Cherqaoui Ayman, Taif Hiba, Laraki Kenza, Fadili Hajar, Snoussi Malak, Zrara Abdelhamid, El Bakkouri Jalila
Systemic lupus erythematosus (SLE) may overlap with Sjögren's syndrome (SS), leading to complex systemic involvement, including neurological and pulmonary manifestations. We report a 60-year-old woman with a 10-year history of SLE-SS overlap presenting with headaches and bilateral visual impairment. Hydroxychloroquine (HCQ) had been discontinued 4 months prior due to HCQ-related optic neuropathy confirmed on optical coherence tomography (OCT). Ophthalmologic evaluation revealed bilateral optic neuritis. Immunological testing showed high-titre antinuclear antibodies (ANAs) in both serum (1:640) and cerebrospinal fluid (CSF) (1:320), with positive anti-dsDNA, anti-SSA/Ro52, anti-Sm/RNP, and anti-ribosomal P antibodies. CSF analysis was normal, without pleocytosis, hyperproteinorrhachia, oligoclonal bands, or intrathecal IgG synthesis (IgG index 0.61). Brain MRI showed non-enhancing periventricular demyelinating lesions, and thoracic CT revealed bilateral bronchiectasis. The patient received intravenous methylprednisolone (500 mg/day × 3 days) followed by oral corticosteroids, with leflunomide added as an immunomodulatory agent given the overlap syndrome context, resulting in a favourable clinical outcome. The absence of intrathecal immunoglobulin synthesis and oligoclonal bands supports blood-brain barrier dysfunction rather than a primary demyelinating process, highlighting the diagnostic challenge in SLE-SS overlap. ANA detection in CSF may serve as a supportive marker for neuropsychiatric lupus (NPSLE) in overlap syndromes.