Atypical Sjögren’s Syndrome Associated with Autoimmune Autonomic Neuropathy: A Presentation Resembling Hereditary Dysautonomia

Introduction: Sjögren’s Syndrome (SS) is a systemic chronic autoimmune disorder of unknown etiology characterized by salivary and lacrimal glands immune-mediated damage, leading to dryness of the mouth (xerostomia) and eyes (xerophthalmia). But atypical sjogren’s syndrome refers to cases where the syndrome’s symptoms are diverse and not limited to the classic dry eyes and dry mouth or where it presents with systemic complications like neuropathies, skin rashes, vasculitis, etc. These presentations can delay diagnosis because they don’t immediately suggest the disease, requiring doctors to consider Sjogren’s in patients with symptoms like peripheral neuropathy, joint pain without arthritis, etc.Methods-Casereport: We report a 33-year-old male with lifelong symptoms of dysautonomia, including orthostatic hypotension, fatigue, heat intolerance, paroxysmal supraventricular tachycardia, and exercise intolerance since childhood. Neurological examination revealed distal sensorimotor weakness. Laboratory workup was notable for positive lupus anticoagulant and borderline elevated anticardiolipin IgM antibodies, suggesting a possible autoimmune basis for his symptoms. Lip biopsy of the patient suggested, features can be considered to be suggestive of Sjogren’s.Discussion: His orthostatic hypotension test was positive. And his Nerve biopsy showed chronic axonopathy. His initial treatment included IVIG, with modest clinical improvement. He later was started on T. Gabapentine 100 mg OD for neuropathy and Metoprolol 25 mg OD, for the management of his blood pressure. Currently, he is just continuing the therapy as planned.Conclusion: This case highlights the importance of considering atypical Sjögren’s syndrome as a differential diagnosis when autoimmune etiologies present alongside dysautonomic features. It also emphasizes the need for further research into optimal management and treatment strategies in this area.