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University of Ottawa Heart Institute, Ottawa, ON, Canada
INTRODUCTION: Goodpasture’s syndrome is a condition associated with anti-glomerular basement membrane (GBM) antibodies, characterized by acute pulmonary hemorrhage and glomerulonephritis. The following case describes the manifestations of Goodpasture’s syndrome in a patient who suffered an acute myocardial infarction complicated by cardiogenic shock and venous thromboembolism (VTE).
CASE PRESENTATION: A 43-year-old man was admitted to hospital with an acute myocardial infarction. A chronic smoker, he had been treated with chemotherapy and chest radiation five years earlier for Hodgkin’s lymphoma. With the development of cardiogenic shock, an emergency coronary angiogram was performed. This revealed fresh thrombus in the left main coronary artery and significant triple vessel disease. The patient was therefore referred for emergency coronary artery bypass graft surgery. While on cardiopulmonary bypass (CPB), three saphenous vein grafts were completed. Following surgery, the patient’s hemodynamics improved dramatically. However, hemoptysis represented a major postoperative challenge. Bronchoscopy was repeatedly performed due to ongoing hemoptysis, revealing diffuse alveolar hemorrhage in both lungs. Potential diagnoses of malignancy and infection were excluded by bronchio-alveolar lavage. A diagnosis of pulmonary embolism (PE) was also considered, and computed tomography on postoperative day (POD) 9 demonstrated a right lower lobe embolus. A lower limb venous Doppler study subsequently revealed bilateral deep vein thrombosis. Although initially treated with intravenous heparin, worsening hemoptysis necessitated the insertion of an inferior vena cava filter. Given the persistent and diffuse nature of the alveolar hemorrhage, a diagnosis of vasculitis was also entertained. On POD 19, the results of a vasculitis work-up became available, revealing an anti-GBM level of 33. With microscopic hematuria, and proteinuria demonstrated on a 24-hour urine collection, mild glomerulonephritis was also present. A diagnosis of Goodpasture’s syndrome was therefore made, and pulse corticosteroid and cyclophosphamide therapy was initiated. Daily plasmapheresis was started on POD 20. The patient’s clinical improvement was striking. Within two days, the hemoptysis had completely resolved and the patient was extubated. A renal biopsy was deemed clinically unnecessary given the patient’s rapid recovery. After a total of twelve plasma exchanges, a repeat anti-GBM level was negative. The patient was finally discharged home on POD 35 on long-term coumadin, prednisone and cyclophosphamide.
DISCUSSIONS: Goodpasture’s syndrome is an autoimmune disease characterized by the production of anti-GBM antibodies. Reports of Goodpasture’s developing after structural damage to the kidney or lung suggest that antigen release or basement membrane exposure may initiate anti-GBM production in susceptible individuals. A previous history of Hodgkin’s lymphoma, radiation and chemotherapy may have played such a role in this patient. Anti-GBM production is known to occur in the context of malignancy secondary to monocyte-mediated glomerular injury or tumour antigen deposition in the kidney [1]. Furthermore, previous case reports highlighting a relationship between Goodpasture’s syndrome and Hodgkin’s disease allude to potential immunological mechanisms [2]. Pulmonary hemorrhage in Goodpasture’s syndrome is thought to occur following nonspecific lung injuries that increase alveolar-capillary permeability and allow lung deposition of anti-GBM. Given this patient’s history of smoking, cardiogenic shock, and exposure to CPB, an acute-on-chronic lung injury may have provided the ideal environment for anti-GBM deposition and the formation of pulmonary hemorrhage. To our knowledge, this is the first report documenting an association between cardiogenic shock and CPB with the presentation of Goodpasture’s syndrome.
CONCLUSION: This unusual case of a patient presenting with cardiogenic shock and VTE obscured the diagnosis of Goodpasture’s syndrome. A diagnosis of vasculitis should be considered in the context of prolonged hemoptysis following cardiac surgery.
DISCLOSURE: A. Kulik, None.
REFERENCES
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