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IgG4-Related Disease of the Pharynx with Spontaneous Regression

      Immunoglobulin G4-related disease (IgG4-RD) is a rare chronic fibroinflammatory condition that may affect any organ, with features of tumefaction, fibrosis, and infiltration of IgG4-positive plasma cells.
      • Stone JH
      • Zen Y
      • Deshpande V
      Mechanisms of disease: IgG4-related disease.
      ,
      • Bhatti RM
      • Stelow EB
      IgG4-related disease of the head and neck.
      Treatment is with corticosteroids, although spontaneous remissions have been reported in IgG4-RD of the pancreas (type 1 autoimmune pancreatitis)
      • Kubota K
      • Kamisawa T
      • Hirano K
      • et al.
      Clinical course of type 1 autoimmune pancreatitis patients without steroid treatment: a Japanese multicenter study of 97 patients.
      ,
      • Hirano K
      • Tada M
      • Isayama H
      • et al.
      Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment.
      and submandibular glands.
      • Suzuki M
      • Nakamaru Y
      • Takagi D
      • et al.
      Spontaneous regression of swollen submandibular glands in IgG4-related disease.
      We report a rare case of a patient with IgG4-RD of the pharynx that spontaneously regressed without treatment.
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