Ocular presentation of polyarteritis nodosa. Clinical course and management with steroid and cytotoxic therapy

Ophthalmology. 1993 Dec;100(12):1775-81. doi: 10.1016/s0161-6420(93)31405-3.


Background: Polyarteritis nodosa is a necrotizing vasculitis affecting medium and small-sized arteries throughout the body, including ocular tissues. Untreated polyarteritis nodosa carries a very poor prognosis. Treatment with steroids increases the 5-year survival to 48%, whereas addition of cytotoxic immunosuppressive treatment improves the outcome dramatically.

Methods: The authors reviewed five cases of polyarteritis nodosa with a spectrum of ocular findings which preceded and contributed to its diagnosis.

Results: The ophthalmic manifestations of polyarteritis nodosa in these patients included scleritis, peripheral ulcerative keratitis, nongranulomatous uveitis, retinal vasculitis, pseudotumor of the orbit, and central retinal artery occlusion associated with temporal arteritis. In four patients, cytotoxic immunosuppressive therapy was begun promptly, and elimination of inflammation was achieved.

Conclusions: The authors' presentation of these cases is intended to emphasize the fact that ocular inflammation can be the earliest presenting manifestation of polyarteritis nodosa and that its recognition may lead to the early institution of therapy, which can decrease morbidity and mortality in this disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Azathioprine / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Eye Diseases / drug therapy*
  • Eye Diseases / etiology*
  • Female
  • Humans
  • Inflammation / etiology
  • Male
  • Middle Aged
  • Polyarteritis Nodosa / complications*
  • Polyarteritis Nodosa / drug therapy*
  • Prednisone / therapeutic use


  • Cyclophosphamide
  • Azathioprine
  • Prednisone