Radiotherapy of granulomatosis with polyangiitis occurring in the eyelid: A case report and literature review

Medicine (Baltimore). 2021 Jan 22;100(3):e22794. doi: 10.1097/MD.0000000000022794.

Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is a chronic systemic vasculitis characterized by necrotizing granulomatous vasculitis. The disease mainly affects the middle and small blood vessels and mainly occurs in the upper respiratory tract (nose and paranasal sinuses), lower respiratory tract (lungs), and kidneys. Disease occurrence in the eyelid area is relatively rare. The standard GPA treatment is combination therapy with adrenocortical hormone and immunosuppressants. Radiotherapy as a treatment option for GPA has not been widely investigated.

Patient concerns: A 29-year-old man presented with a 1.0 × 1.0 cm mass without exophthalmos and decreased vision in the left lower eyelid. Computed tomography revealed a mass-like high-density shadow below the left eye with a computed tomography value of 80-108 U.

Diagnosis: The laboratory investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies (titer = 1:40). Biopsy of the lower left eyelid mass revealed necrosis and granulomatous reaction with a large number of inflammatory cell infiltration. After consultation with the pathology department, the diagnosis was determined as left lower eyelid GPA.

Interventions: The patient received 9MeV electron beam radiation therapy in the area of the left lower eyelid lesion.

Outcomes: The lesion in the patient was significantly reduced and the symptom relieved obviously. No symptom recurrence or significant toxicity occurred during or after the treatment. The patient remains under routine follow-up.

Conclusion: We present a case of a male patient with GPA located exclusively in the eyelid area, who underwent successful radiotherapy and achieved a complete response. The lesson we learned from this case study is that for GPA patients, when the standard treatment model fails to achieve good results, novel treatments such as radiotherapy should be considered according to the situation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Diagnosis, Differential
  • Eyelid Diseases / diagnosis*
  • Eyelid Diseases / diagnostic imaging
  • Eyelid Diseases / surgery
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / diagnostic imaging
  • Granulomatosis with Polyangiitis / radiotherapy
  • Humans
  • Male
  • Tomography, X-Ray Computed