Churg-Strauss Syndrome Revealed by Acute Coronary Artery Disease: A Case Report*

doi:10.4236/ojrd.2013.32007

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Open Journal of Respiratory Diseases, 2013, 3, 39-43

http://dx.doi.org/10.4236/ojrd.2013.32007 Published Online May 2013 (http://www.scirp.org/journal/ojrd)

Churg-Strauss Syndrome Revealed by Acute

Coronary Artery Disease: A Case Report*

Maurice Estivals1#, Marc Périé2, Daniel Colombier3, Bruno Farah4

1Department of Pulmonology, Hôpital du Pays d’Autan, Castres, France

2Department of Cardiology, CHT G. Bourret, Nouméa, New Caledonia

3Department of Radiology, Clinique Pasteur, Toulouse, France

4Department of Interventional Cardiology, Clinique Pasteur, Toulouse, France

Email: #[email protected]

Received April 2, 2013; revised May 2, 2013; accepted May 9, 2013

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or

sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is

rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A

52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypi-

cal asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory

distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries

requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment,

unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was es-

tablished considering that five criteria of the American College of Rheumatology were found. Prednisolone was pre-

scribed at 1 mg/kg/day, which co mpletely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two

months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocar-

dium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy.

These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac as-

sessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a

prognosis of CSS.

Keywords: Vasculitis; Churg-Strauss Syndrome; Acute Coronary Artery Disease; Eosinophilia; Cardiac MRI

1. Introduction

According to the Chapel Hill classification, Churg-Strauss

syndrome (CSS) is a necrotizing vasculitis of small-to-

medium-sized vessels. It is a rare disease, with 0.5 - 6.8

million cases per year. Physiologically, it is characterized

by eosinophilia, an inflammatory disease, and by the

presence of neutrophilic p olynuclear anticytop lasmic an-

tibodies in 35% - 50% of patients. Symptoms include

asthma, rhinitis, sinus polyposis and mononeuritis multi-

plex. Cardiac findings are observed in about 50% - 60%

of cases in the form of pericarditis, myocarditis or

ischemic cardiopathy. These play an important role in

prognosis, as they are responsible for 48% of deaths.

Ischemic events are rare, and are rarely suggestive.

2. Case Report

A 52-year-old non-smoker male was admitted to the

Emergency Department in February 2009 with cardio-

genic shock occurring in the context of acute coronary

syndrome (ACS). An electrocardiogram revealed a lower

branch ST elevation with extensive lesion beneath the

anterior endocardium. Prior to this, the patient had suf-

fered from moderate persistent asthma for 10 months and

had been treated with a combination of fluticasone 250

mg/salmeterol (two puffs/day).

Emergency coronary angiography revealed occlusion

of the right coronary artery and the medial left anterior

descending (LAD) artery. Nitrates could not be used lo-

cally due to the initial cardiogenic shock. Stents were

placed on the LAD a rtery. However, iterative obstructions

led to multiple stenting of the LAD and the circumflex

arteries with several resuscitations due to cardiac arrest.

*Conflicts of interests: none to report.

#Corresponding author.

M. ESTIVALS ET AL.

The patient was th en transferred to intensive care with

mechanical ventilation for 48 hours and placement of an

intra-aortic bal loon pum p for three days. He was then sent

to Sydney for a coronary angiography and intracoronary

ultrasound analysis. The right coronary artery was found

to have been initially occluded but was completely cleared

by the local injection of nitrates. No atheroma was found

in the left network, which led to a diagnosis of coronary

artery spasms in a healthy arterial network in a man

without family history of cardiovascular disease.

Peripheral eosinophilia of 2000 polynuclear eosino-

phils was found. Suspected, but unproven, parasitosis was

treated with albendazole.

Three weeks after returning from Australia, the patient

was admitt ed to the Departm ent of Pulm onology due to a n

episode exacerbated asthma. He showed signs of chronic

rhinitis with occasionally purulent rhinorrhea linked to

heartburn-associated hyposmia. He reported a weight l oss

of 7 kg in the past year, although his weight had remained

stable since the coronary event. On pulmonary examina-

tion, he had diffuse wheezing and a prolonged expiratory

phase without stridor. The cardiovascular examination

was normal. ENT examination confirmed a mild rhinor-

rhea associated with posterior discharge. Late-onset

asthma associated with ENT findings, possible gastroe-

sophageal reflux (GER) and peripheral eosinophilia sug-

gested a systemic disease. Laboratory analyses revealed

pronounced peripheral eosinophilia with 16,900 polynu-

clear eosinophils, i.e., 68.6% of a total sample of 24.6 ×

10³ leucocytes.

Chest CT (Figure 1) revealed the presence of lingular

infiltrates. No mediastinal adenopathy, pleural effusion or

pericardial r eaction was found. Sliding hiatus h ernia was

also found.

Facial CT (Figure 2) confirmed sinusitis, mainly af-

fecting the frontal and maxillary areas associated with

chronic sinusitis.

Macroscopic bronchoscopic findings were normal

without vocal chord anomaly but with a marked inflam-

mation of the larynx. Bronchoalveolar lavage (BAL) re-

vealed eosinophilic alveolitis (560 white elements per

mm3 including 49% of eosinophils).

At the bacteriological, virological and mycological

levels, the BAL fluid was sterile.

Histologic examination of bronchial biopsies did not

find abnormal cells but confirmed a marked extravascular

eosinophilia of the sup erficial chorion.

Immunologi cal t ests (C AN, ANC A, rhe umatoi d factors,

quantitation of immunoglobulins, and IgE dosage), para-

sitic serology, aspergillus-specific IgE and allergy tests

were all negative.

Parasitological stool tests yielded negative results three

times.

PHmetry and esogastric fibroscopy confirmed the pre-

Figure 1. Chest CT. Appearance of isolated infiltrate of the

left upper lobe, bilateral bronchial syndrome.

Figure 2. Sinus CT. Appearance of frontal and bilateral

chronic maxillary sinusitis.

sence of GER. The electrocardiogram showed a sinus

rhythm without changes in repolarization.

The echocardiogram performed six weeks after the

initial episode revealed normal left ventricular function

and cardiac rhythm.

CSS diagnosis was established due to the fact that

Lanham’s three criteria were met. In addition, Churg-

Strauss vasculitis could explain the significant coronary

artery disease, in the absence any other cardi ovascular ri sk

factors.

Oral corticosteroid therapy was administered, starting

with a dosage of 1 mg/kg/day of prednisolone, together

with GER treatment and disease modifying inhaled ther-

apy (formoterol 48 µg/day and budesonide 1600 mg/day)

for asthma.

Rapid and co mplete suppression of the cough and ENT

symptoms (rhinorrhea and dysphonia) was observed.

After four weeks, a return to the previous weight was

noted, the asthma remained und er control and the lev el of

M. ESTIVALS ET AL. 41

eosinophils returned to normal.

In April 2009, two months after the initial ACS, a

thorough cardiac checkup wa s performed. The patient was

found to be free of all symptoms of coronary disease, and

the electrocardiogram revealed normal results. The cor-

onal CT showed satisfactory stent permeability without

any sign of restenosis, in addition to a normal coronary

network. No parietal irregularity or calcification was

observed.

On cardiac magnetic resonance imaging (MRI), the left

ventricle (LV) was not dilated (LVEDV: 130 ml, indexed

75 ml/m2) and no thinning of the myocardial wall was

found on T1 sequences. There was no vascular dysfunc-

tion or pericardial discharge or thickening. A clear

subendocardial hypersignal was observed in the medial

septum and the anterior median areas on T2 STIR se-

quences, suggesting an edema.

Finally, diffuse abnormalities of delayed subendocar-

dial enhancement that did not correspond to coronary

systematization of the median anterior, median inferior

and lateral walls were found. These showed at some

points a nodular character, particularly on the inferior-

median wall.

The global and segmental function of the LV was

maintained with an LV ejection fraction (LVEF) of 63%

(minor axis), 55% (four cavities) and 56% (two cavities).

The perfus ion was het erogene ous with a sli ght defect i n

the median septal wall, suggesting microcirculatory issues

(Figure 3). It was concluded that the T2 sequence

anomalies and delayed enhancement were compatible

with myocardial injury within the scope of vasculitis and

probably associated with sub-endocardial fibrosis (Fig-

ures 4 and 5). LV function was maintained with LVEF at

around 60%.

Considering the myocardial injury observed on the

MRI, a decision was made to start immunosuppressive

treatment in association with corticosteroid therapy (aza-

thioprine 2 mg/kg/d ay).

3. Discussion

CSS is characterized by necrotizing vasculitis, which

affects both small and medium-sized blood vessels at the

pulmonary and systemic levels, and is associated with

intra- and extravascular granuloma, eosinophilia, and

infiltration of eosinophils at the tissue level in asthmatic

patients who often suffer from rhinitis or chronic sinusi-

tis [1]. It is a rare condition (0.5 to 6.8/106 per year). The

diagnostic criteria were established by Lanham [2] at the

Hammersmith Hospital in London.

Here, the diagnosis of CSS was established because

three of the criteria of this classification with a sensitivity

and specificity of 95% were found [2]. It should be noted

that although ENT and asthmatic symptoms were present,

the allergy results were negative and IgE levels were

Figure 3. Cardiac MRI. Short axis perfusion. First-pass per-

fusion sequence after bolus intravenous injection of Gado-

linium. Hyposignal in the anterior-septal wall suggesting

microcirculatory issues.

Figure 4. Cardiac MRI. DES 2 cav. 2. Delayed enhancement

sequence 10 min after intravenous injection of Gadolinium in

slices, two cavities long axis. Subendocardial contrast en-

hancement in two distinct areas: anterior-median and infe-

rior-median. A pseudo-nodular enhancement can be ob-

served in the latter.

normal. Asthma was moderated with normal respiratory

function under disease modifying treatment; it was never

corticodependent and was not treated with leukotriene

inhibitors. Other possible diagnoses were eliminated (Car-

rington’s disease, Loeffler’s syndrome, allergic broncho-

pulmonary aspergillosis, Wegener’s granu lomatosis, Ho dg -

kin’s lymphoma and drug-induced eosinophilic pneumo-

nia).

We did not consider here the po ssibility that treatment

with inhaled substances could have caused CSS [3], as

peripheral eosinophilia was not available at treatment

onset.

M. ESTIVALS ET AL.

Figure 5 . Card iac MRI. T2 ST IR sho rt axis . Weighted “black

blood” T2 sequence with fat saturation. Subendocardial hy-

persigna l in t he an terior-s epta l and infer ior a re as s ugges ting

myocardial edema.

Our patient had an ANCA-negative phenotype, and

showed evidence of pulmonary infiltrates on CT images,

but no pleural effusion; bronchial biopsies revealed

marked extravascular eosinophilia on the superficial

chorion. According to the French Vascu litis Study Group

criteria [4], he was at risk of suffering a cardiac event.

Cardiac findings are present in about 50% of cases of

CSS [1,5] and are responsible for almost half of CSS

deaths [6]; they are sometimes difficult to identify. The

most frequent cardiac symptoms are pericarditis and myo-

carditis. Vascular problems and heart failure due to LV

dysfunction have also been described [7-10]. Several

studies have confirmed the benefits of cardiac MRI in the

early diagnosis of cardiac disease [5,11,12], while other

diagnostic techniques do not yield useful results.

Cardiac localizations are more frequent in the ANCA-

negative phenotype [4,5,12] as was the case in our ob-

servation. In the series published by Neumann et al. [5],

22 out of 49 patients showed clinical signs of cardiac

disease, ANCA were often negative and elevated eosi-

nophilia was often found. A total of 50% of patients had

LV dysfunction, 73% had vascular leakages and 41% had

pericarditis. Endomyocardial lesions were detected by

MRI in 59% of patients who then had a less favorable

diagnosis.

LV function was initially normal apart from the acute

episode, and there was no vascular injury or pericardial

effusion. The coronary angiography revealed a coronary

network free from atheroma and restenosis, and perme-

able stents. There were no unfavorable symptom and

initial treatment solely based on corticosteroid therapy

seemed justified, as in the case reported by Wagner et al.

[7].

The detection of cardiac injury by MRI had an impact

on treatment, as cardiomyopathy plays a role in four of

five factors that determine poor prognosis for CSS,

namely: age over 65 years, severe gastrointestinal injury,

renal failure with elevated serum creatinine levels (>150

µm/L), and heart disease [5,12-14].

However, due to the low number of cases describe d, it

seems premature to intensify CSS treatment based solely

on MRI results from asymptomatic patients [12].

Here, the patient had a favorable prognosis in terms of

ENT involvement, but a poor prognosis in terms of heart

disease [14]. Therefore, immunosuppressive treatment

was started.

Acute cor ona ry event s have been poo rly repo rted in t he

literature [7,9,10,15]. They rarely suggest CSS and no

case of multiple stenting cou ld be found in the literature.

The mechanisms mentioned are coronary spasms, which

are possi bly favo red more by the to xicity of eosinophi ls or

by myocardial granulomas than by the localization of

vasculitis at the level of the coronary arteries [15,16]. In

the case reported by Wagner et al., the patient’s history

suggested undiagnosed CSS. His phenotype was ANCA

negative, with very h igh initial eosinophilia Tanaka et al.

[12] reported a case of ACS due to a vasospasm with

significant eosinophilia (>5000/mm3) and ANCA-nega-

tive phenotype [15]. The authors suggested a link between

coronary vasospasm due to CSS and eosinophilia. It is

interesting to note that in the case reported here, eosino-

philia was not found during the ACS. Significant eosi-

nophilia was only found a month after the event (Figure

6).

What, then, is the mechanism of vasospasm?

The overall prognosis for patients with CSS remains

favorable (79% alive at 10 years) [13]. Heart disease is

Figure 6. Level of eosinophils in time.

M. ESTIVALS ET AL.

responsible for almost 50% of deaths [6], so intensive

initial treatment is recommended.

Two years after initial diagnosis, the patient remains

treated with azathioprine and small doses of corticoster-

oids (5 mg/day of prednisone). There has been no relapse

at the cardiovascular level, but respiratory function re-

mains below normal (LVEF = 3.4 liters at 100% of theo-

retical values, Tiffeneau = 73%, and mean peak expira-

tory flow 25/75 at 2.73 liters so 73% of theoretical values).

The level of eosinophils was at 6 80/mm3.

In conclusion, CSS can be accompanied and revealed

by ACS. The mechanism of ACS in the healthy coronary

network is a vasospasm that seems independent of the

coronary localization of the vasculitis, although its origins

remain uncertain. In such cases, cardiac MRI should be

systematically performed to identify myocardial injuries

that can be useful for the prognosis. This will ensure early

start of immunosuppressive therapy.

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