A case of IgG4‐related autoimmune disease with multiple organ involvement

H. Nakamura The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki UniversityCorrespondence[email protected]

H. Wada The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

T. Origuchi Nagasaki University School of Health Science

A. Kawakami The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

N. Taura The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

T. Aramaki The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

K. Fujikawa The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

N. Iwanaga The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

Y. Izumi The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

K. Aratake The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

H. Ida The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

T. Taguchi Second Department of Pathology, Nagasaki University School of Medicine

J. Irie Department of Pathology, Nagasaki Municipal Hospital

M. Akiyama Department of Internal Medicine, Nagasaki Municipal Hospital, Nagasaki, Japan

A. Mizokami Department of Internal Medicine, Nagasaki Municipal Hospital, Nagasaki, Japan

T. Tsutsumi Department of Internal Medicine, Nagasaki Municipal Hospital, Nagasaki, Japan

K. Eguchi The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University

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Abstract

A 52‐year‐old male was admitted with autoimmune pancreatitis (AIP), showing mononuclear cell infiltration in both the pancreas and salivary glands with both normal sialography and anti‐SS‐A/SS‐B antibodies. Although the AIP improved with glucocorticoid treatment, subsequent abdominal computed tomography (CT) revealed a nodular shadow in the bilateral kidneys, which was confirmed as interstitial nephritis by renal biopsy. The patient's serum immunoglobulin G4 (IgG4) level was 10 times higher than the upper limit of the normal range. IgG4‐positive mononuclear cell infiltration was detected in the salivary gland, pancreas, and kidney. A new entity proposed as ‘IgG4‐related autoimmune disease’ was considered.

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A case of IgG4‐related autoimmune disease with multiple organ involvement

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A case of IgG4‐related autoimmune disease with multiple organ involvement

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