[IgG4-related XXXX :a clinical analysis and literature review].

@entity1 @entity1200 @entity358 @entity1576
OBJECTIVE: To summarize the clinical features, diagnosis and treatment experience of IgG4-related @entity358 for the sake of improving clinical understanding of this disease and reducing the misdiagnosis and mistreatment rates. METHODS: To analyze the general situation, clinical manifestation, laboratory examination, histopathology, therapy and prognosis of 2 patients with IgG4-related @entity358 , who were admitted in the department of @entity1576 at Changhai Hospital. Publications related to @entity358 were reviewed. RESULTS: Both @entity1 were male with elevated serum IgG4 level (2.25 g/L and 10 g/L respectively). In one @entity1 , radiologic finding showed solid nodules in the lung with ileocecal involvement. He responded well to glucocorticoid. The other @entity1 's computed tomography of lung demonstrated bronchovascular type. Glucocorticoid therapy was effective to both @entity1 . A total of 69 cases with IgG4-related @entity358 were reported worldwide, among whom 39 cases were admitted with chief complaints of respiratory symptoms. However, there were 41 cases suffering extra-pulmonary involvement. Serum IgG4 levels detected in 48 cases were significantly elevated (307-52 500 mg/L). The radiographic pattern of solid nodule type was the most frequent, accounting for 50.7% (35 cases). A total of 31 (44.9%) @entity1 received glucocorticoid therapy with good response and prognosis. CONCLUSION: IgG4-related @entity358 is a rare @entity1200 lack of specific symptoms. Serum immunological examination, radiographic characteristics and histopathology should be comprehensively considered for diagnosis. Glucocorticoid is so far the most acceptable therapy with good response rate.
