Eosinophilic granulomatosis with polyangiitis and severe cardiac involvement in a patient surviving for 34 years

Abstract

Introduction

Many studies have reported a poor prognosis for eosinophilic granulomatosis with polyangiitis (EGPA) patients with cardiac involvement.

Case study

A woman developed EGPA at 37 years of age, with weight loss, numbness in the right upper and lower extremities, muscle weakness, skin rash, abdominal pain, chest pain, an increased peripheral blood eosinophil count (4165/µL), and necrotizing vasculitis on peroneal nerve biopsy. The patient was treated with prednisolone, immunosuppressants, intravenous immune globulin, and mepolizumab, but she experienced many relapses, with chest pain, abdominal pain, numbness, and paralysis, over a long period. The patient died from aspiration pneumonia at 71 years of age after undergoing left total hip arthroplasty for left hip neck fracture.

Results

Autopsy showed bronchopneumonia in the lower lung lobes on both sides, as well as infiltration of inflammatory cells, including neutrophils and lymphocytes. There was no evidence of active vasculitis in either the lung or colon. At autopsy the heart showed predominantly subendocardial fibrosis and fatty infiltration, but no active vasculitis or eosinophilic infiltration.

Conclusion

To our knowledge, there have been no autopsy reports of EGPA patients who have survived for 34 years with recurrent cardiac lesions. In this case, the cardiac involvement (active vasculitis and eosinophilic infiltration) had improved by the time of death.

Keywords:

• Eosinophilic granulomatosis with polyangiitis
• anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
• asthma
• intravenous immune globulin (IVIG)
• mepolizumab

Disclosure statement

All authors declare that they have no competing interests.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.