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Severe ophthalmic involvement in granulomatosis with polyangiitis resistant to cyclophosphamide

Published:February 04, 2022DOI:https://doi.org/10.1016/j.jcjo.2022.01.014
      A 63-year-old female presented with acute right vision loss, optic disc edema, headache, and elevated inflammatory markers. She had positive cANCA and was diagnosed with granulomatosis with polyangiitis. She developed nodular scleritis (Fig. 1A) and evolved to right orbital apex syndrome with marked pain, diffuse ophthalmoplegia, and ptosis. Despite treatment with intravenous steroids and 3 doses of cyclophosphamide, her vision worsened to no light perception. Rituximab was started, and it stopped the disease progression. Unfortunately, the patient's vision remained unchanged with diffuse optic atrophy and scleromalacia. Enhancement of the sclera (Fig. 1B) and orbital apex (Fig. 1C, D) are demonstrated on T1-weighted magnetic resonance imaging with gadolinium enhancement.
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