Necrotizing vasculitis developing following solid organ transplantation is very rare. It has been reported after renal transplantation and in some cases leads to graft failure. In addition, coronary artery vasculitis has been rarely described after orthotopic cardiac transplantation. We describe the first case of de novo development of necrotizing vasculitis in the lung of a patient who underwent bilateral sequential lung transplantation.
A 27 year-old female lung transplant recipient was admitted to the hospital with a history of progressive dyspnea, cough and fever of 103.1 F. The patient was six months post bilateral sequential lung transplantation for cystic fibrosis. She was on maintenance immunosuppression and had no history of an underlying connective tissue disease. One month post transplantation, the patient had a Rhizopus pulmonary infection that was treated with amphotericin B and posiconazole. At the time of admission she was hypotensive and hypoxemic, requiring supplemental oxygen. Lung examination revealed coarse breath sounds bilaterally with wheezing. Initial laboratory data revealed a normal complete blood count and comprehensive metabolic panel. A chest radiograph showed bilateral airspace disease and a chest computed tomographic scan (CT scan) showed bilateral pleural effusions and extensive airspace disease with ground glass opacities. She underwent emergent bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsies. The BAL showed acute inflammation but no bacteria, fungi, or viruses were isolated. Lung biopsy showed minimal acute rejection (A1) and no evidence of chronic rejection or evidence of infection. Specifically, there was no evidence of the previously treated Rhizopus infection on cytopatholgy or tissue culture. She was started on empiric broad spectrum intravenous antibiotics. She initially received high dose intravenous corticosteroids for possible allograft rejection. She required intubation and mechanical ventilation on day two of hospitalization for worsening respiratory failure. During the course of her hospitalization, cytomegalovirus (CMV) digene hybrid antigen was positive although CMV cultures were negative . The patient continued to have respiratory insufficiency and therefore underwent an open lung biopsy and tracheotomy. The lung biopsy results revealed necrotizing vasculitis and organizing diffuse vascular hemorrhage with no evidence of acute or chronic rejection. The bacterial, fungal, mycobacterial and viral cultures were negative. Serologic testing including anti-neutrophilic cytoplasmic antibodies (ANCA), myeloperoxidase antibody, serine protease antibodies, antinuclear antibodies, anti DNA, and antiribonuclear antibodies were all negative. The patient was given another course of intravenous corticosteroids, five days of intravenous immunoglobulin (IVIG) and mycophenolate mofetil was replaced with azathioprine. Following this treatment regimen, the patient improved rapidly, was able to be extubated and had minimal oxygen requirements. The chest radiograph showed resolution of the airspace disease and ground glass opacities.
The development of necrotizing vasculitis following solid organ transplantation is rare. There are a small number of renal transplant recipients who have developed severe necrotizing vasculitis and had significant graft impairment. Pulmonary capillaritis can occur in the lung transplant recipient and is felt to be a form of acute allograft rejection. This histologic pattern was not seen in our patient. Our patient had no evidence of a pre-existing connective tissue disorder and developed the necrotizing vasculitis while on an immunosuppression regimen. Our patient improved dramatically with a combination of parentral corticosteroids, IVIG and azathioprine.
To our knowledge, this is the first reported case of de novo necrotizing vasculitis.
Oluwole Onadeko, No Financial Disclosure Information; No Product/Research Disclosure Information