Case Presentation:

A 13‐year‐old white female presented to the emergency room with complaints of fever, neck stiffness, headache, sore throat, phonophobia, photophobia, nausea, and malaise. She was febrile, in obvious discomfort, and on exam had nuchal rigidity, pain with neck flexion and extension, but no cervical lymphadenopathy. She had a slightly elevated white blood cell count, mild lymphopenia, and anemia; normal urine analysis and cerebrospinal fluid studies; negative rapid strep and Monospot. She continued to have headache and neck pain unrelieved by pain medications and was admitted for observation. Her fever resolved on day 3. Following the lumbar puncture (LP), she complained of midline back pain and persistent headache that were not relieved by Toradol, oxycodone, or caffeine citrate. MRI of the brain and spine showed enhancement from T2 to T11, concerning for epidural abscess versus post‐LP changes. Antibiotics were initiated at that time. She developed fever again on day 7, which persisted despite antibiotics. Repeat MRI after 5 days was unremarkable, with resolution of the previously noted enhancement, which was believed to be inconsistent with an epidural abscess, and antibiotics were discontinued. Shortly thereafter, she complained of left‐sided postauricular pain where a single tender 0.5 × 0.5 cm node was palpated. The fevers persisted daily, and she developed bilateral parotid gland swelling. Further questioning revealed a history of recurrent “neck swelling” for several years, occurring once to twice monthly for the last 6–12 months and at times associated with low‐grade fever or exercise. Rheumatological workup was positive for ANA >1:5120, RF 99 (elevated), and IgG 2517 (elevated). Also positive were anti‐SS‐A and anti‐SS‐B antibodies, direct IgG Coombs, and lupus anticoagulants. Parotid swelling improved by day 13, but she developed pain in multiple joints bilaterally. Her clinical picture was consistent with Sjögren's syndrome, either primary or secondary to systemic lupus erythematosus (SLE). She began prednisone, Plaquenil, and low‐dose aspirin on day 14 with no change in her joint symptoms and continued to have fevers. Prednisone was increased on day 15. Fevers and joint symptoms resolved, and she was discharged on day 16.


Sjögren's is an autoimmune disease characterized by lymphocytic infiltration of the salivary and lacrimal glands usually affecting adults and can be very difficult to diagnose in children. Children often present with nonspecific symptoms and do not meet the classic diagnostic criteria used for adults except for recurrent parotitis. Signs of the disease are shown early on in laboratory studies, which are alone nonspecific. The diagnosis of Sjögren's is further supported by ruling out other autoimmune diseases such as SLE with the absence of lab values consistent with that disease process


The purpose of reporting this case is to show an example of a complex presentation and the difficulty in diagnosing primary Sjögren's in children.


M. Srinivasan ‐ none; W. Bour ‐ none; D. Ankney ‐ none