Flare-up of eosinophilic granulomatosis with polyangiitis (EGPA ; Churg-Strauss syndrome) in a patient undergoing long-term hemodialysis treatment

Abstract

A 57-year-old woman was admitted to our hospital because of asthma, fever, and purpura. Sixteen years before admission, she had been diagnosed with asthma. Six years before admission, symptoms of EGPA (eosinophilic granulomatosis with polyangiitis) occurred with peripheral neuropathy and rapid progressive glomerulonephritis. Renal biopsy showed necrotizing crescentic glomerulonephritis. Immunosuppressive therapy improved her extra-renal symptoms, and resulted in normalization of her MPO-ANCA titer. However, her renal function did not recover and hemodialysis was initiated. She was treated with maintenance hemodialysis with low-dose prednisolone (PSL) without relapse for 5 years. After reducing the PSL dose from 5 mg per day to 3 mg per day because of infection, her asthma worsened and she exhibited hypereosinophilia, purpura, and fever, with an increased MPO-ANCA titer. On the basis of a diagnosis of EGPA relapse, immunosuppressive therapy, including steroid pulse therapy, was started and led to the remission of vasculitis. Although, it is very rare for patients with EGPA to be treated with maintenance hemodialysis, it is known that ANCA-associated vasculitis can occur during hemodialysis treatment. Physicians should consider the risk of a flare-up of EGPA at the time of asthma exacerbation with marked eosinophilia during maintenance hemodialysis in patients with EGPA, and then should check MPO-ANCA.