Case Presentation:
A 66-year-old woman with a past medical history of scleroderma, inflammatory lung disease, and remote bilateral breast cancer status post bilateral mastectomy and chemotherapy was admitted for lung biopsy. The patient developed flu-like symptoms and was started on oral antibiotics after she was seen in an urgent care clinic. Despite this, she developed worsening dyspnea and hemoptysis. Her outpatient CT chest showed multiple lung cavities with bilateral fibrotic changes. Her initial blood test was positive for anti-neutrophil cytoplasmic antibodies (ANCA), anti-cyclic citrullinated peptide antibodies (CCP), and rheumatoid factor (RF). Because of this, she was admitted for a lung biopsy as a workup for vasculitis disease. Initially, the patient was isolated in a room with negative pressure to rule out tuberculosis (TB). Her QuantiFERON TB test and acid fast bacilli smears were negative. Later, a thoracic surgery team was consulted to perform lung biopsy through video-assisted thoracoscopic surgery (VATS). Her lung biopsy revealed metastatic breast cancer that was positive for estrogen receptor, and a necrotizing granulomatous inflammation that was consistent with granulomatosis with polyangiitis (GPA) formerly known as Wegener ‘s disease. The oncology team recommended to start her on anastrozole as a treatment for breast cancer. The rheumatology team recommended a loading dose of intravascular methylprednisone and then a maintenance dose of oral prednisone. The patient underwent Permacath insertion to start her on outpatient rituximab infusion as a treatment for vasculitis. In addition, she was started on trimethoprim-sulfamethoxazole as Pneumocystis pneumonia prophylaxis as well as her home dose of mycophenolic acid as scleroderma therapy.
Discussion:
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, which includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is an organ- and life-threatening vasculitis. Several studies reported that up to 8% of patients with a malignant disease had ANCA-associated vasculitis. Sixty-three percent of the patients with malignancy related vasculitis had hematologic neoplasm. There are two forms of vasculitis: primary and secondary vasculitis. Secondary vasculitis has been linked to several processes, such as medications side effect, allergic reaction, rheumatologic and neoplastic disease. A close relationship between the diagnosis of malignancy and onset of vasculitis has been reported in a number of patients. Most of the vasculitis were cutaneous leukocytoclastic (45%) and polyarteritis nodosa (36%). The exact pathogenesis of malignancy-associated vasculitis is unclear. However, we hypothesise that the inflammatory responses provoked by the underlying neoplasm might contribute to the pathogenesis. This case report is the only report in the medical literature that showed an association between GPA and recurrent breast cancer.
Conclusions:
In this report, we describe a very rare case of a patient who presented lung cavities and was diagnosed with recurrent metastatic breast cancer with concomitant GPA vasculitis in the same lung lesion. We are anticipating that this case will serve to spread the awareness of cancer screenings in patients with newly diagnosed vasculitis.