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.