A CASE OF ANIT-NEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS RELAPSING WITH MYOCARDITIS AND RESPONDING TO RITUXIMAB

 

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A CASE OF ANIT-NEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS RELAPSING WITH MYOCARDITIS AND RESPONDING TO RITUXIMAB

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Kentaro
Fujii
Kentaro Fujii kfujii225@gmail.com Keiyu Hospital Nephrology Yokohama Japan *
Yoshikazu Hara y-hara@keiyu-hospital.com Keiyu Hospitalephrology Nephrology Yokohama Japan -
Hiroto Matasuda acehiro2011@yahoo.co.jp Keiyu Hospital Nephrology Yokohama Japan -
Yoshiaki Fujii yfujii@coffee.ocn.ne.jp Keiyu Hospital Nephrology Yokohama Japan -
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Cardiac involvement in microscopic polyangiitis (MPA) is rare. Although pericarditis and myocardial infarction have been previously reported, myocarditis has not yet been documented.

Case report

An 81-year-old man diagnosed with MPA and treated with glucocorticoids for five years was maintained on oral prednisolone (PSL) 5 mg daily. He presented with fever without exacerbation of urinary findings or renal function; Laboratory tests revealed an elevated creatine kinase level of 570 U/L, with electrocardiography demonstrating widespread ST-segment elevation without reciprocal depression. Emergency coronary angiography revealed no evidence of myocardial infarction, whereas subsequent myocardial biopsy confirmed myocarditis with histiocytic, eosinophilic, and lymphocytic infiltration. The patient was managed with intravenous methylprednisolone pulse therapy (500 mg/day for 3 days), followed by oral PSL. The treatment regimen was further supplemented with two infusions of rituximab (500 mg each). Electrocardiographic changes and inflammatory marker levels improved rapidly. The PSL dose was tapered to 5 mg daily, with the patient receiving maintenance rituximab every 6 months.

Myocarditis has been reported in eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA); however, the condition is rare in MPA. Cardiac involvement in patients with MPA usually manifests as ischemic heart disease or pericarditis. In the present case, MPA was initially diagnosed based on positivity for myeloperoxidase–anti-neutrophil cytoplasmic antibody (ANCA) and renal biopsy findings of pauci-immune crescentic glomerulonephritis. However, differentiation from GPA or EGPA has not been fully established. Myocardial biopsy revealed prominent histiocytic rather than eosinophilic infiltration, suggesting ANCA-associated vasculitis with features overlapping those of GPA. Although no standardized treatment regimen has been established for ANCA-associated myocarditis, rituximab has been reported to be effective in EGPA-associated cases. In this patient, combination therapy with corticosteroids and rituximab resulted in a rapid improvement in myocarditis and the prevention of relapse. These findings suggest that rituximab is an effective treatment option for myocarditis associated with ANCA-associated vasculitis.

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