{"id":"b74cad2f3392","type":"article","url":"https://hartvaat.nl/2026/02/24/cardiale-sarcoidose-als-eerste-uiting-van-sarcoidose-verloopt-ernstiger/","title":"Cardiale sarcoïdose als eerste uiting van sarcoïdose verloopt ernstiger","title_en":"Diagnostic delay and phenotypic differences in cardiac sarcoidosis: a descriptive study of diagnostic and follow-up clinical data","category":"algemeen","category_label":"Algemeen","professions":["cardioloog"],"tags":["cardiale-sarcoidose"],"journal":"Open Heart","doi":"http://openheart.bmj.com/cgi/content/short/13/1/e003934?rss=1","source_url":"https://doi.org/http://openheart.bmj.com/cgi/content/short/13/1/e003934?rss=1","authors":["Kullberg","S.","Faxen","J.","Cagan","J.","Torabzadeh","H.","Eklund","A.","Smed-So&#x0308;rensen","A.","Darlington","P.","Eldhagen","P.","Rossides","M."],"significance":5,"published":"2026-02-24","source_date":"2026-02-24","image":"","kennis":["https://hartvaat.nl/kennis/coronairlijden/esc-richtlijn-chronisch-coronairlijden-2024/","https://hartvaat.nl/kennis/cardiometabool/peripartum-cardiomyopathie/"],"congress":"","summary_en":"Worse prognosis in cardiac sarcoidosis (CS) is likely linked to diagnostic delay and to cardiac first presentation ('de novo'). In this retrospective study (95 CS patients, 2003-2024), the median time from first symptoms to diagnosis was 9 months. Patients with diagnostic delay more often received a CRT defibrillator. Time to diagnosis did not differ between de novo CS (n=49) and patients with prior extracardiac sarcoidosis (n=46), but de novo patients more often had severe symptoms at onset. At a median 46 months, de novo patients more often had reduced ejection fraction and an ICD than those with prior extracardiac sarcoidosis — despite more immunosuppressive therapy. Disease thus runs a more severe course when cardiac sarcoidosis is the first manifestation, and may not be adequately altered by immunosuppression.","created":"2026-07-03T10:32:25Z","updated":"2026-07-03T13:31:22Z","licence":"Citeer vrij, met bronvermelding en een link naar hartvaat.nl (de url van het record). Samenvattingen zijn redactioneel werk van HartVaat; de oorspronkelijke publicaties blijven van hun uitgevers (doi). Geen medisch advies.","body_markdown":"Een slechtere prognose bij cardiale sarcoïdose (CS) hangt waarschijnlijk samen met diagnostische vertraging en met een cardiale eerste presentatie ('de novo'). In deze retrospectieve studie (95 CS-patiënten, 2003-2024) was de mediane tijd van eerste klachten tot diagnose 9 maanden. Patiënten met diagnostische vertraging kregen vaker een CRT-defibrillator. Tussen de novo CS (n=49) en patiënten met voorafgaande extracardiale sarcoïdose (n=46) verschilde de tijd tot diagnose niet, maar de novo patiënten hadden vaker ernstige klachten bij aanvang. Na een mediane 46 maanden hadden de novo patiënten vaker een verminderde ejectiefractie en een ICD dan patiënten met voorafgaande extracardiale sarcoïdose — ondanks meer immunosuppressieve therapie. De ziekte verloopt dus ernstiger bij cardiale sarcoïdose als eerste uiting, en wordt mogelijk niet voldoende beïnvloed door immunosuppressie.","abstract_original":"<sec><st>Background</st>\n<p>Worse prognosis in cardiac sarcoidosis (CS) is likely associated with diagnostic delay and cardiac involvement as first sarcoidosis (de novo) presentation, but data are limited.</p>\n</sec>\n<sec><st>Methods</st>\n<p>We retrospectively investigated 95 patients with CS diagnosed 2003&ndash;2024. Using electronic health records, the date of first CS symptoms/signs, immunosuppressant therapy and follow-up data including left ventricular ejection fraction (LVEF), biomarkers and cardiac device therapy were extracted. Median time from first symptoms/signs to CS diagnosis (9 months) was used to define delayed diagnosis.</p>\n</sec>\n<sec><st>Results</st>\n<p>Implantation of cardiac resynchronisation therapy defibrillator was more likely in patients with diagnostic delay (p=0.01). No difference was observed in time to diagnosis between patients with de novo CS (n=49) and those with prior extracardiac sarcoidosis (ECS) (n=46). Severe symptoms at disease onset were more common in de novo CS. At a median of 46 months from diagnosis, de novo patients more often had reduced LVEF (p=0.006) and an implantable cardioverter defibrillator (p&lt;0.05) than those with prior ECS despite receiving more immunosuppressant therapy. De novo patients with diagnostic delay more often had reduced LVEF at CS presentation.</p>\n</sec>\n<sec><st>Conclusions</st>\n<p>Symptom presentation is likely associated with diagnostic delay, but the disease presentation and course seem more severe in de novo CS and may not be altered by immunosuppressants, or demand more aggressive therapy.</p>\n</sec>"}