Granulomatosis with Polyangiitis ( 2 фото )

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A previously healthy 20-year-old woman presented to the ophthalmology clinic with a 9-day history of pain and decreased vision in the right eye, as well as a 2-week history of a rash and gum swelling. On physical examination, purpura with overlying vesicles and bullae were seen on her chest, back, and anterior legs (Panel A, anterior surface of the left shin). Edema and dark-red discoloration of the gingiva with overlying petechiae were observed — findings consistent with “strawberry gingivitis” (Panel B). Scleral and conjunctival injection with a focal overlying nodule — findings consistent with nodular scleritis — were noted in the right eye (Panel C). Findings on funduscopic examination of the right eye aroused concern about optic neuritis. The patient was admitted to the hospital for further evaluation. Fever, dyspnea, and hemoptysis developed. Friable, hemorrhagic nasopharyngeal mucosa (Panel D) and edematous, ulcerated laryngeal mucosa (Panel E) were seen on endoscopy. Urinalysis showed proteinuria and hematuria. Computed tomography of the chest showed nodules in both lungs (Panel F). A test for proteinase 3 antineutrophil cytoplasmic antibodies was positive. A diagnosis of granulomatosis with polyangiitis was made. Treatment with pulse-dose intravenous methylprednisolone and cyclophosphamide (which was chosen instead of rituximab in this case), followed by a tapering dose of prednisone, was initiated. At the 3-month follow-up, the patient’s condition had improved substantially.
Jian Zhu, M.D. , and Chenghao Fu, M.D.
Published January 10, 2026
N Engl J Med 2026;394:281
DOI: 10.1056/NEJMicm2513376
VOL. 394 NO. 3


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