Primary Sjogren's syndrome: a great masquerader

Area of Science:

• Neurology
• Rheumatology
• Immunology

Background:

• Primary Sjogren's syndrome is a chronic autoimmune disease primarily affecting exocrine glands.
• It can present with diverse systemic manifestations beyond typical sicca symptoms.
• Multisystem involvement necessitates a broad differential diagnosis for complex presentations.

Observation:

• A 41-year-old female presented with acute onset paresthesia and quadriparesis, initially mimicking Guillain-Barre syndrome.
• She had a history of fatigue, polyarthralgia, dysphagia, and became edentulous, suggesting underlying systemic disease.
• Clinical examination revealed pallor, emaciation, oral thrush, and areflexic quadriparesis.

Findings:

• Plasmapheresis provided temporary improvement in neurological deficits.
• Diagnostic workup including Schirmer's test, parotid scintigraphy, and salivary gland biopsy confirmed primary Sjogren's syndrome.
• Treatment with corticosteroids resulted in dramatic improvement in hemoglobin and leukocyte counts, alongside neurological recovery.

Implications:

• This case underscores the varied and sometimes atypical presentations of primary Sjogren's syndrome.
• It emphasizes the importance of considering autoimmune disorders in patients with unexplained neurological and systemic symptoms.
• Prompt diagnosis and targeted immunosuppressive therapy can lead to significant clinical improvement in complex Sjogren's syndrome cases.