Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report

Abstract

Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate. Nevertheless, some patients are refractory to this therapy. Alternative approaches include rituximab, mepolizumab, and intravenous immunoglobulin. Accumulating evidence highlight a new promising drug in EGPA therapy-imatinib mesylate (IM), tyrosine kinase inhibitor. This drug is a key pharmacological agent in treating various types of hematological malignancies and FIP1L1/PDGF-RA-positive hypereosinophilia. In this article, we present a case demonstrating successful treatment of EGPA with IM; we also discuss possible mechanisms of IM efficacy in EGPA treatment and future perspectives of this therapeutic approach.

Imatinib might constitute a treatment option for lung involvement in COVID-19

“…In fact, this drug has been reported as arthritis suppressor and inhibitor of IL-6 and other pro-inflammatory cytokines according to murine models [15,16]. In this regard, positive effects have been observed lowering inflammation in patients diagnosed with rheumatoid arthritis [17][18][19], asthma [20] and other chronic inflammatory disorders such as Crohn's disease [21,22] and refractory eosinophilic granulomatosis with polyangiitis [23]. Likewise, imatinib has been linked to improving pulmonary endothelial barrier dysfunction and edema observed in acute lung injury and sepsis [24,25].…”

Serum levels of PDGF-CC as a potential biomarker for the diagnosis of Kawasaki disease

“…Another retrospective study demonstrated that PDGF can be used as a marker for the clinical diagnosis of cerebral amyloid angiopathy-related inflammation/vasculitis [ 18 ]. In line with these, imatinib mesylate, a PDGFR tyrosine kinase inhibitor, has been shown to have therapeutic potential for refractory eosinophilic granulomatosis with polyangiitis and giant cell arteritis [ 24 , 25 ]. These studies emphasize the potential role of PDGF in vasculitis.…”

Jak Inhibitors in the Treatment of Systemic Vasculitides

“…It was previously shown that Tcells and associated cytokines such as IL-6, IL-10, IL-12, IL-23 and type I interferons play an important role in of AAV pathogenesis via JAK/STAT pathway activation (28,29). Imatinib mesylate which is a tyrosine kinase inhibitor was recently reported to be effective in the treatment of a case with EGPA (30). Recently, a case series including 10 patients with AAV (6 with GPA, 3 with MPA and 1 with EGPA) reported the efficacy and safety of TOF.…”