title: Everyday clinical practice in IgG4-related dacryoadenitis and/or sialadenitis: results from the SMART database.
abstract: Abstract Objective. Immunoglobulin (Ig)G4-related disease (IgG4-RD) is a new disease entity that has only been identified this century. Clinical information is thus lacking. We established the Sapporo Medical University and Related Institutes Database for Investigation and Best Treatments of IgG4-related Disease (SMART) to clarify the clinical features of IgG4-RD and provide useful information for clinicians. Methods. Participants comprised 122 patients with IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), representing lacrimal and/or salivary lesions of IgG4-RD, followed-up in December 2013. We analyzed the sex ratio, mean age at onset, organ dysfunction, history or complications of malignancy, treatments, rate of clinical remission, and relapse. Results. The sex ratio was roughly equal. Mean age at diagnosis was 59.0 years. Positron emission tomography revealed that the ratio of other organ involvements was 61.4%. Complications of malignancy were observed in 7.4% of cases. Glucocorticoid was used to treat 92.1% of cases, and the mean maintenance dose of prednisolone was 4.8 mg/day. Rituximab was added in three cases, and showed good steroid-sparing effect. The clinical remission rate was 73.8%, and the annual relapse rate was 11.5%. Half of the cases experienced relapses within 7 years of initial treatment. Conclusion. We analyzed the clinical features and treatments of IgG4-DS using SMART, providing useful information for everyday clinical practice.
authors.affiliation: Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine , Sapporo , Japan.
authors.forename: Motohisa
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Yajima
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takahashi
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Yokoyama
authors.forename: Keisuke
authors.initials: K
authors.lastname: Ishigami
authors.forename: Yui
authors.initials: Y
authors.lastname: Shimizu
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Tabeya
authors.forename: Chisako
authors.initials: C
authors.lastname: Suzuki
authors.forename: Yasuyoshi
authors.initials: Y
authors.lastname: Naishiro
authors.forename: Ken-Ichi
authors.initials: K
authors.lastname: Takano
authors.forename: Ken
authors.initials: K
authors.lastname: Yamashita
authors.forename: Masato
authors.initials: M
authors.lastname: Hashimoto
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Keira
authors.forename: Saho
authors.initials: S
authors.lastname: Honda
authors.forename: Takashi
authors.initials: T
authors.lastname: Abe
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Masaya
authors.initials: M
authors.lastname: Mukai
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Himi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Kohzoh
authors.initials: K
authors.lastname: Imai
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Shinomura
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy; D003607:Dacryocystitis / Q000175:diagnosis* / Q000188:drug therapy; D016208:Databases, Factual; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012793:Sialadenitis / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D011239:Prednisolone
keywords: Autoimmune pancreatitis; Cancer; IgG4-related disease; Mikulicz's disease; rituximab