European Annals of Allergy and Clinical Immunology THE OFFICIAL JOURNAL OF AAITO THE OFFICIAL JOURNAL OF SPAIC HOME PAGE

EDITORS IN CHIEF
L. Cecchi (Firenze - Italy)

M. Morais - Almeida (Lisbon - Portugal)

 

HONORARY EDITOR
A. Sabbah (Angers - France)

 

ASSOCIATE EDITORS
A. Tedeschi (Milano - Italy)

R. Asero (Milano - Italy)

 

EDITORIAL BOARD
M.B. Bilò (Ancona - Italy)
F. Bonifazi (Ancona - Italy)

L.M. Borrego (Lisbon - Portugal)
K. Brockow (München - Germany)
A.À. Cruz (Salvador - Brasil)
L. Delgado (Oporto - Portugal)
P. Demoly (Montpellier - France)
G. D'Amato (Napoli - Italy)
M. Drouet (Angers - France)
M. Fernandez-Rivas (Madrid - Spain)
A. Fiocchi (Milano - Italy)

J. Fonseca (Oporto - Portugal)
F. Mastrandrea (Taranto - Italy)
M. Maurer (Berlin - Germany)
G. Moscato (Pavia - Italy)
A. Musarra (Reggio Calabria - Italy)
C. Nunes (Portimao - Portugal)
M. Olivieri (Verona - Italy)
P. Parronchi (Firenze - Italy)
G. Passalacqua (Genova - Italy)
G. Pauli (Strasbourg - France)
A. Perino (Torino - Italy)
L.K. Poulsen (Copenaghen - Denmark)
O. Quercia (Faenza - Italy)
A. Romano (Roma - Italy)
D. Solé (Sao Paulo - Brasil)
A. Todo Bom (Coimbra - Portugal)
S. Voltolini (Genova - Italy)

 

SCIENTIFIC COMMITTEE
L. Antonicelli (Italy)
A. Bener (Turkey)
H. Bazin (Belgium)
J. Bellanti (USA)
C. Geller-Bernstein (Israel)
M. Cugno (Italy)
B. David (France)
S. Durham (UK)
G.P. Girolomoni (Italy)
R. Jarish (Austria)
S.G.O. Johansson (Sweden)
F. Levi-Shaffer (Israel)
H. Lowenstein (Denmark)
J.L. Malo (Canada)
A.G. Palma-Carlos (Portugal)
G. Scadding (UK)
G. Scadding (UK)
E. Stevens (Belgium)
R. van Ree (Netherlands)

 

FOUNDER AND CORRESPONDING MEMBER
G.M. Halpern (USA)

Table of Contents »

Acute cardiac disease in a patient with hyper-IgE syndrome


A. Castilano1, H. Watti2, A. Abdulbaki2, K. Modi2, J.A. Bocchini Jr3, S.L. Bahna1 sbahna@lsuhsc.edu

Show more: Authors information and Publication history

Doi
https://doi.org/10.23822/EurAnnACI.1764-1489.13

Summary
We describe the case of a 24-year-old male with hyper-IgE syndrome (HIES) which was diagnosed at 4 years of age and died from a very rare cardiac complication. He had typical clinical and laboratory manifestations of HIES, including total serum IgE as high as > 100,000 IU/mL. Stem cell transplantation was not available. During the 20-year follow-up, he suffered numerous various infections of the skin and deep organs, partial lung resection, as well as multiple bone fractures. At age 24, he developed acute decompensated heart failure associated with elevated serum troponin I and brain natriuretic protein. Two-dimensional echocardiogram revealed global hypokinesis of the left ventricle with estimated ejection fraction 20-25%, and catheterization revealed ectasia of multiple coronary arteries. Endomyocardial biopsy showed lymphocytic myocarditis, focal necrosis, mild fibrosis, and myxoid degeneration, but cultures were negative. The patient improved on corticosteroid therapy and was discharged on heart failure therapy and external defibrillator. Six weeks later, he developed supraventricular tachycardia and persistent global hypokinesis and was treated with amiodarone. A trial of intravenous immunoglobulin was initiated and was repeated as outpatient every four weeks for four times. However, his cardiac function did not improve and he developed severe hypotension and pulseless electrical activity arrest. Resuscitation was unsuccessful. To the best of our knowledge, this is the first reported case of HIES complicated with lymphocytic myocarditis. Both immunologists and cardiologists need to be aware of such a complication and practice caution in using immunosuppressants when the patient's immune status is markedly compromised.


FULL TEXT
Cite this article as:
Castilano A et al. Acute cardiac disease in a patient with hyper-IgE syndrome. Eur Ann Allergy Clin Immunol. 2017;49(5):231-234. doi:10.23822/EurAnnACI.1764-1489.13