ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

Marco Folci, Giacomo Ramponi, Dana Shiffer, Aurora Zumbo, Michele Agosti, Enrico Brunetta

Research output: Contribution to journalReview articlepeer-review


The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.

Original languageEnglish
Pages (from-to)1732175
JournalJournal of Immunology Research
Publication statusPublished - 2019


  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy
  • Cyclophosphamide/therapeutic use
  • Cytoplasmic Granules/metabolism
  • Granulomatosis with Polyangiitis/drug therapy
  • Hematologic Neoplasms/drug therapy
  • Humans
  • Myeloblastin/metabolism
  • Neutrophils/immunology
  • Peptides/metabolism
  • Rituximab/therapeutic use


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