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[A case of polyangiitis overlap syndrome]
R Mizuno1, S Fujimoto, H Shiiki
1First Department of Internal Medicine, Nara Medical University.
Summary
A rare case of polyangiitis overlap syndrome presented with severe abdominal pain and gastric ulcers. This eosinophilic vasculitis involved the stomach and adrenal gland, proving resistant to standard treatments.
Area of Science:
- Internal Medicine
- Pathology
- Rheumatology
Background:
- Polyangiitis overlap syndrome is a rare systemic vasculitis characterized by inflammation of blood vessels.
- Eosinophilia and elevated IgE levels can be associated with certain types of vasculitis, including eosinophilic granulomatosis with polyangiitis.
Observation:
- A 47-year-old male presented with fever, severe epigastric pain, significant eosinophilia (9,812/microliter), and elevated serum IgE (934 IU/ml).
- Diagnostic imaging revealed multiple hemorrhagic gastric ulcers and a left adrenal tumor.
- Gastric ulcers were refractory to conservative management, necessitating total gastrectomy and left adrenalectomy.
Findings:
- Histopathological examination of surgical specimens showed necrotizing angiitis with eosinophilic infiltration and thrombus formation in both the stomach and adrenal gland.
- Persistent eosinophilia despite corticosteroid and immunosuppressant therapy was noted.
- The patient subsequently developed polymononeuropathy in the lower limbs and necrotizing toe lesions, resistant to medical treatment and ganglionic blockade.
Implications:
- The clinical presentation and pathological findings supported a diagnosis of polyangiitis overlap syndrome, with features overlapping Allergic Granulomatosis and Polyarteritis Nodosa.
- This case highlights the complex and potentially severe gastrointestinal and systemic manifestations of eosinophilic vasculitis.
- The refractoriness to initial treatments underscores the challenges in managing this rare condition and the need for further research into therapeutic strategies.