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Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.
Gary Y J Chew1, Paul A Gatenby
1Department of Immunology, The Canberra Hospital, Woden, Australian Capital Territory, and Australian National University Medical School, Canberra, Australia. gary.chew2@act.gov.au
Clinical Rheumatology
|September 1, 2006
Summary
Hypocomplementemic urticarial vasculitis syndrome (HUVS) can manifest with cardiac and muscular complications. This case highlights inflammatory myositis with vasculitis recurring despite immunosuppression, suggesting a novel autoimmune disease development.
Area of Science:
- Rheumatology
- Immunology
- Cardiology
Background:
- Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder.
- HUVS typically involves skin, joints, and kidneys, but cardiac and muscular involvement is less common.
Observation:
- A patient with established HUVS presented with congestive heart failure.
- During hospitalization, profound proximal muscle weakness and elevated creatinine kinase levels were noted.
Findings:
- Muscle biopsy revealed inflammatory myositis with vasculitis, indicating disease recurrence.
- The myositis occurred despite ongoing immunosuppressive therapy for HUVS.
Implications:
- This case suggests that HUVS may be associated with the development of secondary autoimmune conditions.
- The findings support the 'waste disposal' hypothesis, where the immune system targets self-antigens released from damaged tissues.
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