Arch Cardiovasc Dis. 2017 Aug - Sep;110(8):456-465. doi: 10.1016/j.acvd.2016.12.014. Epub 2017 May 26.
Cardiac sarcoidosis: Diagnosis, therapeutic management and prognostic factors.
Archives of cardiovascular diseases
Catherine Chapelon-Abric, Damien Sene, David Saadoun, Philippe Cluzel, Olivier Vignaux, Nathalie Costedoat-Chalumeau, Jean-Clarles Piette, Patrice Cacoub
Affiliations
Affiliations
- Département de Médecine Interne et d'Immunologie Clinique, CHU Pitié-Salpétrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France. Electronic address: [email protected].
- Département Médecine Interne, Hôpital Lariboisière, 75475 Paris, France.
- Département de Médecine Interne et d'Immunologie Clinique, CHU Pitié-Salpétrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
- Service de Radiologie, CHU Pitié-Salpétrière, 75013 Paris, France.
- Service de Radiologie, Hôpital Cochin, 75014 Paris, France.
- Département Médecine Interne, Hôpital Cochin, 75014 Paris, France.
- Département Médecine Interne, CHU Pitié-Salpétrière, 75013 Paris, France.
- UMR 7211, Inflammation-Immunopathology-Biotherapy Department, Département de Médecine Interne et d'Immunologie Clinique, Sorbonne Universités, UPMC Université Paris, CHU Pitié-Salpétrière, 75005 Paris, France.
PMID: 28566197
DOI: 10.1016/j.acvd.2016.12.014
Abstract
BACKGROUND: Cardiac sarcoidosis (CS) is a severe localization, observed mostly in cardiology departments. Despite appropriate cardiological treatment, CS is a potentially life-threatening condition, and accounts for 13-85% of sarcoidosis-related deaths.
AIMS: This retrospective study aimed to give an overview of CS in a non-cardiac unit, to analyse the effect of first-line immunosuppressive treatment on outcome and survival and to evaluate factors associated with relapses.
METHODS: From 534 cases of sarcoidosis, we selected 59 patients with CS according to "2006 international criteria". We performed an in-depth analysis regarding symptoms, physical signs and cardiac investigation results. Patients were followed for a median period of 60 months.
RESULTS: The median age at cardiac signs was 42 years. Echocardiography abnormalities, isotopic defects and abnormal magnetic resonance imaging findings were observed in 81%, 84% and 92% of patients, respectively. First-line treatment included steroids alone in 24 patients and steroids plus immunosuppressive therapy in 35 patients. Forty-seven (80%) patients recovered; 12 stabilized or worsened. The recovery rate was 75% in the steroids alone group versus 83% in the steroids plus immunosuppressive therapy group. Five (9%) patients died during follow-up, with two deaths attributed to CS. The overall 1- and 5-year survival rates were 98% and 92%, respectively.
CONCLUSION: Our series of patients with CS who received steroids alone or combined with immunosuppressive therapy had a good prognosis, with an overall 5-year survival rate of 92%. The recovery rate was 85%, with no significant difference between patients treated with steroids alone or plus immunosuppressive therapy.
Copyright © 2017. Published by Elsevier Masson SAS.
Keywords: Cardiac sarcoidosis; Corticoïdes; Cyclophosphamide; Methotrexate; Méthotrexate; Rechute taux de guérison; Relapse; Sarcoïdose cardiaque; Steroids; Survival rate
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