A textured clinical mosaic involving autoimmune calcific constrictive pericarditis: a case report.

Background: Constrictive pericarditis is a rare condition whereby chronic pericardial inflammation leads to pericardial stiffening and predominantly right-sided heart failure. While idiopathic and infectious forms are most common, autoimmune causes may be involved with often elusive disease manifestations.

Case summary: A 34-year-old woman presented with severe right-sided heart failure and atrial fibrillation secondary to rapidly progressive calcific constrictive pericarditis following preterm delivery due to placenta previa. Given the refractoriness to medical therapy and dependence on i.v. diuretic therapy, surgical pericardiectomy was performed without any clinical benefit (prompt heart failure relapse). Further tests revealed a mosaic of multiorgan manifestations (alveolar haemorrhage, pleuritis, factor XI deficiency, altered lymphocyte subpopulations, cutaneous nodules demonstrating Koebner's phenomenon, placental vasculopathy causing miscarriage and preterm delivery), which ultimately led to the diagnosis of systemic lupus erythematosus (SLE). Instatement of immunosuppression (corticosteroid initially, subsequently substituted by mycophenolate) led to the resolution of constriction and other disease manifestations at 18-month follow-up.

Discussion: This case of SLE presenting with advanced calcific constrictive pericarditis demonstrates the crucial role played by aetiological diagnosis in constrictive pericarditis. It also exemplifies the often-subtle nature of extra-cardiac manifestations in autoimmune processes, particularly in SLE, which may lead to significant diagnostic uncertainty and delay in the instatement of disease-specific therapy. The benefits of the latter are manifest in this case as the introduction of mycophenolate determined a complete reversal of constrictive physiology despite refractoriness to surgical pericardiectomy.