title: Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.
abstract: Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.
authors.affiliation: Department of Dermatology, Almeida Hospital, Oita, Japan. dermatol@kouhoukai.or.jp
authors.forename: Kotaro
authors.initials: K
authors.lastname: Ito
authors.forename: Shinichi
authors.initials: S
authors.lastname: Imafuku
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hamaguchi
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Juichiro
authors.initials: J
authors.lastname: Nakayama
mesh_terms: D000230:Adenocarcinoma / Q000150:complications / Q000276:immunology*; D003882:Dermatomyositis / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000276:immunology; D016726:Plasma Cell Granuloma, Pulmonary / Q000150:complications / Q000276:immunology*; D013274:Stomach Neoplasms / Q000150:complications / Q000276:immunology*; D014157:Transcription Factors / Q000097:blood*
chemical_list: D007074:Immunoglobulin G; C511956:TRIM33 protein, human; D014157:Transcription Factors
keywords: anti-transcription intermediary factor-1-γ/α antibody; dermatomyositis; immunoglobulin G4-related disease; inflammation; pseudotumor