title: Concomitant occurrence of IgG4-related pleuritis and periaortitis: a case report with review of the literature.
abstract: IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Some recent reports have demonstrated that this disease can occur in the respiratory system including the pleura. Herein, we describe the first documented case of concomitant occurrence of IgG4-related pleuritis and periaortitis. A 71-year-old Japanese female with a history of essential thrombocythemia presented with persistent cough and difficulty in breathing. Computed tomography demonstrated thickening of the right parietal pleura, pericardium, and periaortic tissue and pleural and cardiac effusions. Histopathological study of the surgical biopsy specimen of the parietal pleura revealed marked fibrous thickening with lymphoplasmacytic infiltration. Phlebitis was noted, however, only a few eosinophils had infiltrated. Immunohistochemical study revealed abundant IgG4-positive plasma cell infiltration and high ratio of IgG4-/IgG-positive plasma cells (84%). Therefore, a diagnosis of IgG4-related pleuritis was made with consideration of the elevated serum IgG4 level (684 mg/dL). Recently, the spectrum of IgG4-related sclerosing disease has expanded, and this disease can occur in the pleura, pericardium, and periaortic tissue. Although histopathological analysis of the pericardium and periaortic tissue was not performed in the present case, it was suspected that thickening of the pericardium and periaortic tissue was clinically due to IgG4-related sclerosing disease. Our clinicopathological analyses of IgG4-related pleuritis and pericarditis reveal that this disease can present as dyspnea and pleural and pericardial effusion as seen in the present case, therefore, it is important to recognize that IgG4-related sclerosing disease can occur in these organs for accurate diagnosis and treatment.
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hodohara
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Aya
authors.initials: A
authors.lastname: Furuya
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Aya
authors.initials: A
authors.lastname: Fujishiro
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Okuno
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Miyuki
authors.initials: M
authors.lastname: Yoshii
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Horinouchi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ayaka
authors.initials: A
authors.lastname: Shirakawa
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ayumi
authors.initials: A
authors.lastname: Harada
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Muneo
authors.initials: M
authors.lastname: Iwai
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Yoshida
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Kagotani
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshida
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Okabe
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D001706:Biopsy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis* / Q000097:blood; D007150:Immunohistochemistry; D010496:Pericardium / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology*; D010998:Pleurisy / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011237:Predictive Value of Tests; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013256:Steroids / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D015854:Up-Regulation
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G; D013256:Steroids
keywords: IgG4-related sclerosing disease; periaortitis; pleuritis