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Microscopic polyarteritis presenting with chest infections and acute appendicitis
1Department of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin.
Abstract:
We describe a 38-year-old male with antineutrophil cytoplasmic auto-antibody (ANCA) positive microscopic polyarteritis who presented with recurrent chest infections, lung haemorrhage, renal insufficiency and acute appendicitis. Appendectomy was followed by resolution of abdominal symptoms and the surgical specimen revealed vasculitis of the serosal vessels. A renal biopsy was performed because of impaired renal function and this revealed focal necrotising glomerulonephritis with absence of immune deposits. Chest infections were treated with antibiotics resulting in partial clinical response, but pulmonary symptoms relapsed and a complete resolution was achieved only after plasma exchange and the administration of cyclophosphamide. Our observation emphasises the protean manifestations of microscopic polyarteritis and the relationship between ANCA and disease activity.
Insights
This case study highlights antineutrophil cytoplasmic auto-antibody (ANCA) positive microscopic polyarteritis, a rare condition with diverse symptoms including lung and kidney issues. Prompt treatment involving plasma exchange and cyclophosphamide is crucial for managing this ANCA-associated vasculitis.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Microscopic polyarteritis is a rare systemic vasculitis associated with antineutrophil cytoplasmic auto-antibodies (ANCA).
- ANCA-associated vasculitis typically affects small blood vessels, leading to diverse organ damage.
- Understanding the varied clinical presentations is key for timely diagnosis and effective management.
Observation:
- A 38-year-old male presented with recurrent chest infections, pulmonary hemorrhage, renal insufficiency, and acute appendicitis.
- Appendectomy revealed vasculitis of serosal vessels, and renal biopsy showed focal necrotizing glomerulonephritis without immune deposits.
- Initial antibiotic treatment for chest infections provided only partial relief, with relapse occurring.
Findings:
- Complete resolution of pulmonary symptoms was achieved after plasma exchange and cyclophosphamide treatment.
- The case demonstrates the protean manifestations of ANCA-positive microscopic polyarteritis.
- A strong correlation between ANCA levels and disease activity was observed.
Implications:
- This case underscores the importance of considering ANCA-associated vasculitis in patients with unexplained multi-organ involvement.
- Aggressive immunosuppressive therapy, including plasma exchange and cyclophosphamide, is effective in managing severe manifestations.
- Further research into the pathogenesis and treatment of microscopic polyarteritis is warranted.