A 42-yr old man initially presented with exertional dyspnoea and cough. He had no previous medical history of significance and was a non-smoker. The clinical examination and biochemistry were unremarkable, except for elevated serum angiotensin converting enzyme (ACE). Chest x-ray showed bulky hilar lymphadenopathy with extensive reticulonodular and mass like opacities in both lungs with a middle zone predominance (Figure 1). A non-contrast computed tomography (CT) scan of the thorax demonstrated numerous nodules in a perilymphatic distribution (subpleural and peribronchovascular) together with perihilar conglomerate masses (Figure 2). Several enlarged partly calcified hilar, paratracheal and subcarinal mediastinal lymph nodes were evident. Histopathology of a transbronchial lung biopsy showed numerous well-formed, non-necrotising epithelioid granulomas (Figure 3), but tests for bacilli, fungi and tuberculosis were negative. Based on clinical, radiological and pathological findings the diagnosis of pulmonary sarcoidosis was made.
Five years after initial presentation the patient represented with dyspnoea and peripheral edema. ACE was within normal limits and electrocardiogram (EKG) was normal. Findings on chest x-ray and CT scan showed minimal change compared to previous imaging. Transthoracic echocardiography showed normal LV function, and a severely dilated RV with moderately impaired contractile function. Pulmonary pressures were only mildly elevated as measured by both echocardiography and right-heart catheterisation. A cardiac magnetic resonance (CMR) scan confirmed a dilated RV with impaired systolic function (Figure 4), RV ejection fraction (EF) was 34% (normal 44-63%). Extensive, patchy delayed gadolinium enhancement was observed in the RV free wall, as well as subtle mid-wall delayed enhancement in the interventricular and the interatrial septum (Figure 5). The CMR findings led to a diagnosis of right heart failure secondary to sarcoidosis infiltration of the RV wall. The evidence of RV infiltration by sarcoidosis led to immediate immunosuppressive therapy in accordance with general recommendations for cardiac sarcoidosis, and insertion of an implantable cardioverter-defibrillator (AICD). Follow-up interrogation of the AICD revealed an episode of non-sustained fast ventricular tachycardia.
high resolution coronal. reformats from a non-contrast helical CT shows striking bilateral bulky hilar and mediastinal adenopathy. Mass-like fibrosis is encasing the hila and central bronchi causing traction bronchiectasis. There are several partially calcified hilar lymph nodes. There are also extensive perivascular and perilymphatic nodules throughout both lobes with relative sparing of the lower zones and right upper lobe bullae.