IgG4-related lung disease with atypical CT imaging: a case report.

IgG4-related lung disease is a rare disease, diagnosed when typical pathologic features are seen in the context of increased serum levels of IgG4 and the elevated tissue's IgG4-positive plasma cells. Here we reported the case of a 24-year-old woman with IgG4-related lung disease. This patient presented with fever, cough and shortness of breath. Thoracic computed tomography (CT) images demonstrated multiple nodules or masses with high density in both lungs, and thickened interlobular septa. The 'halo sign' was observed around the high-density lesions of the upper lobes. This range of CT images' characteristics is atypical, which differs from previous reports of this condition.

[1]  Eun Joo Lee,et al.  A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis , 2014, Tuberculosis and respiratory diseases.

[2]  R. Feng,et al.  Refractory IgG4-related lung disease with constitutional symptoms and severe inflammation. , 2014, American journal of respiratory and critical care medicine.

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

[4]  K. Takata,et al.  Cutaneous multicentric Castleman's disease mimicking IgG4-related disease. , 2012, Pathology, research and practice.

[5]  J. Stone.  IgG4-related disease: nomenclature, clinical features, and treatment. , 2012, Seminars in diagnostic pathology.

[6]  K. Imai,et al.  The immunobiology and clinical characteristics of IgG4 related diseases. , 2012, Journal of autoimmunity.

[7]  A. Gown,et al.  Pulmonary Hyalinizing Granuloma With Associated Elevation in Serum and Tissue IgG4 Occurring in a Patient With a History of Sarcoidosis , 2012, The American journal of surgical pathology.

[8]  V. Costes,et al.  [Eosinophilic angiocentric fibrosis: a form of IgG4-related systemic disease?]. , 2012, Annales de pathologie.

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

[10]  K. Uchida,et al.  Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease , 2011, Journal of Gastroenterology.

[11]  Cristina Matos,et al.  Castleman's disease: an unusual presentation. , 2011, Jornal Brasileiro de Pneumologia.

[12]  S. Kawakami,et al.  Characteristic findings in images of extra-pancreatic lesions associated with autoimmune pancreatitis. , 2010, European journal of radiology.

[13]  K. Imai,et al.  The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. , 2010, Autoimmunity reviews.

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

[15]  O. Matsui,et al.  Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. , 2009, Radiology.

[16]  J. Chan,et al.  Lymphadenopathy of IgG4-related Sclerosing Disease , 2008, The American journal of surgical pathology.

[17]  T. Hishima,et al.  Inflammatory pseudotumors in multiple organs associated with elevated serum IgG4 level: recovery by only a small replacement dose of steroid. , 2008, Internal medicine.

[18]  O. Matsui,et al.  High-resolution CT findings in pulmonary hyalinizing granuloma. , 2007, Journal of thoracic imaging.

[19]  S. Aida,et al.  IgG4-positive pulmonary disease. , 2007, Journal of thoracic imaging.

[20]  Y. Nakatani,et al.  Pulmonary hyalinizing granuloma with laryngeal and subcutaneous involvement: report of a case successfully treated with glucocorticoids. , 2004, Internal medicine.

[21]  M. Suga,et al.  Multicentric Castleman's disease manifesting in the lung: clinical, radiographic, and pathologic findings and successful treatment with corticosteroid and cyclophosphamide. , 2003, Internal medicine.