title: [A case report of IgG4-related sclerosing disease with lung involvement].
abstract: IgG4-related sclerosing disease(IgG4-RSD) is a kind of lymphoplasmacytic disease with multi-organ involvement and is characterized by serum IgG4  elevation and tissue IgG4 positive plasma cell infiltration. Autoimmune pancreatitis, sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis  and lymphadenopathy make up its main clinical manifestations. This difficult case was a middle-aged female with onset as muiltiple lymph nodes and  glands enlargement, including lacrimal gland, salivary glands and pancreas. Meanwhile, repeated examinations of auto-antibodies and serum IgG4  were all negative. The patient didn't respond well to glucocorticoid therapy, and further progressed to rare lung involvement presenting as lung nodule.  This complex entity was eventually diagnosed as IgG4-RSD by the support of histopathology evidence of IgG4 immunohistochemistry stain. Though  IgG4-RSD has been known for years, it is still underappreciated in China and case reports are scarce. The case report here with literature review is just to  enhance the recognition of this disease regarding its pathogenesis, various clinical manifestations, diagnosis and therapy.
authors.affiliation: Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing 100191, China.
authors.forename: Ying-jian
authors.initials: YJ
authors.lastname: Zhang
authors.forename: Jin-xia
authors.initials: JX
authors.lastname: Zhao
authors.forename: Rui
authors.initials: R
authors.lastname: Liu
authors.forename: Xiang-yuan
authors.initials: XY
authors.lastname: Liu
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008168:Lung / Q000473:pathology; D008875:Middle Aged; D010195:Pancreatitis / Q000276:immunology / Q000473:pathology; D012598:Sclerosis / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D003074:Solitary Pulmonary Nodule / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G