Vasculitis and Breast Cancer: Mind the Hint

Abstract

Diffuse alveolar haemorrhage related to an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis is an extremely rare form of presentation of breast cancer. Here we report the case of a 77-year-old woman with a histological diagnosis of a papillary ductal carcinoma of the breast presenting with a diffuse alveolar haemorrhage secondary to a perinuclear ANCA-associated vasculitis. To our knowledge, this is the first case ever reported of a diffuse alveolar haemorrhage related to an ANCA-associated small vessel vasculitis as a form of presentation of breast cancer. The therapeutic approach of this paraneoplastic vasculitis included the use of corticosteroids and plasmapheresis, a very useful technique to remove endothelial aggressors (circulating antibodies) as a strategy to earn time for a proper therapeutic decision specifically directed for disease modification, but that can also be associated with several severe adverse effects, which are illustrated in our case.

Angiography Revealing a Possible Paraneoplastic Vasculitis: A Case Report

“…Furthermore, a possible diagnosis of microscopic polyangiitis was considered, given the patient's extensive GI bleed without cutaneous manifestations, ocular disturbances, renal or other upper respiratory tract involvement. This reasoning is similar to that proposed by Esperança-Martin et al in the case of a patient with paraneoplastic vasculitis associated with breast cancer [1]. While further analysis of the vasculitis is required for confirmation, the patient is currently still in the ICU and intubated.…”

“…This was considered even though a temporal relationship between her primary malignancy and vasculitis could not be established due to three factors. One, the extensiveness of her vasculitis suggests there could be a parallel course [1]. Secondly, it is possible that vasculitis appeared as a sign of recurrence of her malignancy as vasculitis may occur in conjunction, before, or after a malignancy [7].…”

“…Vasculitis refers to a rare group of conditions affecting blood vessels. It is categorized into the following three subtypes: radiation-induced vasculitis, systemic vasculitis, and paraneoplastic vasculitis, which is even rarer and accounts for 2-5% of all cases of vasculitides [1]. Radiation-induced vasculitis has been found in patients undergoing radiotherapy and is diagnosable upon imaging [2].…”

“…This has most commonly been associated with hematologic cancers [8] as a leukocytoplasmic vasculitis belonging to the small vessel vasculitis; however, a recent study involving 15 cases has reported vasculitis concurrent with solid malignancies as well [7]. One theory that has been proposed to explain its pathogenesis involves the release of immunogenic factors from the tumor forming immune complexes within the circulatory system leading to a vasculitis picture [1,9,10]. As more and more cases are documented, the following three criteria to establish the diagnosis of a true paraneoplastic vasculitis have been suggested: a close temporal relationship between the malignancy and vasculitis, a parallel course [1], and the resolution of the vasculitis upon treatment of the underlying malignancy [7].…”