How is granulomatosis with polyangiitis associated with end-stage renal disease (ESRD)?

Updated: Mar 05, 2020
  • Author: Julia R Nunley, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Answer

Granulomatosis with polyangiitis is an uncommon systemic disorder characterized by granulomatous and necrotizing inflammation of the upper and lower respiratory tracts and the kidneys. Chronic renal failure occurs in 35% of patients. Skin involvement occurs in 14-77% of patients and is associated with a higher frequency of renal involvement. [9, 10] Cutaneous lesions include purpura (most commonly on lower legs), subcutaneous nodules, and ulcers.

Although cutaneous biopsy findings are frequently nonspecific, 2 distinct histologic findings have been described, including leukocytoclastic vasculitis and granulomatous inflammation. Leukocytoclastic vasculitis is more common and is associated with palpable purpura, a more aggressive course, a higher frequency of renal disease, and an overall poorer prognosis.


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