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Updated: May 2, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary arterial hypertension associated with IgG4-related disease
Motoko Ishida1, Tomoya Miyamura, Shinji Sato
1Department of Internal Medicine and Rheumatology, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, Japan.
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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