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Primary Sjogren's syndrome presenting with generalized autonomic failure
R Sakakibara1, S Hirano, M Asahina
1Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan. sakaki@med.m.chiba-u.ac.jp
European Journal of Neurology
|September 24, 2004
Summary
Primary Sjogren's syndrome can lead to autoimmune autonomic ganglionopathy, causing generalized autonomic failure. This case highlights the progression of Sjogren's syndrome impacting the autonomic nervous system.
Area of Science:
- Rheumatology
- Neurology
- Immunology
Background:
- Sjogren's syndrome is an autoimmune disorder primarily affecting exocrine glands.
- Autonomic dysfunction can occur in autoimmune diseases but is often underdiagnosed.
Observation:
- A 64-year-old woman with primary Sjogren's syndrome developed progressive generalized autonomic failure.
- Symptoms included orthostatic hypotension, cardiovascular denervation, and gastrointestinal dysmotility.
- Autonomic testing revealed significant impairment in autonomic function.
Findings:
- The patient presented with Raynaud's phenomenon, dry eyes/mouth, and positive autoimmune markers, consistent with Sjogren's syndrome.
- Neuropathy assessment was normal, but autonomic testing demonstrated widespread autonomic dysfunction.
- The clinical presentation and test results strongly suggested autoimmune autonomic ganglionopathy as the cause of autonomic failure.
Implications:
- This case underscores the potential for Sjogren's syndrome to cause severe autonomic dysfunction.
- Early recognition and management of autonomic failure in Sjogren's syndrome are crucial.
- Further research is needed to understand the mechanisms linking Sjogren's syndrome and autoimmune autonomic ganglionopathy.