Pulmonary arterial hypertension associated with IgG4-related disease

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

doi:10.2169/internalmedicine.53.0154

Pulmonary arterial hypertension associated with IgG4-related disease

Intern Med. 2014;53(5):493-7. doi: 10.2169/internalmedicine.53.0154.

Authors

Motoko Ishida¹, Tomoya Miyamura, Shinji Sato, Daisaku Kimura, Eiichi Suematsu

Affiliation

¹ Department of Internal Medicine and Rheumatology, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, Japan.

PMID: 24583442
DOI: 10.2169/internalmedicine.53.0154

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.

Publication types

Case Reports

MeSH terms

Antibodies, Anti-Idiotypic / immunology*
Autoimmune Diseases / complications*
Autoimmune Diseases / drug therapy
Autoimmune Diseases / immunology
Female
Glucocorticoids / therapeutic use
Humans
Hypertension, Pulmonary / etiology*
Hypertension, Pulmonary / immunology
Hypertension, Pulmonary / physiopathology
Immunoglobulin G / immunology*
Lymphatic Diseases / complications*
Lymphatic Diseases / drug therapy
Lymphatic Diseases / immunology
Prednisolone / therapeutic use
Young Adult

Substances

Antibodies, Anti-Idiotypic
Glucocorticoids
Immunoglobulin G
Prednisolone