Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis

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Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis

Some forms of idiopathic retroperitoneal fibrosis (RF) have recently been considered to be a part of the spectrum of immunoglobulin G4 (IgG4)-related disease. This case report is the first description of a spontaneous remission in a patient with pathologically proven IgG4-related RF. Although the pathogenesis and long-term disease behavior of IgG4-related RF remains unknown, we believe that an ...

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Rapidly resolved IgG4-related retroperitoneal fibrosis after steroid pulse therapy

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been establ...

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Isolated IgG4-related interstitial lung disease: unusual histological and radiological features of a pathologically proven case

IgG4-related lung disease is commonly associated with autoimmune pancreatitis. Recently, isolated IgG4-related interstitial lung disease (ILD) without other organ involvement has newly been reported in two cases with clinical features of nonspecific interstitial pneumonitis (NSIP).We report the first case of an isolated IgG4-related ILD in a 78-year-old man with dry cough and dyspnea, whose cli...

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IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. A recurrent case after a 5-year history of spontaneous remission.

CONTEXT A new clinicopathological concept of IgG4-related sclerosing disease affecting various organs has recently been proposed in relation to autoimmune pancreatitis. This report describes the case of IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis, which recurred after a long period of spontaneous remission. CASE REPORT An 80-year-old...

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Biopsy-proven IgG4-related lung disease

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve single or multiple organs. Biopsy-proven lung involvement of this disease is occasionally reported, but not well understood. METHODS Patients with the diagnosis of biopsy-proven IgG4-related lung disease (IgG4-RLD) from Peking Union Medical College Hospital between January 2011 and July 201...

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ژورنال

عنوان ژورنال: Internal Medicine

سال: 2017

ISSN: 0918-2918,1349-7235

DOI: 10.2169/internalmedicine.56.7996