New development of cardiac tamponade on underlying effusive–constrictive pericarditis: an uncommon initial presentation of scleroderma

Abstract

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.

Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature

“…The flow diagram of the bibliography search and the reason for exclusion are depicted in Figure and appendix. Finally, 22 articles corresponding to 31 SSc patients were included. Of note, in all cases echocardiographic study was performed.…”

Fatal Cardiac Tamponade in Newly Diagnosed Systemic Sclerosis with Early Cutaneous Disease: A Case Report

“…The finding of mild chronic pericardial effusion is fairly common in SSc and patients are rarely symptomatic for it. The development of cardiac tamponade in these patients is even rarer [6]. Therefore, a previously undiagnosed patient with early cutaneous changes of SSc who develops cardiac tamponade constitutes a potential complexity of diagnostic considerations.…”

“…Thus patients may be prone to cardiac tamponade before the accumulation of large volumes of pericardial effusion. This subnormal distensibility of the pericardium probably predisposes SSc with pericardial effusions to a higher likelihood of having a tamponade [6]. It is therefore for this reason that Langley et al recommended looking out for the onset of a tamponade and being prepared to treat it especially if there is the occurrence of a sudden cardiovascular collapse [5].…”

Cardiovascular complications of systemic sclerosis: What to look for

“…Large pericardial effusions can develop before skin thickening and diagnosis of systemic sclerosis. 81,83,84 Thus, systemic sclerosis should be considered in patients with pericardial effusions of unknown etiology.…”