Granulocyte Colony-Stimulating Factor and Neutrophilic Aortitis - Abstract
Objectives: We describe a case of Lipegfilgrastim associated aortitis and review the literature.
Results: There are 18 published cases of G-CSF associated aortitis. It appears to occur predominantly in middle-aged females of Asian ethnicity. This has been described with a range of G-CSF products in both malignancy and in healthy individuals. The commonest presenting symptom was fever and 11/18 cases were associated with leucocytosis. Two patients developed complications of dissection and aneurysm formation. Treatment varied across cases with 8/18 treated with systemic corticosteroids with other cases resolving with cessation of G-CSF alone or with non-steroidal anti-inflammatories.
Discussion: Aortitis is a rare but potentially life threatening complication of G-CSF with potential for complications of dissection and aneurysm. Diagnosis is one of exclusion of other potential causes; most importantly infective etiology in patients who may be immunosuppressed. The underlying pathogenesis of this condition, natural history and optimal treatment currently remain unknown however treatment with corticosteroids appears to be effective.
Methods: A review of the literature to date published in Pubmed was performed with cross-referencing of included articles. Seven relevant publications were included for review in detail and these 18 cases are summarised.
Conclusion: Whilst G-CSF therapy is usually well tolerated, fever and leucocytosis should prompt consideration of G-CSF associated aortitis. Optimal management of this condition includes withdrawal of G-CSF therapy and consideration of systemic corticosteroid use given the potential for development of life-threatening complications. Further research is required to better elucidate the underlying immunopathogenesis, natural history and optimal management of this condition.