From clinic to echocardiography to microscope – the multimodal journey of a rare disease

Abstract

Abstract Introduction Constrictive pericarditis can be a systemic manifestation of immune-mediated diseases. A timely diagnosis followed by ethiologic workup is essential for improving patient prognosis. Case presentation A 67-year-old female patient who suffered multiple episodes of anasarca in the past two years was referred to our center for heart failure with preserved ejection fraction. She was previously investigated for autoimmune diseases based on persistent inflammatory syndrome and recurrent serositis. The physical exam showed systemic congestion, hepatomegaly, and jaundice. Sinus tachycardia and low voltage were observed on the ECG. Transthoracic echocardiography showed septal bounce, “annulus reversus” and “paradoxus”, thickened pericardium without effusion, and a dilated inferior vena cava. Cardiac catheterization was performed, revealing equalization of diastolic pressures and square root sign, confirming constriction. A partial pericardiectomy was performed. The histopathologic study showed polyclonal plasma cells with frequent IgG plasma cells and a IgG4/IgG ratio of 40%, considered a mark of the IgG4 disease as causal. Conclusion Multimodality imaging completed the clinical suspicion of constrictive pericarditis. Complex pathologic analysis of the pericardium led to considering a diagnosis of IgG4 disease, and the patient was referred to a tertiary center for diagnosis and adequate therapeutic management. Constrictive pericarditis can be an associated manifestation of IgG4-related diseases.