DON’T LET THE DRY MOUTH FOOL YOU

Abstract

SESSION TITLE: Signs and Symptoms of Chest Disease SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Sarcoidosis is a well-known though poorly understood disease process with a multitude of manifestations. Clinicians should be aware of the diverse manifestations, as any evidence of active sarcoidosis requires consideration for treatment. We present a case of Mikulicz syndrome, a rare presentation of sarcoidosis in which the parotid and lacrimal glands are infiltrated by granulomas with resulting dry mouth, dry eyes and occasionally recurrent fevers. CASE PRESENTATION: The patient is a thirty-seven-year-old African-American female with a history of pulmonary and dermatologic sarcoidosis. She was diagnosed after a skin biopsy revealed noncaseating granulomas in the setting of chronic dyspnea with bilateral hilar lymphadenopathy. Systemic glucocorticoids were initiated with improvement in her dyspnea and skin lesions. Over the next several years, she did not tolerate weaning attempts and experienced frequent flares requiring escalating doses of methotrexate and glucocorticoids that led to significant side effects to include diabetes, obesity and bilateral hip avascular necrosis. Ultimately, her dyspnea and cough resolved on methotrexate and her skin lesions improved with a combination of intralesional triamcinolone injections and hydroxychloroquine with successful weaning off of her steroids. However, in subsequent visits, she reported swelling in her jaw along with a dry mouth with no recurrent skin lesions or dyspnea. On exam, enlargement of the parotid and lacrimal glands was recognized as Mikulicz syndrome. As a result, adalimumab was initiated with resolution of her symptoms. DISCUSSION: Mikulicz syndrome is a rare presentation of sarcoidosis, occurring in only 2% of patients with systemic sarcoidosis. It can easily be overlooked or attributed to a different disease process such as Sjogren’s syndrome, IgG4 related disease, lymphoma, vasculitis and HIV. However, it has been associated with significant symptoms affecting quality of life and most importantly represents active disease that requires consideration of treatment. In this case, the early recognition of Mikulicz syndrome led to appropriate treatment with a TNF-alpha antagonist that otherwise may not have been started as her skin and pulmonary sarcoid was relatively quiescent. CONCLUSIONS: Sarcoidosis can present with symptoms in nearly any organ system with significant impact on treatment course and quality of life. Providers who routinely treat sarcoid should be aware of the diverse manifestations of the disease, including the relatively rare presentations like Mikulicz syndrome as they may alter treatment strategy. Reference #1: Ungprasert, Patompong, Cynthia S. Crowson, and Eric L. Matteson. "Clinical characteristics of parotid gland sarcoidosis: a population based study.” JAMA otolaryngology--head & neck surgery 142.5 (2016): 503. Reference #2: Rao, Dinesh, et al. "A Case Report of Mikulicz Syndrome.” Journal of radiology case reports 11.7 (2017): 1. DISCLOSURES: No relevant relationships by Arthur Holtzclaw, source=Web Response No relevant relationships by John Sherner, source=Web Response