Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease characterized by necrotizing inflammation of the small blood vessels. ANCA-associated vasculitis is subclassified into three variants: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis (MPA). Myeloperoxidase (MPO) ANCA is a marker antibody for MPA. Interstitial pneumonia (IP) is occasionally complicated with MPA. However, only a few cases of idiopathic IP develop MPO-ANCA-positive conversion and MPA. Therefore, we present a case of a 70-year-old Japanese man with idiopathic IP who developed MPO-ANCA-positive conversion and MPA. We performed renal biopsy, which revealed pauci-immune crescentic glomerulonephritis. The patient was treated with intravenous methylprednisolone pulse therapy and oral prednisone, and the patient’s laboratory data gradually improved with steroid therapy. The association between the production of MPO-ANCA and IP remains unclear, and the present case suggests that IP plays a role in inducing MPO-ANCA production. Patients with idiopathic IP should be followed-up carefully for an examination of increased MPO-ANCA levels and MPA development. In addition, early gastric cancer was detected during upper gastrointestinal endoscopy in our case, and it could also be important not to miss malignancy in patients with ANCA-associated vasculitis.
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Funding
This work was supported in part by Grants-in-Aid for Scientific Research (19H03677, 19K21596, and 20K08612) from the Ministry of Education, Culture, Sports, Science, and Technology of Japan (MEXT), and the Takeda Research Foundation.
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S.N. contributed to data acquisition and manuscript writing. AE TH ME and TM critically revised the article for important intellectual content. All authors have read and approved the final manuscript.
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The Division of Integrative Renal Replacement Therapy is financially supported by Chugai Pharmaceutical, Terumo, and JMS (T. H. and T. M.). The other authors declare that they have no conflicts of interest.
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This work was approved by the Institutional Review Board of Tohoku Medical and Pharmaceutical University Hospital (2021–4-072). This study was conducted in accordance with the Declaration of Helsinki of the World Medical Association. Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.
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Nakayama, S., Endo, A., Hirose, T. et al. MPO-ANCA-positive conversion and microscopic polyangiitis development in idiopathic interstitial pneumonia: a case report. CEN Case Rep 12, 39–44 (2023). https://doi.org/10.1007/s13730-022-00717-y
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DOI: https://doi.org/10.1007/s13730-022-00717-y