Multisystem presentation of primary Sjögren syndrome

Optic neuritis as an initial presentation of primary Sjögren syndrome

BACKGROUND Primary Sjögren syndrome (pSS) is a progressive autoimmune disease that primarily affects exocrine glands. The clinical presentation of pSS may vary from an asymptomatic condition to severe skin symptom, resulting in a difficult and challenging diagnosis and treatment. METHODS AND RESULTS Here, we report a 47-year-old Chinese woman who lost vision in the right eye for 7 days. She h...

Recurrent parotitis as a presentation of primary pediatric Sjögren syndrome.

Parotitis is a common condition seen in the pediatric population, usually as an isolated occurrence associated with viral or bacterial infection. The differential diagnosis expands when recurrent parotitis is encountered. One etiology is primary pediatric Sjögren syndrome (SS), an autoimmune condition typically associated with dryness of the eyes and mouth in adults. Pediatric patients often pr...

Atypical Presentation of Sjögren-Larsson Syndrome

Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95). This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if int...

Orbital Myositis and Primary Sjögren Syndrome.

Neurological involvement in Primary Sjögren Syndrome.

OBJECTIVES To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of neurological involvement in a cohort of Primary Sjögren Syndrome (pSS) patients followed up in a single center. MATERIAL AND METHODS From a total of 93 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensu...