title: Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study.
abstract: BACKGROUND
  This study was designed to assess the epidemiology, characteristics, and outcome of cardiac sarcoidosis (CS) in Finland.
  
  
  RESULTS
  We identified in retrospect all adult (>18 years of age) patients diagnosed with histologically confirmed CS in Finland between 1988 and 2012. A total of 110 patients (71 women) 51±9 years of age (mean±SD) were found and followed up for outcome events to the end of 2013. The annual detection rate of CS increased >20-fold during the 25-year period, reaching 0.31 in 1×10(5) adults between 2008 and 2012. The 2012 prevalence of CS was 2.2 in 1×10(5). Nearly two thirds of patients had clinically isolated CS. Altogether, 102 of the 110 patients received immunosuppressive therapy, and 56 received an intracardiac defibrillator. Left ventricular function was impaired (ejection fraction <50%) in 65 patients (59%) at diagnosis and showed no overall change over 12 months of steroid therapy. During follow-up (median, 6.6 years), 10 patients died of a cardiac cause, 11 patients underwent transplantation, and another 11 patients suffered an aborted sudden cardiac death. The Kaplan-Meier estimates for 1-, 5-, and 10-year transplantation-free cardiac survival were 97%, 90%, and 83%, respectively. Heart failure at presentation predicted poor outcome (log-rank P=0.0001) with a 10-year transplantation-free cardiac survival of only 53%.
  
  
  CONCLUSIONS
  The detection rate of CS has increased markedly in Finland over the last 25 years. With current therapy, the prognosis of CS appears better than generally considered, but patients presenting with heart failure still have poor long-term outcome.
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Riina
authors.initials: R
authors.lastname: Kandolin
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Jukka
authors.initials: J
authors.lastname: Lehtonen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Juhani
authors.initials: J
authors.lastname: Airaksinen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Tapani
authors.initials: T
authors.lastname: Vihinen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Heikki
authors.initials: H
authors.lastname: Miettinen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Kari
authors.initials: K
authors.lastname: Ylitalo
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Kari
authors.initials: K
authors.lastname: Kaikkonen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Suvi
authors.initials: S
authors.lastname: Tuohinen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Petri
authors.initials: P
authors.lastname: Haataja
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Tuomas
authors.initials: T
authors.lastname: Kerola
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Jorma
authors.initials: J
authors.lastname: Kokkonen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Markus
authors.initials: M
authors.lastname: Pelkonen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Päivi
authors.initials: P
authors.lastname: Pietilä-Effati
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.).
authors.forename: Seppo
authors.initials: S
authors.lastname: Utrianen
authors.affiliation: From the Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland (R.K., J.L., M.K.); Heart Center, Turku University Hospital, Turku, Finland (J.A., T.V.); Heart Center, Kuopio University Hospital, Kuopio, Finland (H.M.); Medical Research Center Oulu, University and University Hospital of Oulu, Oulu, Finland (K.Y., K.K.); Heart Hospital, Tampere University Hospital, Tampere, Finland (S.T., P.H.); Päijät-Häme Central Hospital, Lahti, Finland (T.K.); Central Finland Central Hospital, Jyväskylä, Finland (J.K.); South Ostrobothnia Central Hospital, Seinäjoki, Finland (M.P.); Vaasa Central Hospital, Vaasa, Finland (P.P.-E.); and South Karelia Central Hospital, Lappeenranta, Finland (S.U.). markku.kupari@hus.fi.
authors.forename: Markku
authors.initials: M
authors.lastname: Kupari
mesh_terms: D000328:Adult; D000368:Aged; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D005387:Finland / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome
keywords: epidemiology; prognosis; sarcoidosis; therapeutics