Pulmonary Arterial Hypertension Associated with IgG4-related Disease

Motoko Ishida; Tomoya Miyamura; Shinji Sato; Daisaku Kimura; Eiichi Suematsu

doi:10.2169/internalmedicine.53.0154

CASE REPORTS

Pulmonary Arterial Hypertension Associated with IgG4-related Disease

Motoko Ishida, Tomoya Miyamura, Shinji Sato, Daisaku Kimura, Eiichi Suematsu

著者情報

キーワード: IgG4-related disease, pulmonary arterial hypertension

ジャーナルオープンアクセス

2014 年 53 巻 5 号 p. 493-497

DOI https://doi.org/10.2169/internalmedicine.53.0154

詳細

抄録

A 22-year-old woman with generalized lymphadenopathy and symmetrical swelling of the lacrimal and submandibular glands was diagnosed with IgG4-related disease. Biopsy specimens of the lips, lymph nodes, gastrointestinal tract and bronchus showed IgG4-positive plasma cell infiltration. Echocardiography and right heart catheterization revealed a high mean pulmonary arterial pressure. The patient was treated with 50 mg of prednisolone daily and rapidly improved. This is the first reported case of pulmonary arterial hypertension associated with IgG4-related disease.

責任著者(Corresponding author)

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