BackgroundPirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone’s effect on mortality and adverse events profile outside the restrictions of a clinical trial.MethodsThis is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort).ResultsWe observed a high 3-years survival rate of 73% without excluding patients who discontinued treatment for different reasons. The survival was compared to the survival of an IPF cohort from a tertiary referral center (RBH cohort). After exclusion of severe cases (DLco< 30%), in unadjusted analysis, the survival in the UHH cohort was better than in the RBH cohort (HR:0.32, 95% CI: 0.19–0.53, p < 0.0001). After adjustment for age, gender and FVC, the survival remained higher in the UHH cohort (HR:0.28, 95% CI: 0.16–0.48, p < 0.0001). We observed a similar safety profile compared to previously published data and a lower rate of drug discontinuation due to photosensitivity reactions. Conclusion: Pirfenidone provides a survival benefit in a real-life IPF cohort compared to previously used medications. Counselling patients and proactively managing possible adverse effects can reduce the necessity to discontinue pirfenidone.

Extrathoracic lung hernias can be congenital or acquired. Acquired hernias may be classified by etiology into traumatic, spontaneous, and pathologic. We present a case of a 40-year-old male with a history of bronchial asthma and a blunt chest trauma who presented complaining of sharp chest pain of acute onset that began after five consecutive days of vigorous coughing. Upon physical examination a well-demarcated deformity overlying the third intercostal space of the left upper anterior hemithorax was revealed. Thoracic CT scan showed that a portion of the anterior bronchopulmonary segment of the left upper lobe had herniated through a chest wall defect. The role of imaging, especially chest computed tomography with multiplanar image reconstructions and maximum (MIP) and minimum intensity projection (MinIP) reformats can clearly confirm the presence of the herniated lung, the hernial sac, the hernial orifice in the chest wall, and exclude possible complications such as lung tissue strangulation.

We present a case of primary renal angiosarcoma. We focus on the characteristic striated pattern of the tumor on T2-w MR sequence as well as on other radiological features and correlate them with the pathologic findings. A review of the imaging characteristics of cases published in the literature was subsequently performed.

Coronary artery fistulas represent abnormal communications between a coronary artery and a major vessel like venae cavae, pulmonary arteries or veins, the coronary sinus, or a cardiac chamber. The latter is called coronary cameral fistula is a rare condition and is most of the times congenital but can be also post traumatic or post surgical, especially after cardiovascular interventional procedures. Most patients are asymptomatic and coronary-cameral fistulae are discovered incidentally during angiographic evaluation for coronary vascular disorders, while other patients have a clinical presentation ranging from angina pectoris to heart failure. In this article, we report a rare case of an aneurysmal right coronary cameral fistula draining into the left ventricle. Echocardiography usually represents the first diagnostic imaging approach, but often due to a poor acoustic window may not show the entire course of the fistula which is crucial for the final diagnosis. ECG-gated cardiovascular CT may play an important role in the evaluation of the origin, course, termination and morphology of the fistula, its relation to the adjacent anatomical structures as well as the morphology and contractility of the heart. Cardiac MRI instead plays an additional crucial role regarding not only the above mentioned factors but also in estimating the blood flow within the fistula, providing more detailed information about the cardiac function but also about myocardial wall viability.