Patient with Churg Strauss Syndrome and Myocarditis Treated with Cyclophosphamide

doi:10.4236/ojneph.2012.23006

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Open Journal of Nephrology, 2012, 2, 35-37

http://dx.doi.org/10.4236/ojneph.2012.23006 Published Online September 2012 (http://www.SciRP.org/journal/ojneph)

Patient with Churg Strauss Syndrome and Myocarditis

Treated with Cyclophosphamide

Liam Mullen, Janice Harper, Sukumaran Binukrishnan

Royal Liverpool University Hospital, Liverpool Heart an d Chest Hospital, Liverpool, UK

Email: Liam.M[email protected]

Received April 5, 2012; revised May 25, 2012; accepted June 10, 2012

ABSTRACT

Cardiac involvement in Churg Strauss Syndrome is common and a poor prognostic indicator. Myocarditis in Churg

Strauss Syndrome can present in different ways. It has been shown that basic cardiac investigations including echocar-

diography can be normal even in symptomatic patients. More recently cardiac magnetic resonance imaging (MRI) has

been shown to be more sensitive in its diagno sis. Our case report describes a 45 year old male who presented with pal-

pitations and breathlessness. Echocardiography was normal but cardiac MRI demonstrated abnormalities consistent

with Myocarditis. He was treated with Cyclophosphamide and follow up MRI imaging demonstrated complete resolu-

tion of these abnormalities which was accompanied by resolution of symptoms. This case therefore supports the use of

cardiac MRI in Churg Strauss Synd rome as a sensitiv e diag nostic too l and as a means of monito ring respon se to therapy.

It also supports the therapeutic effectiveness of Cyclophosphamide therapy in Churg Strauss related Myocarditis,

something that has yet to be assessed on a large scale.

Keywords: Cardiac MRI; Cyclophosphamide; Myocarditis; Churg Strauss Syndrome

1. Introduction

Cardiac involvement in Churg Strauss Syndrome is

common, with a prevalence of up to 62% [1]. It is the

leading cause of mortality in this disease [2]. The necro-

tising granulomatous Myocarditis associated with Churg

Strauss can manifest in different ways. Clear diagnosis is

difficult without myocardial biopsy. Unlik e patients with

renal disease, patients with cardiac involvement are usu-

ally ANCA (perinuclear type) negative [3,4]. Further-

more traditional cardiac investigations including echo-

cardiography can be normal even in symptomatic pa-

tients. More recently cardiac MRI has been proposed as a

useful diagnostic and monitoring tool in this condition.

Here we present a case of Churg Strauss Myocarditis in

which cardiac MRI was utilised to good effect.

2. Case Report and Methods

A 45 year old man, recently diagnosed with Churg Strauss

Syndrome, presented with a two week history of palpita-

tions, presyn cope and breathlessness on exertio n. He had

been taking Prednisolone since 1999 when he had been

initially diagnosed with chronic eosinophilic pneumonia,

and was currently prescribed 30mg daily.

He was admitted in January 2010 for investigation of

his symptoms. Electrocardiography and serum Troponin

T were normal. A 24 hour tape showed sinus rhythm with

22 isolated ventricular ectopics and 962 episodes of ven-

tricular bigeminy. An echocardiogram demonstrated

normal biventricular size and function, and no significant

abnormalities.

He was incidentally prescribed Myfortic (Mycopheno-

lic Acid) 720 mg twice a day in February 2010, in addi-

tion to Prednisolone, on the basis of persistent sinus

symptoms.

Based on his ongoing cardiac symptoms he subse-

quently underwent cardiac MRI in April. This demon-

strated a mildly reduced left ventricular ejection fraction

(LVEF) of 46%. There was mild hypo kinesia of the basal

septum with late contrast hyper-enhancement; consistent

with Myocarditis (see Figure 1). There was no evidence

of endocardial disease or myocardial hypert r op hy .

Based on the MRI findings he was commenced on

Cyclophosphamide in May and Myfortic therapy was

withdrawn. He received 12 doses (at 15 mg/Kg body

weight) as an intravenous infusion every 2 weeks.

A cardiac MRI was repeated in August after 6 doses of

Cyclophosphamide. This demonstrated clear improve-

ment with a LVEF of 62%. There was very subtle hyper-

enhancement in the basal septum but significantly less

than previ o us ly. He was al so now asymptomati c .

He received his last Cyclophsophamide dose in October

2010 and was then commenced on Azathioprine therapy

at 150 mg a day. A third MRI was pe rfor med two mon th s

L. MULLEN ET AL.

after conversion to maintenance immunosuppression in

December 2010. He remained symptom free at this time.

This demonstrated preserved LV function, with an ejec-

tion fraction of 61%. There was now no evidence of the

previously no ted abnormalities (see Figure 2).

In summary; a 45 year old man with Churg Strauss

syndrome, on long term oral corticosteroid therapy, de-

veloped symptoms of palpitations and breathlessness.

Echocardiography was normal but cardiac MRI demon-

strated mildly impaired LVEF and basal hypokinesia;

consistent with Myocarditis. His symptoms were suc-

cessfully treated with Cyclophosphamide and repeat car-

diac MRI was able to demonstrate resolution of the ab-

normalities.

Figure 1. MRI April 2010. 2D Horizontal Long Axis view

demonstrating hyper-enhancement in basal septum after

gadolinium administration.

Figure 2. MRI December 2010. 2D Horizontal Long Axis

view showing no hyper-enhancement in basal septum after

gadolinium administration.

3. Discussion

Cardiac MRI has been shown to be more sensitive than

basic echocardiography in diagnosing Myocarditis [5].

MRI can demonstrate delayed enhancement in affected

myocardial segments which has been shown to correlate

with histologically proven myocardial inflammation or

fibrosis [6]. One study showed such abnormalities to

occur even in some asymptomatic patients [5]. Cardiac

MRI has also been advocated for monitoring in patients

who receive immunosuppressive treatment [7]. Follow

up cardiac MRI in some of these patients showed resolu-

tion of abnormalities after treatment which correlated

with clinical improvement. Our case report further sup-

ports the use of cardiac MRI as a diagnostic tool. With-

out evidence of myocardial disease on MRI it would

have been difficult to justify the increment in immuno-

suppressive therapy. Cardiac MRI therefore appears to be

very useful in Churg Strauss Syndrome patients present-

ing with cardiac symptoms, particularly when other in-

vestigations are nor mal. This case also supports its use in

monitoring for expected resolution after immunosup-

pressive therapy. The resolution of MRI abnormalities

indicates they were due to myocardial inflammation,

rather than fibrosis. The potential role of cardiac MRI as

a screening tool for cardiac involvement in asymptomatic

patients is less clear and requires further research. None-

theless it could be argued that baseline imaging is useful

given the high lifetime prevalence and mortality of car-

diac disease in Churg Strauss Syndrome. It would allow

for easier diagnosis in the event of the future develop-

ment of symptoms.

This case also supports the therapeutic effectiveness of

Cyclophosphamide in Churg Strauss Myocarditis. To our

knowledge there have, as of yet, been no large scale trials

to support its use. Its prescription is largely on an em-

pirical basis but case reports have shown benefit. One of

the largest studies of its kind to date showed a clinical or

radiological improvement (demonstrated on cardiac MRI)

in 6 of 8 patients with Myocarditis treated with Cyclo-

phosphamide [8]. The patient in our case report showed

radiological and clinical resolution of his Myocarditis

with Cyclophosphamide. He developed cardiac disease

despite long term steroid treatment and his symptoms did

not improve with Myfortic therapy. Our case would

therefore support th e use of Cyclophosphamide in Churg

Strauss Myocarditis above these other therapies.

4. Conclusion

In conclusion, we would advocate the use of cardiac MRI

in Churg Strauss Syndrome Myocarditis; both as a sensi-

tive diagnostic tool and as a means of monitoring re-

sponse to immunosuppressive therapy. Our case also

supports the therapeutic effectiveness of Cyclophos-

L. MULLEN ET AL.

phamide in this condition.

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