What are the possible complications of granulomatosis with polyangiitis (GPA)?

Updated: Aug 31, 2021
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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In a longitudinal cohort consisting of 158 patients with GPA, from the National Institutes of Health (NIH), 86% of patients experienced permanent damage from their disease. [43] Permanent damage includes the following:

  • End-stage renal disease
  • Chronic pulmonary dysfunction
  • Hearing loss
  • Destructive sinus disease
  • Saddle nose deformities
  • Perforation of the nasal septum
  • Proptosis
  • Blindness

Respiratory problems may result from upper-airway obstruction (eg, subglottic stenosis) or pulmonary involvement (eg, pleural effusion, dyspnea, diffuse alveolar hemorrhage [DAH]).

Many patients in the NIH cohort (42%) also experienced permanent treatment-associated morbidity, including hemorrhagic cystitis, osteoporotic fractures, urothelial (bladder) cancer, myelodysplasia, and avascular necrosis. [43] Urotoxic adverse events associated with cyclophosphamide use are related to cumulative dose and oral administration. Cyclophosphamide treatment of systemic vasculitis increases the risk of urothelial cancer 5-fold over that of the general population. [44]

Furthermore, the development of other cancers associated with immunosuppression in patients with AAV is a concern, as it is for patients with other inflammatory rheumatologic and nonrheumatologic diseases and for patients who have undergone organ transplantation. Increased rates of leukemia, lymphoma, and nonmelanoma skin cancers have been reported in a number of studies of treated patients with AAV. The observed overall incidence of cancers in this population is 1.6-2.4 times higher than in the general population. [45] Clinicians caring for these patients should keep this increased risk in mind and refer and/or screen appropriately.

Additionally, an increased rate of cardiovascular events is noted in patients with AAV. A European study that reviewed outcomes during long-term follow-up of patients with GPA and microscopic polyangiitis determined that within 5 years of diagnosis, 14% of patients experienced at least 1 cardiovascular event. This was 3.7 times higher than was expected in the background population. This study determined that older age, diastolic hypertension, negative PR3-ANCA status, and positive MPO-ANCA status are independent determinants of cardiovascular outcomes in patients without prior cardiovascular disease. [46]

A study of a Canadian population-based database with newly diagnosed GPA reported a hazard ratio (HR) of 1.86 for myocardial infarction (MI) and 1.50 for ischemic stroke. The HR for cardiovascular disease (composite outcome of MI or stroke) was highest during the first year after GPA diagnosis (HR 2.88). [47]

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