Pitfalls in the management of isolated pulmonary Takayasu's arteritis after surgery: a case report of an experience during 34 months after a pulmonary artery graft replacement

Background: Several controversial matters still remain unresolved in the management of Takayasu's arteritis, especially after vascular intervention. First, a definitive diagnostic tool has not been established to assess disease activity correctly. Second, the optimal medical regimen has not been established to prevent restenosis of the vascular lesion. Surgical treatments have been rarely performed to relieve critical vascular stenosis in isolated pulmonary Takayasu's arteritis, but their postoperative courses on long-term follow-up periods have not been sufficiently reported. Case Presentation: A 48-year-old man underwent a successful graft replacement for severe right main pulmonary artery stenosis due to isolated pulmonary Takayasu's arteritis. The patient had remained asymptomatic with no clinical inflammatory signs under adequate anticoagulation therapy since then. However, stenosis of the prosthetic graft accompanied by marked pulmonary hypertension was detected 18 months after surgery. Anti-inflammatory treatment with only 5 mg/day of oral prednisolone was then implemented, and the stenosis remained unchanged with the patient being stable for the next 16 months. Conclusions: This is the first published case report that describes the actual clinical course with a long-term follow-up period after surgery for isolated pulmonary Takayasu's arteritis, including images of the stenosed prosthetic graft. This case suggests that patients should be followed with multiple complementary diagnostic techniques on the assumption that restenosis is highly possible and unpredictable even after surgery. Besides, sufficient anti-inflammatory treatment should be applied as soon as possible after surgery no matter how inactive the disease appears to be, although its optimal regimen especially during the inactive inflammatory phase needs to be further established.