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Background Pericarditis and pericardial effusion (PE) are rare but potentially life-threatening complications in chronic graft-vs-host disease (cGVHD). Limited data are currently available in this setting, based on small single-center retrospective studies. The purpose of this study is to characterize the clinical presentation, management, and outcomes of pericarditis and PE complicating cGVHD in allogeneic hematopoietic cell transplant recipients. Material and Methods We included all consecutive patients referred to our pericardial diseases referral center from 1st May 2008 to 1st May 2022. All patients had no previous cardiovascular or autoimmune diseases and suffered from severe cGVHD with involvement of at least 2 organs. Pericarditis and PE were diagnosed according to ESC guidelines. Conditioning-related toxicity and infections were carefully excluded. Results Pericarditis was diagnosed in 4 patients (2 males, mean age 48.5 ±9.0 years), of whom 2 developed mild PE. PE without pericarditis was diagnosed in 5 patients (80% male, aged 53.2 ±8.9 years), in 2 patients it was reported as severe, requiring pericardial drainage. The average time after allogeneic hematopoietic stem cell transplantation was 22 months (range, 4 to 58 months). All patients required escalation of the corticosteroid therapy they were chronically taking, and in 4 cases the therapy was boosted with non-steroidal anti-inflammatory drugs. Only one patient developed a recurrence and died of multiorgan failure associated with severe cGVHD (involving eyes, mucous membranes, skin, joints, liver, pleuro-pericardial effusion) refractory to immunosuppressive therapy. Overall survival was 89% and no patient developed constrictive pericarditis. Mean follow-up was 69±55 months. Conclusions Pericarditis and PE related to cGVHD typically occur with mild clinical signs in the context of severe cGVHD, however in our study 22% of patients required pericardial drainage at diagnosis. The mainstay of treatment is high-dose steroids and usually the majority of patients have a favorable outcome.

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