Abstract
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease known to affect exocrine glands, leading to symptoms such as dry eyes and mouth. However, pSS can manifest in other systems, including rare genitourinary presentations. Urinary retention, although unusual, has been associated with pSS throughout the disease, particularly when related to neurogenic bladder dysfunction. However, it is unusual for neurogenic bladder dysfunction to be the initial presentation of pSS. We present the case of a 38-year-old woman who initially presented with unexplained urinary retention, followed by the development of systemic symptoms such as hand arthritis, morning stiffness, photosensitivity, and dry eyes. As Shirmer's test was positive and anti-SSA was strongly positive, she was diagnosed with pSS. Treatment with hydroxychloroquine alleviated both her systemic symptoms and urinary retention.