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
Received April 5, 2012; revised May 25, 2012; accepted June 10, 2012
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
Cardiac involvement in Churg Strauss Syndrome is
common, with a prevalence of up to 62% . It is the
leading cause of mortality in this disease . 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
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
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
opyright © 2012 SciRes. OJNeph
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-
Figure 1. MRI April 2010. 2D Horizontal Long Axis view
demonstrating hyper-enhancement in basal septum after
Figure 2. MRI December 2010. 2D Horizontal Long Axis
view showing no hyper-enhancement in basal septum after
Cardiac MRI has been shown to be more sensitive than
basic echocardiography in diagnosing Myocarditis .
MRI can demonstrate delayed enhancement in affected
myocardial segments which has been shown to correlate
with histologically proven myocardial inflammation or
fibrosis . One study showed such abnormalities to
occur even in some asymptomatic patients . Cardiac
MRI has also been advocated for monitoring in patients
who receive immunosuppressive treatment . 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 . 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.
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-
Copyright © 2012 SciRes. OJNeph
L. MULLEN ET AL.
Copyright © 2012 SciRes. OJNeph
phamide in this condition.
 R. M. Dennert, et al., “Cardiac Involvement in Churg-
Strauss Syndrome,” Arthritis and Rheumatism, Vol. 62,
No. 2, 2010, pp. 627-634.
 G. Pela, G. Tirabassi, P. Pattoneri, L. Pavone, G. Garini
and G. Bruschi, “Cardiac Involvement in the Churg-
Strauss Syndrome,” American Journal of Cardiology, Vol.
97, No. 10, 2006, pp. 1519-1524.
 J. Vinit, et al., “Heart Involvement in Churg-Strauss Syn-
drome: Retrospective Study in French Burgundy Popula-
tion in Past 10 years,” European Journal of Internal
Medicine, Vol. 21, No. 4, 2010, pp. 341-346.
 R. Sablé-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L.
Mouthon, D. Jayne, D. Blockmans, J. F. Cordier, P. De-
laval, X. Puechal, D. Lauque, J. F. Viallard, A. Zoulim
and L. Guillevin, “The French Vasculitis Study Group.
Antineutrophil Cytoplasmic Antibodies and the Churg-
Strauss Syndrome,” Annals of Internal Medicine, Vol.
143, No. 9, 2005, pp. 632-638.
 J. Marmursztejn, O. Vignaux, P. Cohen, P. Guilpain, C.
Pagnoux, H. Gouya, L. Mouthon, P. Legmann, D. Duboc
and L. Guillevin, “Impact of Cardiac Magnetic Reso-
nance Imaging for Assessment of Churg-Strauss Syn-
drome: A Cross-Sectional Study in 20 Patients,” Clinical
and Experimental Rheumatology, Vol. 27, Suppl. 52,
2009, pp. S70-S76.
 H. Mahrholdt, C. Goedecke, A. Wagner, et al., “Cardio-
vascular Magnetic Resonance Assessment of Human
Myocarditis: A Comparison to Histology and Molecular
Pathology,” Circulation, Vol. 109, No. 10, 2004, pp.
 H. Baccouche, A. Yilmaz, D. Alscher, K. Klingel, J. F.
Val-Bernal and H. Mahrholdt, “Images in Cardiovascular
Medicine. Magnetic Resonance Assessment and Therapy
Monitoring of Cardiac Involvement in Churg-Strauss
Syndrome,” Circulation, Vol. 117, No. 13, 2008, pp.
 J. Marmursztejn, P. Cohen, D. Duboc, C. Pagnoux, L.
Mouthon, P. Guilpain, P. Legmann, L. Guillevin and O.
Vignaux, “Cardiac Magnetic Resonance Imaging in Ch urg -
Strauss-Syndrome. Impact of Immunosuppressants on
Outcome Assessed in a Prospective Study on 8 Patients,”
Clinical and Experimental Rheumatology, Vol. 28, Suppl.
57, 2010, pp. 8-13.