Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg−Strauss syndrome): a case report and review of the literature

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome, is a necrotizing systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Cardiovascular complications, including eosinophilic myocarditis, are a major cause of mortality in this disorder. Acute pericarditis with slight pericardial effusion is a typical manifestation in EGPA, though hemodynamically significant pericardial effusion has been reported in a few cases. We report a case that initially presented with isolated cardiac tamponade, which was followed by systemic manifestations of EGPA over 3 weeks. Including the present case, previous EGPA cases with cardiac tamponade are reviewed to delineate its clinical characteristics.

An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report

“…Three of those studies were also able to confirm eosinophilic infiltration in the pericardium with biopsy, as we have demonstrated (Fig. 1e ) [ 8 ]. Since the cause of EGPA is unknown and is difficult to study due to low disease prevalence (less than 1.4 per 100,000 adults), unusual case presentations like ours are an important step in helping to elucidate how different clinical phenotypes correlate with disease pathophysiology [ 3 ].…”

“…Very few cases of orbital inflammatory pseudotumors associated with EGPA have ever been described and may only present in less than 1% of all patients with the disease [ 4 – 6 ]. In addition, her cardiac tamponade was another unusual clinical presentation, reported in only ten other cases of EGPA [ 3 , 7 , 8 ]. Three of those studies were also able to confirm eosinophilic infiltration in the pericardium with biopsy, as we have demonstrated (Fig.…”

A case report and literature review of Churg–Strauss syndrome presenting with myocarditis

“…The mean age at diagnosis is 48 years, [14] and both sexes are affected equally. We have little data on race distribution.…”

Myopericarditis as a presentation of eosinophilic granulomatosus with polyangiitis (EGPA)

“…Death due to myocarditis alone is highly unusual, which prompted a postmortem investigation in this case, highlighting the importance of further investigation for a secondary cause in patients presenting systemically unwell with symptoms refractory to standard therapy. A second group detailed a case of cardiac tamponade leading to a new diagnosis of EGPA, even rarer than myopericarditis, and indicative of increased severity of disease 15. In all cases, the increasingly recognised role for CMRI is highlighted7 16 although this does not form part of the official guidelines for the management of EGPA, and more extensive studies are certainly warranted for its use in this setting, especially given the paucity of data, mostly derived from individual case reports.…”