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J Thorac Cardiovasc Surg 2006;132:1467-1468
© 2006 The American Association for Thoracic Surgery
Brief Communication |
a Department of Medicine, Division of Rheumatology, Mount Sinai School of Medicine, New York, NY
b Department of Pathology and the Cardiovascular Institute, Mount Sinai School of Medicine, New York, NY.
* Address for reprints: Leslie D. Kerr, MD, Box 1244, Division of Rheumatology, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, New York, NY 10029-6574. (Email: leslie.kerr{at}mssm.edu).
Giant cell arteritis (GCA) is a large-vessel vasculitis that affects older individuals and typically presents with constitutional symptoms, polymyalgia, headache, scalp tenderness, jaw claudication, or sudden vision loss.1
A subset of patients with GCA can present with large artery disease consisting of aortic aneurysm, dissection, or both.2 We have also recently described our own experience with 19 such patients who underwent surgical repair of aortic aneurysms and who were found to have unsuspected giant cell aortitis on histopathologic review.3 This study suggested that large-vessel giant cell aortitis might be a different disease from classic GCA because of its unusual clinical presentation. No conclusions, however, could be drawn regarding the effectiveness of subsequent steroid therapy for this variant of the disease.
A unique opportunity to explore this question arose when a patient with 2 known aortic aneurysms presented. Giant cell aortitis was documented at the repair of the first aneurysm. This patient subsequently lost vision and hearing and regained both after steroid therapy. When the second repair was performed 14 months later, the histopathologic effect of steroid treatment was assessed.
A 67-year-old woman in whom 2 large aneurysms, a thoracic and a thoracoabdominal aneurysm, had been incidentally found on computed tomographic scanning was referred for cardiac catheterization and further management. The catheterization demonstrated severe left main disease, normal left ventricular function, a dilated aortic root with severe aortic insufficiency, and mild narrowing of the right subclavian artery. The patient then underwent a stage I Bentall procedure with osteoplasty of the left main coronary artery. The repair of the thoracoabdominal aneurysm was deferred. On postoperative day 2, she experienced sudden loss of vision in her left eye. The pathology report documented the presence of active giant cell aortitis, and rheumatology consultation was requested. On initial evaluation, the patient denied antecedent symptoms of polymyalgia, headache, scalp tenderness, jaw claudication, joint pains, or constitutional symptoms. She described her vision loss as sudden and accompanied by decreased hearing.
On physical examination, she had no scalp or temporal artery tenderness, and no bruits were appreciated. There was no evidence of synovitis or muscle tenderness. Visual acuity was markedly reduced, and hearing was diminished. Her sedimentation rate was 31 mm/h (Westergren). Treatment with high-dose intravenous steroids (dexamethasone, 4.5 mg every 12 hours) was begun. Over the next 2 weeks, her vision improved only marginally, and the steroids were then tapered to 5 mg/d. This dose was continued, and at 1 month postoperatively, the patient suddenly regained vision in her left eye and noted restoration of hearing. Prednisone, 5 mg/d, was continued for 6 months and then discontinued. Fourteen months after the initial repair, the patient then underwent repair of the remaining thoracoabdominal aneurysm. This specimen demonstrated severe atherosclerotic changes with mural thrombus; no evidence of discrete aortitis was seen. The patient had an uneventful hospital course and was discharged without steroid therapy.
The surgical specimens from both procedures were fixed in formalin, paraffin embedded, and stained with hematoxylin and eosin.
Figure 1, A (2x) and B (10x), depicts the initial thoracic aortic biopsy. This specimen demonstrates active giant cell aortitis. There is an abundance of palisading inflammatory cells, including giant multinucleated cells. The elastic lamina is disrupted by medial necrosis. Figure 2, A (4x) and B (10x), shows the second aortic specimen from the resection of the thoracoabdominal aortic aneurysm 14 months later. In contrast, this specimen demonstrates no evidence of active aortitis. Instead, severe atherosclerotic changes with medial degeneration and a minimal inflammatory infiltrate are seen, which is consistent with healed aortitis.
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The literature currently supports the concept that there is a difference between classic cranial GCA and large-vessel giant cell aortitis. Brack and colleagues2 described a group of patients with upper extremity vascular insufficiency and compared these patients with those with classic cranial GCA. The patients with large-vessel disease rarely presented with headache, jaw claudication, or visual impairment. Nuenninghoff and associates4 found a negative association between cranial symptoms, increased sedimentation rate, and large artery complications from aortitis. Zehr and coworkers5 reported 37 patients with giant cell aortitis, 13 of whom were treated with steroids postoperatively. Because none of these patients underwent repeat operations, the effect of this therapy could not be ascertained. Thus no data exist regarding the steroid responsiveness of this GCA variant.
Thus this case is important because it documents the effect of steroid therapy on giant cell aortitis. The initial biopsy specimen demonstrated active giant cell aortitis. In contrast, the second biopsy specimen obtained after prolonged steroid therapy had no evidence of active disease. Healed aortitis pathologically correlated with clinical improvement. Although these data are limited to one patient, it suggests that large-vessel giant cell aortitis is quite steroid responsive. This histopathologic improvement combined with a dramatically improved clinical outcome underscores the benefits of prompt recognition and treatment of giant cell aortitis.
References
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