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

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

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Bibliographic Details
Published in:The Journal of asthma 2023-12, Vol.60 (12), p.2233-2242
Main Authors: Tanaka, Minaho, Oshikata, Chiyako, Yamashita, Yuga, Isono, Riko, Nakadegawa, Ryo, Masumitsu, Hinako, Motobayashi, Yuto, Osada, Reeko, Takayasu, Hirokazu, Masumoto, Nami, Manabe, Saki, Kaneko, Takeshi, Ueno, Akihisa, Tsurikisawa, Naomi
Format: Article
Language:English
Subjects:
Abdominal Pain
Asthma
Chest Pain
Churg-Strauss Syndrome
Eosinophilia
Female
Granulomatosis with Polyangiitis
Humans
Hypesthesia
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Summary:Many studies have reported a poor prognosis for eosinophilic granulomatosis with polyangiitis (EGPA) patients with cardiac involvement. 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. 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. 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.
ISSN:0277-0903
1532-4303
DOI:10.1080/02770903.2023.2225618