Clinical cases

Idiopathic pulmonary fibrosis – a case report

Abstract

Our understanding of the idiopathic interstitial lung disease (ILD) has undergone dramatic changes in the last decade, mostly in disease classification and diagnostic processes, and the role of high-resolution computed tomography (HRCT) of the chest in assessment of diagnosis and prognosis. The most important change has been the evidence of different histopathologic subgroups that make up the current classification of idiopathic interstitial pneumonias.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition of uncertain aetiology that should be considered in the differential diagnosis of patients who experience breathlessness, cough and reduced exercise tolerance. The role of high-resolution computed tomography in the diagnosis of interstitial lung disease is increasing as our understanding of its diagnostic accuracy improves. The patient described in the present case was a 59-year-old female who presented with 3 years history of dyspnea on exertion, cough, and low grade fever (thyroidectomy for thyroid malignancy 4 months before current presentation). The findings on HRCT were ground-glass opacities and reticular abnormality with subpleural and lower lung zone predominance. She underwent surgical lung biopsy for differential diagnosis between IPF, nonspecific interstitial pneumonia and thyroid lung metastases. The histological examination showed a pattern of usual interstitial pneumonia (UIP) what is the underlying lesion in IPF.

Key words: usual interstitial pneumonia, lung biopsy

Article

A rare cause of pulmonary arterial hypertension diagnosed in an elderly patient

Abstract

Pulmonary arterial hypertension (PAH) is a rare and incurable disease, related to right ventricle overload and failure, which are late consequences of asymptomatic progressive pulmonary vascular occlusion. The clinical approach requires: a high clinical suspicion; the detection and confirmation of PAH by echo-Doppler and right heart catheterisation; identification of an etiology; assessment of functionality and life expectancy; and reversibility testing.

We present the case of a 68 year old male patient presenting with progressive fatigability and shortness of breath, abnormal heart beats in the last 4 years, aggravated in the last year. Clinical findings showed signs of cardiac failure. Multiple echocardiographies demonstrated right atrial and right ventricular dilatation, with severe PAH, subsequent severe pulmonary and tricuspid regurgitation, mild mitral and aortic regurgitation and efficient left ventricular function. Subsequent cardiac catheterization confirms severe PAH, excludes VSD, and sees a left-to-right shunt but an ASD could not be anatomically localized. Left ventricular function and the coronary arteries were normal. Transesophageal echocardiography demonstrated an ASD sinus venosus with bidirectional shunt associated with partial abnormal in pulmonary venous drainage, with right supranumery pulmonary vein with drainage in the sinus venosus, the upper and inferior right pulmonary veins flowing into the right atrium, the upper and inferior left pulmonary veins flowing into the left atrium, associated to important superior and inferior vena cava dilatation.

The patient receives treatment for right heart failure, oral anticoagulation, antiarrhythmic drugs, cardio-pulmonary rehabilitation is initiated and he is referred to a center specialised in PAH, for bosentan treatment.

In this patient, it is surprising that even born with a potentially cyanogenic congenital heart disease, his condition is discovered at an advanced age on the edge for evolution towards an Eisenmenger Syndrome, being fairly asymptomatic until the last year when he receives treatment for left heart failure.

Keywords: pulmonary hypertension - congenital heart diseases - atrial septal defect

Article