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Diagnostics, treatment and outcomes of cardiac sarcoidosis in a Norwegian cohort.
De Bortoli, Alessandro; Nordøy, Ingvild; Connelly, James Patrick; Viermyr, Hans-Kittil; Bjerkreim, Randi Haukaas; Broch, Kaspar; Olsen, Paul Anders Sletten; Gude, Einar; Fevang, Børre; Jørgensen, Silje F; Trøseid, Marius; Steen, Torkel; Aukrust, Pål; Andreassen, Arne K; Skarpengland, Tonje.
Affiliation
  • De Bortoli A; Department of Cardiology, Oslo University Hospital Rikshospitalet, Norway; Department of Cardiology, Vestfold Hospital Trust, Tønsberg, Norway; Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada. Electronic address: albort@siv.no.
  • Nordøy I; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway.
  • Connelly JP; Division for Radiology and Nuclear Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway.
  • Viermyr HK; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway; Research Institute of Internal Medicine, Oslo University Hospital Rikshospitalet, Norway.
  • Bjerkreim RH; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway; Department of Infectious Diseases, Vestfold Hospital Trust, Tønsberg, Norway.
  • Broch K; Department of Cardiology, Oslo University Hospital Rikshospitalet, Norway.
  • Olsen PAS; Department of Cardiology, Oslo University Hospital Rikshospitalet, Norway.
  • Gude E; Department of Cardiology, Oslo University Hospital Rikshospitalet, Norway.
  • Fevang B; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway.
  • Jørgensen SF; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway; Research Institute of Internal Medicine, Oslo University Hospital Rikshospitalet, Norway.
  • Trøseid M; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
  • Steen T; Department of Cardiology, Oslo University Hospital Ullevaal, Norway.
  • Aukrust P; Research Institute of Internal Medicine, Oslo University Hospital Rikshospitalet, Norway; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
  • Andreassen AK; Department of Cardiology, Oslo University Hospital Rikshospitalet, Norway.
  • Skarpengland T; Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital Rikshospitalet, Norway.
Int J Cardiol ; 400: 131809, 2024 Apr 01.
Article in En | MEDLINE | ID: mdl-38272129
ABSTRACT

BACKGROUND:

Evidence-based guidelines for cardiac sarcoidosis (CS) regarding use of second- and third-line agents, treatment duration, surveillance and prognostic factors are lacking.

OBJECTIVE:

To analyze the clinical presentation, diagnostics, treatment, monitoring and clinical outcomes in a Norwegian cohort.

METHODS:

Using discharge diagnoses between 2017 through 2020 from a large tertiary center, we identified 52 patients with CS. We performed a systematic chart review following a pre-specified checklist. The primary outcome of major cardiovascular events (MACE) was defined as a composite of cardiovascular hospitalization, defibrillator therapy, cardiac transplantation, or death.

RESULTS:

18-fluorodeoxyglucose positron emission tomography (FDG-PET) showed pathological tracer uptake in 35/36 (97%) of immunosuppression-naïve patients. Immunosuppressive treatment was administered to 49/52 patients (94%) for a median of 43 (IQR 34) months; 69% were treated with second-line (methotrexate, azathioprine, mycophenolate mofetil) and 25% with third-line (rituximab, infliximab) agents, respectively. Rituximab reduced inflammation as assessed by interval FDG-PET imaging and was overall well tolerated. Median duration to first MACE was 6 (IQR 10) months and 17/23 patients (74%) experienced a MACE within 12 months from CS diagnosis. No mortality was recorded and 20% achieved full remission. Age below the median of 53 years at time of diagnosis was associated with an increased risk of a MACE.

CONCLUSION:

Long-term immunosuppression including a liberal use of non-steroidal agents, appeared essential in treating CS. Although the burden of cardiovascular events was substantial, the survival was excellent in this contemporary cohort. Prospective randomized studies are urgently needed to define the best therapy for these patients.
Subject(s)
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Sarcoidosis / Cardiomyopathies / Myocarditis Type of study: Clinical_trials / Diagnostic_studies / Guideline / Prognostic_studies / Risk_factors_studies Limits: Humans / Middle aged Language: En Journal: Int J Cardiol Year: 2024 Document type: Article Country of publication:

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Sarcoidosis / Cardiomyopathies / Myocarditis Type of study: Clinical_trials / Diagnostic_studies / Guideline / Prognostic_studies / Risk_factors_studies Limits: Humans / Middle aged Language: En Journal: Int J Cardiol Year: 2024 Document type: Article Country of publication: