Key result
Scleroderma rarely manifests initially as fatal cardiac tamponade and effusive-constrictive pericarditis.
Case Report (n=1)
Cardiac tamponade with effusive-constrictive pericarditis can be a rare, fatal initial presentation of scleroderma.
Rare tamponade in scleroderma warrants clinical consideration; single case leaves open prevalence and management data.
A 40-year-old man with a medical history of hypertension was admitted for weight loss, generalised weakness, joint pains and mottling of fingertips. The initial laboratory data revealed microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Intravenous steroids were started for possible diagnosis of systemic lupus erythematosus based on admission assessment. Intravenous immunoglobulin and plasmapharesis were subsequently added to the treatment plan to cover thrombotic thrombocytopenic purpura while his autoimmune panel was pending. The echocardiogram study on day 2 revealed cardiac tamponade for which he underwent pericardiocentesis and right heart catheterisation. The atrial waveforms postpericardiocentesis demonstrated effusive-constrictive pericarditis. His clinical condition kept on deteriorating with reaccumulation of pericardial effusion and further complicated by hemoperitoneum and colonic obstruction. He had cardiorespiratory arrest on his fourth admission day and was not revived. Anti-Scl-70 antibody came back positive. Autopsy findings confirmed the presence of fibrinous pericarditis and hemoperitoneum.
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Subramanian et al. (2013) conducted a case report in Scleroderma with cardiac tamponade and effusive-constrictive pericarditis (n=1). A 40-year-old man presented with cardiac tamponade and effusive-constrictive pericarditis as an uncommon initial manifestation of scleroderma, ultimately resulting in fatal cardiorespiratory arrest.
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