title: Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis.
abstract: Demographic, clinical, and laboratory features that predict underlying malignancy in patients with dermatomyositis (DM) are poorly known. We conducted a retrospective study in all adult patients with a definite (n = 75) or probable (n = 32) diagnosis of DM according to Bohan and Peter criteria or with amyopathic DM (n = 14) who were referred to 2 departments during a 13-year period. The diagnosis of malignancy-associated DM was retained if DM occurred in a context of recently diagnosed malignancy or if a malignancy was diagnosed during the 5 years following the diagnosis of DM. The Kaplan-Meier method was used to assess the cumulative incidence rates of underlying malignancy during the first 5 years of DM. Factors associated with malignancy in patients with DM were identified by Cox proportional hazards models. During the study period, 121 patients fulfilled the inclusion criteria (median age, 52 yr; range, 19-77 yr; women: 70%). For 29 of them, the diagnosis of malignancy-associated DM was retained. The cumulative incidence rate of malignancy was 21 +/- 4% and 28 +/- 5%, 1 year and 5 years after the diagnosis of DM, respectively. The median duration of follow-up of the 92 patients with no malignancy diagnosed was 36 months (range, 1-140 mo). In multivariate analysis, independent factors associated with an underlying malignancy in patients with DM were an age at diagnosis >52 years (hazard ratio [HR], 7.24; 95% confidence interval [CI], 2.35-22.31), a rapid onset of skin and/or muscular symptoms (HR, 3.11; 95% CI, 1.07-9.02), the presence of skin necrosis (HR, 3.84; 95% CI, 1.00-14.85) or periungual erythema (HR, 3.93; 95% CI, 1.16-13.24), and a low baseline level of complement factor C4 (HR, 2.74; 95% CI, 1.11-6.75). Lastly, low baseline lymphocyte count (<1500/mm(3)) was a protective factor of malignancy (HR, 0.33; 95% CI, 0.14-0.80). Taken together, these data may help physicians focus on a group of patients who might benefit from extensive evaluation for malignancy.
authors.affiliation: From the Université Paris 7 (LF, AD, HB, LD, CL, PM, MR) Diderot, Paris; Fédération de dermatologie (LF, AD, HB, LD, CL, PM, MR), AP-HP, Hôpital Saint Louis, Paris; Université Paris 6 (MG, AK, PC) Pierre et Marie Curie, Paris; and Service de médecine interne (MG, AK, PC), AP-HP, Hôpital Saint-Antoine, Paris, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Fardet
authors.forename: Alain
authors.initials: A
authors.lastname: Dupuy
authors.forename: Murielle
authors.initials: M
authors.lastname: Gain
authors.forename: Adrien
authors.initials: A
authors.lastname: Kettaneh
authors.forename: Patrick
authors.initials: P
authors.lastname: Chérin
authors.forename: Hervé
authors.initials: H
authors.lastname: Bachelez
authors.forename: Louis
authors.initials: L
authors.lastname: Dubertret
authors.forename: Celeste
authors.initials: C
authors.lastname: Lebbe
authors.forename: Patrice
authors.initials: P
authors.lastname: Morel
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D003181:Complement C4 / Q000032:analysis; D003882:Dermatomyositis / Q000175:diagnosis* / Q000453:epidemiology; D004890:Erythema / Q000453:epidemiology; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009260:Nail Diseases / Q000453:epidemiology; D009336:Necrosis; D009369:Neoplasms / Q000175:diagnosis* / Q000453:epidemiology; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000453:epidemiology; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology; D055815:Young Adult
chemical_list: D003181:Complement C4