Abstract
We herein report the case of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with anti-glomerular basement membrane (anti-GBM) antibody positivity that successfully treated with mizoribine (MZR) as an immunosuppressive drug for remission maintenance therapy after the initiation of dialysis in addition to plasma exchange (PE) and glucocorticoid treatment to control the disease condition. A 79-year-old woman developed serious renal dysfunction and pulmonary alveolar hemorrhaging due to MPO–ANCA and anti-GBM antibody double-positive vasculitis. She was started on hemodialysis and was treated with methylprednisolone (m-PSL) pulse therapy with PE, followed by oral prednisolone (PSL). The pulmonary alveolar hemorrhaging disappeared, and both antibody titers immediately decreased but then rose again. Thus, m-PSL pulse therapy performed again in combination with combined with MZR treatment. Her poor renal function was irreversible; however, this therapy decreased both antibody titers, and they did not increase again. The patient developed pancytopenia and hyperuricemia. It was considered likely that these conditions developed in association with MZR treatment. We, therefore, measured the patient’s blood concentration of MZR, and the maintenance dose was finally set at 50 mg after each dialysis session. The patient’s pancytopenia and hyperuricemia improved and PSL could be smoothly tapered. This is the first case report of the use of MZR for remission maintenance therapy in a patient on hemodialysis who was positive for both ANCA and anti-GBM antibodies. The findings suggest that MZR can be used safely and effectively in such cases.
Highlights
As the initial treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with antiglomerular basement membrane antibodyWe report a case of myeloperoxidase (MPO)-ANCA-associated vasculitis with anti-GBM antibody positivity that was successfully treated with mizoribine1 3 Vol:.(1234567890)CEN Case Reports (2020) 9:42–47(MZR) as an immunosuppressive drug for remission maintenance therapy after the initiation of dialysis in addition to plasma exchange (PE) and GC treatment to control the disease condition
McAdoo et al retrospectively analyzed the clinical features and long-term outcomes of a large cohort of 568 AAV patients from 4 European centers, including 41 patients with anti-GBM antibody disease and 37 double-positive patients, and reported that among the double-positive patients, older patients and patients with a longer duration of symptoms before the diagnosis were likely to have AAV
In the acute phase, the clinical course of the double-positive group closely resembles that of anti-GBM antibody disease, which is associated with serious renal failure and pulmonary alveolar hemorrhaging in approximately one-third of patients
Summary
As the initial treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with antiglomerular basement membrane (anti-GBM) antibody. She was found to have anemia and severe renal dysfunction (serum creatinine: 10.78 mg/dL) and was transferred to our hospital in December 2016 She was treated for asthma using Breo Ellipta as an inhalant and had suffered from interstitial pneumonia for several years, but showed no tendency toward exacerbation. We could not deny that these shadows indicated pneumonia, we considered them to reflect pulmonary alveolar hemorrhage due to the presence of blood in the sputum Based on these findings, we diagnosed the patient with AAV with anti-GBM antibody. We were unable to confirm the linear deposition of IgG to the glomerular basement membrane by a renal biopsy in this case, so we did not prove directly that the elevated titer of anti-GBM antibody in her blood test was associated with a disease condition. We cannot exclude the possibility that the elevated titer of the anti-GBM antibody was not related to the pathogenicity of the disease, as the titer was low
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