry eye and ocular symptoms in SS. Acute dacryoadinitis is considered an atypical manifestation of this syndrome. Classical systemic corticosteroids treatment for SS usually needs association of immunosuppressive drugs, including biological treatments as a recent new option to control the disease progression and inflammatory activity. Topic cyclosporine and pilocarpine have reported benefits for severe xerophthalmia in recent medical trials.