Download Acute Congestive Heart Failure Secondary to an Unusual

Case Report
Acute Congestive Heart Failure Secondary to an
Unusual Underlying Cause
V Ravindran*, JD Barman**, R Hughes***
Abstract
We report a patient who presented with non-specific features and rapidly developed multisystem disease as
a result of Chrug-Strauss syndrome, a rare diffuse primary vasculitis. This case report highlights the importance
of considering primary vasculitides as a differential diagnosis in patients presenting with multiple organ
involvement as early specific therapy in such cases has shown to change the outcome. ©
I
INTRODUCTION
n patients presenting with multisystem disease,
vasculitis should always enter into the differential
diagnosis. Whilst blood tests may be helpful in reaching
a diagnosis, the importance of new and apparently
specific tests such as anti-neutrophil cytoplasmic
antibodies can be overestimated at the expense of
traditional assessments such as a good history and
comprehensive clinical examination and simple tests
such as a blood count. We report a patient who presented
with non-specific features and rapidly developed
multisystem disease and severe congestive heart failure
refractory to conventional treatment. Diagnosis of
primary vasculitis in this case led to definitive treatment
with excellent recovery.
CASE REPORT
A 61-year man was admitted initially under the
surgeons with three-week history of intermittent upper
abdominal pain, nausea and anorexia. Five years ago
he was diagnosed to have late onset asthma and over
the past three years had recurrent episodes of nasal
trouble diagnosed as sinus inflammation by ENT
surgeons. Eight weeks prior to admission his peak flow
dropped what he felt was an exacerbation of asthma
and was prescribed a 5-day course of 30 mg
Prednisolone. On examination he was noted to have
epigastric and right upper quadrant tenderness. Apart
from normocytic normochromic anaemia (Hb 11.3g/dL),
leukocytosis with eosinophilia (Eosinophils 4.6 x 109 /
L, 36 % of total leukocyte count) and elevated CRP (49
mg/L) his initial blood tests were normal. Chest X-ray
*Specialist Registrar, ** Senior House Officer, ***Consultant,
Department of Rheumatology, St Peter’s Hospital, Chertsey,
UK KT16 0PZ.
Received : 16.2.2006; Accepted : 7.7.2006
© JAPI • VOL. 54 • AUGUST 2006
(CXR) demonstrated non-specific shadowing in upper
zones. Abdominal ultrasound and CT scan
demonstrated bilateral pleural effusions and a small
pericardial effusion. One week after admission he had
developed persistent swinging pyrexia (range 38-39°C)
and was commenced on broad-spectrum antibiotics for
presumed atypical pneumonia.
After three days he became very dyspnoeic and
developed significant peripheral oedema with a raised
JVP and widespread lung crepitations. ECG
demonstrated non-specific ST-T changes; CXR
demonstrated cardiomegaly and widespread ground
glass shadowing and both Troponin-I (1.29mg,normal
<0.10 mg) and B-type natriuretic peptide (286 ng/L, in
heart failure >100 ng/L) were elevated. He was
commenced on intravenous diuretic and nitrate therapy
along with low molecular weight heparin and fluid
restriction with a diagnosis of severe congestive heart
failure (CHF) secondary to an acute coronary event. After
4 days symptoms related to CHF had improved only
marginally (at this stage an echocardiogram
demonstrated pericardial effusion and left ventricular
ejection fraction reduced at 42%). His pyrexia remained
unabated with persistently raised inflammatory markers
and marked eosinophilia. His repeated blood and urine
cultures did not grow any organism and atypical
pneumonia screen was negative.
He then developed palpable purpura spread over his
thighs and buttocks and this prompted a
rheumatological referral. A unifying diagnosis of ChurgStrauss syndrome (CSS) was made based on Lanham’s
criteria (asthma, peak peripheral eosinophil count >1.5x
109 /L and systemic vasculitis involving two or more
extrapulmonary organs) and the patient was given three
pulses of 1gram intravenous methyl prednisolone and
was commenced on oral prednisolone 40mg per day.
Within 48 hrs he felt significantly better symptomatically
www.japi.org
659
and became apyrexial. By 4th day both his inflammatory
markers and eosinophil count had settled down to the
normal ranges and he was well enough to be discharged
home. Follow up has shown that he has remained well
since with a decreasing dose of steroid.
vasculature in acute phases may demonstrate multiple
vessel wall irregularities, abrupt terminations and small
aneurysms. Delay in recognising the cardiac
involvement probably accounts for the high cardiac
mortality in CSS.2,3
DISCUSSION
Whilst eosinophilia is a diagnostic marker,
antineutrophil cytoplasmic antibodies (ANCA) are
present in only about half of patients, with a perinuclear
staining and the demonstration of anti-myeloperoxidase
antibodies. 4 CSS often responds rapidly to
glucocorticoids alone.5 Cyclophosphamide may be used
as an adjunct therapy in patients who have substantial
vasculitic end organ involvement and in those who have
not proven responsive to the glucocorticoids.6
We draw the attention of physicians providing acute
medical care to the continuing importance of vasculitis
in the differential diagnosis of multi-system disease.
This man with abdominal pain had a clear history of
late onset asthma and paranasal sinus symptoms, had
eosinophilia and developed lung involvement, vasculitic
rash and heart failure refractory to conventional
treatment.
The 1994 Chapel Hill consensus conference defined
Churg-Strauss syndrome as an “eosinophil-rich
granulomatous inflammation of the respiratory tract,
necrotizing vasculitis affecting small to medium sized
vessels and associated with asthma and eosinophilia”.1
Diagnosis is based mainly on clinical grounds since
eosinophilic granulomas are not always present on
biopsy even in florid cases.2 Lanham’s clinical criteria
are widely used, and he described three phases of the
condition: firstly, an allergic rhinitis, often associated
with recurrent sinusitis and asthma, that becomes
progressively more difficult to treat; secondly, peripheral
blood eosinophilia associated with eosinophilic
pulmonary infiltrates and worsening asthma; and
thirdly, systemic vasculitis, commonly including
peripheral neuropathies and occasionally life
threatening cardiac and severe gastrointestinal disease.2
The spectrum of cardiac involvement ranges from
pericarditis and pericardial effusion and to more serious
cardiac tamponade, CHF caused by an eosinophilic
myocarditis and coronary vessel vasculitis.3 In our
patient abdominal pain and elevated Troponin-I can be
explained by mesenteric vasculitis and coronary vessel
vasculitis respectively. Angiography of relevant
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Announcement
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Also visit our website at www.mdrf-ada.com or www.drmohansdiabetes.com
fordetails regarding registration etc.
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© JAPI • VOL. 54 • AUGUST 2006