A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis

Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis.

[1]  K. Okazaki,et al.  Comprehensive Diagnostic Criteria for IgG4-Related Disease , 2014 .

[2]  Y. Kawabata,et al.  Immunoglobulin G4‐related lung disease: Clinicoradiological and pathological features , 2013, Respirology.

[3]  D. Sahani,et al.  Consensus statement on the pathology of IgG4-related disease , 2012, Modern Pathology.

[4]  K. Ohshima,et al.  Immunoglobulin G4‐positive multi‐organ lymphoproliferative syndrome with antiphospholipid antibody syndrome , 2012, The Journal of dermatology.

[5]  J. Tanigawa,et al.  Immunoglobulin G4-related coronary periarteritis in a patient presenting with myocardial ischemia. , 2012, Human pathology.

[6]  Masao Tanaka,et al.  Cutoff Values of Serum IgG4 and Histopathological IgG4+ Plasma Cells for Diagnosis of Patients with IgG4-Related Disease , 2012, International journal of rheumatology.

[7]  T. Hibi,et al.  Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011 , 2012, Modern rheumatology.

[8]  J. Stone,et al.  Rituximab for the Treatment of IgG4-Related Disease: Lessons From 10 Consecutive Patients , 2012, Medicine.

[9]  Joungho Han,et al.  Immunoglobulin G4-related periaortitis mimicking an intramural hematoma. , 2011, The Annals of thoracic surgery.

[10]  H. Sekiguchi,et al.  Pulmonary manifestations of immunoglobulin G4-related sclerosing disease , 2011, European Respiratory Journal.

[11]  O. Matsui,et al.  IgG4-related Lung and Pleural Disease: A Clinicopathologic Study of 21 Cases , 2009, The American journal of surgical pathology.

[12]  B. Petersen,et al.  Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. , 2006, Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association.

[13]  F. Gejyo,et al.  Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. , 2006, Internal medicine.

[14]  H. Sarles,et al.  Chronic inflammatory sclerosis of the pancreas—An autonomous pancreatic disease? , 1961, The American Journal of Digestive Diseases.

[15]  H. Nakajima,et al.  A new clinicopathological entity of IgG4-related autoimmune disease , 2003, Journal of Gastroenterology.

[16]  T. Nikaido,et al.  High serum IgG4 concentrations in patients with sclerosing pancreatitis. , 2001, The New England journal of medicine.