What is the role of surgery in the treatment of granulomatosis with polyangiitis (GPA)?

Updated: Oct 09, 2019
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

The natural history of GPA is such that inflammation may lead to tissue necrosis and damage to the nose, subglottic areas, trachea, and bronchi caused by fibrosis. Surgical intervention can be considered in these situations.

For subglottic stenosis, which occurs in approximately 20% of patients with GPA, treatment is typically unresponsive to systemic immunosuppressive therapy. However, rituximab may be beneficial. [105]

Surgical interventions are usually required in these patients, including laser treatment, mechanical dilation with injection of long-acting corticosteroids, or resection of the stenotic area with reanastomosis. Dilation-injection therapy is performed most commonly, with good effects. [106, 107] Patients typically require multiple procedures, but almost all can achieve prolonged airway patency. [108]

Other surgical considerations in GPA are as follows:

  • Saddle nose deformity - Can be surgically repaired by an otolaryngologist or plastic surgeon, typically using split-calvarial bone  or rib cartilage grafts [109] ; not usually considered an option until the patient has been in remission for a significant period

  • Obstruction of the nasal lacrimal ducts - Can be corrected surgically

  • Recurrent middle ear infections due to dysfunction of the eustachian tube - Can be treated by introducing ventilating tubes through the tympanic membranes

  • Stenosis of major bronchi - May be treated with dilatation or with silastic stents; intralesional injections of corticosteroids may also be considered

  • Visual impairment caused by stretching or compression of the optic nerve – Urgent surgical decompression of the orbit may be necessary

Renal transplantation is an option in patients with end-stage renal disease due to GPA, and can significantly decrease all-cause mortality, largely by reducing the risk of death from cardiovsacular disease. [110] Glomerulonephritis usually does not affect the transplanted kidney.


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