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Lege Artis Medicinae

SEPTEMBER 20, 2018

[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]

BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna

[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion bet­ween experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]

Lege Artis Medicinae

AUGUST 30, 2018

[How to diagnose idiopathic pulmonary fibrosis. Part 2]

HORVÁTH Ildikó, KERPEL-FRONIUS Anna, HARKÓ Tünde

[Idiopathic pulmonary fibrosis is a severe irreversible lung disease with a progressive course. The disease onset is hard to discover due to the unspecific signs and symp­toms. It occurs mainly in elderly people. In the past decades its prevalence has increased continuously. Physical examination, restrictive pattern on lung function test with decreased diffusion capacity are characteristic features of the disease. Chest X-ray showing fibrotic pattern also points toward the diagnosis of idiopathic pulmonary fibrosis. Differential diagnosis is based on high resolution komputertomográfy. Diag­nosis of IPF is based on the appearance of usual interstitial pneumonia pattern together with the lack of external risk factors and autoimmune or other diseases also known to cause this pattern seen on chest imaging. If no firm diagnosis can be built lung biopsy is required. Multidis­ciplinary teams from clinician, radiologist and pathologist are set in predefined centres that could provide care with novel antifibrotic drugs. These can slow disease progression and are in the frontline in the treatment of the disease. Further research is required to understand the pathomechanism and foster the discovery of further treatment options. ]

Lege Artis Medicinae

JUNE 20, 2018

[Diagnosis of interstitial lung diseases]

MÜLLER Veronika

[Recognition and diagnosis of interstitial lung diseases (ILD) is one of the most challenging tasks of respiratory medicine. In the wide range of different ILD-s idiopathic pulmonary fibrosis (IPF) is one of the most common. Improvements in the diagnosis made the recognition of IPF easier. Typical clinical features and usual interstitial pneumonia pattern on high resolution CT are now enough for the confident diagnosis of IPF in the multidisciplinary ILD-team discussions. Bronchoscopic cryobiopsy is a great achievement of histological sampling as this can give appropriate size tissue for histological analysis making surgical intervention rare. As IPF cannot be cured early diagnosis is crucial. Today available treatments can only slow down progression of the disease, so early stages and better clinical condition of the patients are essential for therapeutic success. Follow-up of IPF patients includes complex lung functional measurements in dedicated centers in Hungary. Former studies confirmed forced vital capacity (FVC) decline as an important measure of disease progression and mortality. Available IPF treatments decrease FVC decline and can prevent the mostly deadly acute exacerbations of the disease. Additionally palliative treatments including supplementary oxygen and in selected patients lung transplantation can be offered. This is the first of a series of three articles about IPF. ]