Constrictive Pericarditis and Bilateral Pleural Effusions Unmasked in an HLA-B27 Positive Patient

Constrictive pericarditis (CP) is most often idiopathic, postviral, postoperative, or autoimmune in etiology. HLA-B27 is strongly associated with spondyloarthropathies, which may have cardiac manifestations including conduction system abnormalities and aortopathy; however, an association with CP is not well described.

A 40-year-old previously healthy man presented with progressive dyspnea, fevers, and cough. Initial imaging demonstrated acute pericarditis with subsequent progression to constrictive physiology. Extensive evaluation was unremarkable except for HLA-B27 positivity. He failed medical therapy and underwent radical pericardiectomy with symptom resolution. Pathology confirmed fibrous pericarditis.

This case suggests a potential autoinflammatory role of HLA-B27 in the development of CP. Although HLA-B27 is known to drive chronic inflammation in spondyloarthropathies, and these conditions have established cardiac manifestations, its relationship to pericardial disease remains poorly defined.

In otherwise idiopathic cases of CP, isolated HLA-B27 positivity may represent a potential predisposing factor warranting further investigation.