Immunoglobulin G4-related constrictive pericarditis identified by cytological examination of pericardial effusion: a case reportReport as inadecuate

Immunoglobulin G4-related constrictive pericarditis identified by cytological examination of pericardial effusion: a case report - Download this document for free, or read online. Document in PDF available to download.

Journal of Medical Case Reports

, 10:359

First Online: 20 December 2016Received: 12 May 2016Accepted: 24 November 2016DOI: 10.1186-s13256-016-1159-1

Cite this article as: Horie, K., Tada, N., Yamaguchi, K. et al. J Med Case Reports 2016 10: 359. doi:10.1186-s13256-016-1159-1


BackgroundImmunoglobulin G4-related disease is increasingly recognized as a systemic autoimmune disorder characterized by immunoglobulin G4-positive lymphocyte infiltration. Organ biopsy and histopathology are the most important diagnostic methods; however, the significance of a cytological examination in immunoglobulin G4-related disease cases is still unclear.

Case presentationA 73-year-old Asian man who was a former tobacco smoker presented with progressive exertional dyspnea, systemic edema, and pericardial effusion. A cytological examination of his pericardial effusion detected three or four plasma cells per high-power field by Giemsa staining. Moreover, immunoglobulin G4-positive plasma cells were detected by immunostaining. Cardiac catheterization after pericardiocentesis revealed that both ventricular pressure traces showed an early diastolic dip and plateau. Positron-emission tomography with F-fluorodeoxyglucose imaging revealed inflammatory foci in his pericardium. A surgical pericardiectomy was performed and the resultant specimen showed significant immunoglobulin G4-positive plasma cell infiltration and marked fibrous thickening of his pericardium; therefore, a diagnosis of constrictive pericarditis due to immunoglobulin G4-related disease was made. Oral administration of 0.6-mg-kg-day prednisolone resolved his heart failure and he was discharged on foot 1 week later.

ConclusionOur experience with this case indicates that cytological examination of pericardial effusion was useful in the diagnosis of immunoglobulin G4-related disease.

KeywordsIgG4-related disease Cytological examination Constrictive pericarditis Positron-emission tomography Case report AbbreviationsF-FDGF-fluorodeoxyglucose

CPConstrictive pericarditis

IgGImmunoglobulin G

IgG4Immunoglobulin G4

IgG4-RDImmunoglobulin G4-related disease

PETPositron-emission tomography

TTETransthoracic echocardiography

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Author: Kazunori Horie - Norio Tada - Keiichirou Yamaguchi - Keitarou Inazawa - Mareyuki Endo - Naoto Inoue


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