title: Stage IV sarcoidosis: comparison of survival with the general population and causes of death.
abstract: The objectives of this study were to compare the survival of sarcoid patients with pulmonary fibrosis with that of the general population and to determine the causes of death and the incidence of evolutive complications. This retrospective cohort included 142 sarcoid patients in radiographic stage IV (74 males; mean ± SD age 48.1 ± 12 yrs). Their survival was compared with that of the general French population, matched for the year and age at diagnosis of stage IV disease, sex and length of follow-up. Expected survival probabilities were calculated year-by-year on the basis of probabilities provided by official demographic data for France. Survival curves were based on the Kaplan-Meier method and compared using the log-rank test. During the follow-up period (7.1 ± 4.8 yrs), pulmonary hypertension (PH) was observed in 29.7% of cases and aspergilloma in 11.3%. Long-term oxygen therapy was required in 12%. Survival was 84.1% at 10 yrs, which was worse than for the general population (p = 0.013). 16 (11.3%) patients died from the following causes: refractory PH (n = 5), chronic respiratory insufficiency (n = 4), acute respiratory insufficiency (n = 2), haemoptysis due to aspergilloma (n = 1), heart sarcoidosis (n = 1), nocardiosis (n = 1) and unknown causes (n = 2). Survival is significantly decreased in stage IV patients. 75% of fatalities are directly attributable to respiratory causes.
authors.affiliation: Dept of Pneumology, Avicenne Hospital, University Paris 13, UPRES EA 2363, Assistance Publique Hôpitaux de Paris, Bobigny, France.
authors.forename: A
authors.initials: A
authors.lastname: Nardi
authors.forename: P-Y
authors.initials: PY
authors.lastname: Brillet
authors.forename: P
authors.initials: P
authors.lastname: Letoumelin
authors.forename: F
authors.initials: F
authors.lastname: Girard
authors.forename: M
authors.initials: M
authors.lastname: Brauner
authors.forename: Y
authors.initials: Y
authors.lastname: Uzunhan
authors.forename: J-M
authors.initials: JM
authors.lastname: Naccache
authors.forename: D
authors.initials: D
authors.lastname: Valeyre
authors.forename: H
authors.initials: H
authors.lastname: Nunes
mesh_terms: D000328:Adult; D001228:Aspergillosis / Q000453:epidemiology / Q000628:therapy; D002423:Cause of Death; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010102:Oxygen Inhalation Therapy; D015995:Prevalence; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000628:therapy; D011859:Radiography; D012189:Retrospective Studies; D017565:Sarcoidosis, Pulmonary / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality* / Q000628:therapy; D012720:Severity of Illness Index; D015996:Survival Rate
chemical_list: D007166:Immunosuppressive Agents