Not all Sicca is Sjögren's and not all Sjögren's is Sicca

Abstract

Symptoms of dry eyes or dry mouth, otherwise known as sicca symptoms, are not always present in patients with Sjögren's syndrome (SS). Approximately 20% of patients with SS do not have sicca symptoms. An unusual case of a patient presenting with complete left-sided facial hemiparesis, a history of partial bilateral sensorineural hearing loss who was found to have elevated antinuclear antibody (ANA) with high titer positive SSA/Ro antibody, evidence of bilateral parotitis on imaging and absence of sicca symptoms, prompted us to perform a literature review. Twelve case reports relating facial nerve palsy and Sjögren's were found and only one described a similar constellation of features of unilateral facial weakness and otalgia. Management of facial nerve palsy related to Sjögren's is unclear but pharmacological agents have included corticosteroids, intravenous immune globulin (IVIG), cyclophosphamide, and plasmapheresis. This case report describes a patient whose facial nerve palsy is attributed to SS, explores peripheral and central nervous system involvement in SS, and provides some recommended treatments.

Keywords: facial nerve palsy and sjogren; sjogren and facial palsy; sjogren and sicca.

Figure 1. MRI brain with and without contrast.

T1-Weighted images in sequential order craniocaudally (right to left image) shows enlarged parotid glands bilaterally, more pronounced on right side with nodular ductal dilations and mixed calcifications. This MRI also shows contrast enhancement of the tympanic segments of bilateral facial nerves, compatible with facial neuritis.