In the Journey of Asthma, researchers presented the case of a patient who was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA)—a form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)—with severe cardiac involvement who continued living for several decades after her life-threatening diagnosis.
A 37-year-old woman presented with general malaise, skin rash, as well as pain across her back, chest, and abdomen. She also complained of dyspnea. In the previous 4 months, she had lost 10 kg. She had difficulties walking due to paresthesia, numbness, and pain in her right upper and lower extremities.
She had previously diagnosed with asthma at 34 years of age and was hospitalized for asthma exacerbations aged 36 years.
Because of her current symptoms, her physicians decided to perform a biopsy of her sural nerve in the right lower extremity, which revealed necrotizing vasculitis with eosinophilic infiltration in the tissue adjacent to the nerve. In addition, echocardiography demonstrated an ejection fraction of 53.8%, thickening of the pericardium, diastolic dysfunction, and diffuse hypokinesis.
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“We diagnosed this patient as having EGPA because her condition followed the characteristic clinical course outlined in the Japanese diagnostic criteria for “allergic granulomatosis with polyangiitis/Churg–Strauss syndrome” (as EGPA was formerly known), with symptoms due to vasculitis following asthma and elevated eosinophil levels in the peripheral blood,” the authors of the report wrote.
This diagnosis was made in 1985, allowing the research team to continue monitoring the patient’s health in the decades since. After starting steroid therapy (oral prednisolone, 40 mg/day), her numbness and paralysis improved and she slowly regained her mobility. Her asthma symptoms and body pain gradually improved as well.
Twelve years later, her budesonide dose was increased to 1600 mcg/day due to asthma exacerbations. Nonetheless, her asthma continued to worsen in the decades ahead. From the 31st to 33rd year after her diagnosis, her ejection fraction varied between 57% and 61%, while her serum B-natriuretic peptide varied between 54 and 72 pg/mL. She later died of respiratory failure at 71 years of age.
“The autopsy revealed no necrotizing vasculitis after treatment with corticosteroids, other immunosuppressants, intravenous immunoglobulin, and mepolizumab,” the authors wrote. “To our knowledge, this is the first report of the autopsy findings in a patient who survived for such a long time.”
Reference
Tanaka M, Oshikata C, Yamashita Y, et al. Eosinophilic granulomatosis with polyangiitis and severe cardiac involvement in a patient surviving for 34 years. J Asthma. Published online June 13, 2023. doi:10.1080/02770903.2023.2225618
