Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Pulmonary Arterial Hypertension Associated with IgG4-related Disease
Motoko IshidaTomoya MiyamuraShinji SatoDaisaku KimuraEiichi Suematsu
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JOURNAL OPEN ACCESS

2014 Volume 53 Issue 5 Pages 493-497

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Abstract

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.

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© 2014 by The Japanese Society of Internal Medicine
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