Case 1
A previously well 58 year old woman with an unremarkable medical history
was seen at the respiratory clinic with a six month history of a dry
cough, easy fatigability and progressive breathlessness on exertion. She
was unaware of any precipitants and had received numerous antibiotic
courses, salbutamol and budesonide inhalers, and prednisolone with mild
improvement of her symptoms. Physical examination including chest
auscultation was normal except for a regular tachycardia of 106
beats/min. Chest x – ray (CXR) revealed a bilateral hilar
lymphadenopathy and cardiomegaly. A contrast – enhanced computed
tomography (CT) scan of the chest showed bilateral reticulonodular
opacities with bilateral hilar lymphadenopathy suggestive of stage II
pulmonary sarcoidosis. An electrocardiogram (ECG) showed sinus
tachycardia with a left bundle branch block (Figure 1) and a
transthoracic echocardiogram revealed a dilated left ventricle with
severely reduced LV systolic function (ejection fraction of 30%) and
dyssynchrony of the interventricular septum (IVS). Laboratory
investigations showed elevated serum angiotensin converting enzyme (ACE)
of 69.6 UI/L (8.0 – 52.0), erythrocyte sedimentation rate (ESR) of 33
mmfall/hr (3.0 – 5.0) and hypercalcaemia of 2.79 mmol/L (2.12 – 2.62).
ANA was negative and sputum for GeneXpert (GeneXpert Dx System Version
4.8) did not detect Mycobacterium tuberculosis DNA. Lung function tests
revealed a restrictive pattern with a forced expiratory volume in the
first second (FEV1) of 1.44L (predicted, 2.18L), a forced vital capacity
(FVC) of 1.88L (predicted, 2.51L) and a FEV1/FVC ratio of 77%
(predicted, 87%). A diagnosis of pulmonary sarcoidosis was made with
possible cardiac involvement and steroid therapy initiated with good
improvement of symptoms within 3 months. The patient developed symptoms
of heart failure (orthopnoea, paroxysmal nocturnal dyspnoea, bilateral
leg swelling) 6 months later when her steroid dose was being tapered.
She was admitted and managed for heart failure (NYHA Class III). Cardiac
MRI done later showed a moderately dilated LV with moderately reduced
ejection fraction (EF) of 40%, discoordinate movement of
interventricular septum and a thinned and hypokinetic apical anterior
wall. There was no active myocardial inflammation or oedema but there
was midwall late gadolinium enhancement of the basal inferior LV wall,
the basal lateral LV wall with patchy near-transmural enhancement of the
apical anterior and mid to apical lateral LV wall (Figure 2) all
suggestive of cardiac sarcoid. The prednisolone dose was increased to
1mg/kg and azathioprine added to allow for prednisolone dose tapering.
She remains stable on above in addition to heart failure therapy (NYHA
class I). Her ECG findings remain unchanged.