title: Certain Autoimmune Manifestations Are Associated With Distinctive Karyotypes and Outcomes in Patients With Myelodysplastic Syndrome: A Retrospective Cohort Study.
abstract: Autoimmune manifestations (AIMs) are common in patients with myelodysplastic syndrome (MDS). This study aimed to investigate whether AIMs are associated with a specific cytogenetic abnormalities and worse survival in patients with MDS. A total of 67 MDS patients with AIMs and 134 age- and sex-matched MDS patients without AIMs, all of whom received medical care at Seoul National University Hospital from January 2000 through July 2014, were enrolled. The clinical features, chromosomal abnormalities, and outcomes were examined. The effect of AIMs on mortality was estimated after adjusting for age, sex, and the International Prognostic Scoring System. The mean age (±SD) at the time of MDS diagnosis was 54.5 ± 17.1 years, and 44.8% of patients were male. Neutrophilic dermatosis (ND; Sweet syndrome and pyoderma gangrenosum) was the most prevalent AIM (n = 24 36%]), followed by Behcet disease (10 [15%]), rheumatoid arthritis (9 [13%]), vasculitis (8 [12%]), myositis (3 [4%]), spondyloarthropathy (3 [4%]), and systemic lupus erythematous (2 [3%]). ND and vasculitis occurred at the time of MDS diagnosis, whereas other AIMs occurred years after MDS diagnosis. Deletion of 5q was associated with ND (P = 0.001), whereas trisomy 8 was associated with Behcet disease (P = 0.015). Strikingly, ND was associated with a 1.8-fold increase in mortality (95% CI 1.033-3.093; P = 0.038). Certain AIMs in MDS patients are associated with distinctive karyotypes and worse survival. A larger study is needed to confirm whether the presence of AIMs influences disease outcome in MDS.
authors.affiliation: From the Division of Rheumatology (SJL, JKP, EYL, EBL, YWS), Seoul National University Hospital; Department of Molecular Medicine and Biopharmaceutical Sciences (SJL, JKP, YWS), Graduate School of Convergence Science and Technology, and College of Medicine, Medical Research Institute, Seoul National University, Seoul; Division of Rheumatology (SHJ), Chung Buk University Hospital, Cheongju, Department of Pathology (KCJ); and Division of Hematology and Oncology (S-SY), Seoul National University Hospital, Seoul, Korea.
authors.forename: Sang Jin
authors.initials: SJ
authors.lastname: Lee
authors.forename: Jin Kyun
authors.initials: JK
authors.lastname: Park
authors.forename: Eun Young
authors.initials: EY
authors.lastname: Lee
authors.forename: Sang Hyun
authors.initials: SH
authors.lastname: Joo
authors.forename: Kyeong Cheon
authors.initials: KC
authors.lastname: Jung
authors.forename: Eun Bong
authors.initials: EB
authors.lastname: Lee
authors.forename: Yeong Wook
authors.initials: YW
authors.lastname: Song
authors.forename: Sung-Soo
authors.initials: SS
authors.lastname: Yoon
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000453:epidemiology* / Q000235:genetics*; D001528:Behcet Syndrome / Q000235:genetics; D002869:Chromosome Aberrations / Q000706:statistics & numerical data*; D005260:Female; D006801:Humans; D059785:Karyotype; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000453:epidemiology* / Q000235:genetics* / Q000401:mortality; D011379:Prognosis; D017511:Pyoderma Gangrenosum / Q000235:genetics; D012189:Retrospective Studies; D016463:Sweet Syndrome / Q000235:genetics