Korean Circ J.  2014 Nov;44(6):437-440. 10.4070/kcj.2014.44.6.437.

Endomyocardial Biopsy and Magnetic Resonance Imaging of Acute Myocarditis with Adult-Onset Still's Disease

• Yamazoe M
• Mizuno A
• Suyama Y
• Nishi Y
• Suzuki K
• Niwa K
• Okada M

• KMID: 2223862
• DOI: http://doi.org/10.4070/kcj.2014.44.6.437

Abstract

A 36-year-old female with a high-grade fever and epigastric abdominal pain was prescribed antibiotics, but developed hypoxia and dyspnea. An echocardiography revealed diffuse hypokinesis and massive pericardial effusion, after which diagnostic cardiac catheterization and an endomyocardial biopsy (EMB) were peformed to reveal fibrosis and infiltration of inflammation cells composed primarily of neutrophils. Clinical manifestation of a spiking fever, leukocytosis, elevated ferritin levels, skin rash and EMB findings led to a diagnosis of adult-onset Still's disease (AOSD) with acute myocarditis. Pulse therapy of intravenous methylprednisolone was performed for three days, followed by a daily dose of prednisone (60 mg). After a course of steroid therapy for fever and pericardial effusion, and conducting a left ventricular ejection fraction, the patient showed improvement and was discharged asymptomatic within 32 days of admission. This study is the first to report on a case of myocarditis in AOSD diagnosed by neutrophil infiltration in the myocardium.

Keyword

Myocarditis; Still disease, adult-onset; Magnetic resonance imaging; Heart failure

MeSH Terms

Abdominal Pain
Adult
Anoxia
Anti-Bacterial Agents
Biopsy*
Cardiac Catheterization
Cardiac Catheters
Diagnosis
Dyspnea
Echocardiography
Exanthema
Female
Ferritins
Fever
Fibrosis
Heart Failure
Humans
Inflammation
Leukocytosis
Magnetic Resonance Imaging*
Methylprednisolone
Myocarditis*
Myocardium
Neutrophil Infiltration
Neutrophils
Pericardial Effusion
Prednisone
Still's Disease, Adult-Onset*
Stroke Volume
Anti-Bacterial Agents
Ferritins
Methylprednisolone
Prednisone

Figure

• 

  Fig. 1 A: erythema on the right forearm (white arrow). B: erythema on the abdomen (white arrow). C: echocardiography showed pericardial effusion (white arrow) before steroid treatment (LVEF 20%, LVDd 50.3 mm, LVDs 42.9 mm, IVST 8.6 mm). D: echocardiography showed complete disappearance of pericardial effusion after steroid treatment (LVEF 65.4%, LVDd 44.4 mm, LVDs 28.1 mm, IVST 9.1 mm). LVEF: left ventricular ejection fraction, LVDd: left ventricular end-diastolic dimension, LVDs: left ventricular end-systolic dimension, IVST: interventricular septal thickness.

• 

  Fig. 2 A: endomyocardial biopsy in hematoxylin and eosin stain ×10 revealed diffuse infiltration of inflammation cells. B: endomyocardial biopsy in hematoxylin and eosin stain ×20. Neutrophils (white arrow) and a few eosinophils (white arrowhead) infiltrated the cardiac muscle. C: endomyocardial biopsy in Masson's stain ×10. Cardiac muscle was partially replaced by fibrosis (white arrow). D: medial layer of cardiac muscle was delayed-enhanced in gadolinium cardiac-magnetic resonance imaging (white arrow).

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