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Constrictive pericarditis requiring pericardiectomy: an unusual first presentation of extra-articular rheumatoid arthritis-a case report.

ABSTRACT: We report an unusual case of rheumatoid arthritis presenting for the first time with pericardial constriction and bilateral pleural calcification, in the absence of prior articular disease. A 46-year-old Caucasian male, who initially presented with shortness of breath, intermittent chest tightness and general malaise, underwent extensive diagnostic workup over a period of six months involving multiple hospital admissions. He was found to have pericardial constriction on echocardiogram and ultimately required surgical pericardiectomy due to decompensation. After multiple diagnostic tests and specialist opinion, the aetiology of pericardial disease was ultimately confirmed to be extra-articular rheumatoid disease without synovitis. Significant pericardial constriction can occur as the initial presentation of rheumatoid disease and anti-CCP is a highly specific confirmatory test. Pericardial pathological specimen can be unhelpful in determining this aetiology, and constrictive physiology can occur due to chronic inflammation/fibrosis in the absence of significant calcification.

SUBMITTER: Egwu C

PROVIDER: S-EPMC11375588 | biostudies-literature | 2024 Sep

REPOSITORIES: biostudies-literature

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Constrictive pericarditis requiring pericardiectomy: an unusual first presentation of extra-articular rheumatoid arthritis-a case report.

Egwu Chinelo C Scullion Malo M Gingles Christopher C Kerrigan Sean S

European heart journal. Case reports 20240819 9

<h4>Background</h4>We report an unusual case of rheumatoid arthritis presenting for the first time with pericardial constriction and bilateral pleural calcification, in the absence of prior articular disease.<h4>Case summary</h4>A 46-year-old Caucasian male, who initially presented with shortness of breath, intermittent chest tightness and general malaise, underwent extensive diagnostic workup over a period of six months involving multiple hospital admissions. He was found to have pericardial co ...[more]

PMID: 39239137

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Project description:BackgroundThe extent of pericardiectomy is an important issue in constrictive pericarditis but its impact on long-term outcomes has been rarely reported. We compared long-term results of radical pericardiectomy with conventional phrenic to phrenic pericardiectomy.MethodsNinety patients who underwent pericardiectomies between February 1995 and April 2015 were reviewed retrospectively. They were classified into conventional (n = 37) and radical (n = 53) groups according to pericardiectomy being performed anterior or posterior to the phrenic nerves, respectively. The follow-up duration at outpatient clinic was 37.6 (11.7, 86.6) months and the survival data until 91.6 (54.5, 147.0) months were obtained. The last echocardiographies were done at 22.4 (4.35, 60.85) months.ResultsThe early mortality rate was 4.4% (4/90). They all belonged to the conventional group and died of low cardiac output syndrome. The survival rate was higher in the radical group (P = .032, 74.7 ± 9.2% versus 50.4 ± 11.9% in 20 years). NYHA class of both groups had recovered until the last follow-up but the radical group showed better recovery (P < .001). The conventional pericardiectomy (HR = 6.181; 95% CI (1.042, 36.656)), redosternotomy (HR = 6.441; 95% CI (1.224, 33.889) and preoperative grade of tricuspid regurgitation (HR = 15.003; 95% CI (1.099, 204.894) were associated with late mortality. Right ventricular systolic pressure decreased, and pericardial thickening resolved only in the radical group with significant intergroup differences as time went on. Tricuspid regurgitation worsened after the operation in both groups, but it deteriorated more in the conventional group. However, it improved over time in the radical group.ConclusionsRadical pericardiectomy led to greater improvement in right ventricular systolic pressure and lesser deterioration of tricuspid regurgitation with the passage of time than did the conventional procedure. Conventional pericardiectomy and preoperative higher grade tricuspid regurgitation were associated with long-term mortality.

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Constrictive pericarditis requiring pericardiectomy: an unusual first presentation of extra-articular rheumatoid arthritis-a case report.

Publications

Constrictive pericarditis requiring pericardiectomy: an unusual first presentation of extra-articular rheumatoid arthritis-a case report.

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