Abstract
A 44-year-old woman with a history of treatment-refractory scleromyxoedema presented with clinical heart failure along with dyspnoea and orthopnoea. Her previous transthoracic echo (TTE) 3 months prior showed normal left ventricular (LV) and right ventricular (RV) function with a stable pleural effusion. She had a raised N-terminal pro-B-type natriuretic peptide and creatinine kinase of 773 pmol/L and 4534 U/L, respectively. Her TTE showed new biventricular dysfunction, with diffuse hypokinesis and a LV ejection fraction (EF) of 20–25%. She underwent a coronary angiogram with right heart studies, which ruled out ischaemic cardiomyopathy or pulmonary hypertension. A cardiac magnetic resonance imaging (cMRI) confirmed biventricular failure (LVEF 37%; RVEF 28%) with the known stable pleural effusion (Figure 1A ). Late gadolinium-enhanced images on cMRI showed diffuse patchy enhancement consistent with infiltrative disease. An endomyocardial biopsy showed myocarditis with interstitial fibrosis. There was polymorphous inflammatory infiltrate with accompanying myocyte damage. Interstitial mucin deposition was later confirmed with a colloidal iron stain, a hallmark of scleromyxedema. A thigh MRI and biopsy showed a necrotising myositis, confirming systemic disease. She responded to high-dose prednisolone, thalidomide, and intravenous immunoglobulin. Her follow up TTE showed stable LV function and improved RV function.
Published Version
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