ANCA in CV-EGPA

Q: patients with cardiovascular (CV) involvement in eosinophilic granulomatosis with polyangiitis (EGPA)(Churg-Strauss) are prone to have? (select one)

A) negative ANCA

B) positive ANCA

Answer: A

Although lungs and skin tend to be more involved in EGPA, half of the mortality comes from CV involvement. One unique characteristic of CV involvement in EGPA is a tendency to have less likely positive antineutrophil cytoplasmic antibody (ANCA) but relatively a higher peripheral blood eosinophil count. CV involvement may manifest as heart failure, pericarditis, or rhythm issues. 

Interestingly, CV patients in EGPA have a shorter duration of EGPA-associated symptoms, probably due to vital hemodynamic organ involvement.

#rheumatology

#cardiology

References: 

1. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009; 88:236.

2. Dennert RM, van Paassen P, Schalla S, et al. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum 2010; 62:627.

3. Szczeklik W, Miszalski-Jamka T, Mastalerz L, et al. Multimodality assessment of cardiac involvement in Churg-Strauss syndrome patients in clinical remission. Circ J 2011; 75:649.