RESUMO
Idiopathic pulmonary fibrosis (IPF) is a very specific form of a chronic, progressive fibroproliferative interstitial pneumonia of unknown aetiology. The disease is generally associated with a poor prognosis. Several international evidence-based guidelines on the diagnosis and management of IPF and other interstitial lung diseases (ILDs) have been published and updated in the last decade, and while the body of evidence for the use of some treatment modalities has grown, others have been shown to be futile and even harmful to patients. In a patient who presents with the classic clinical features, restrictive ventilatory impairment with impaired diffusion and a high resolution computed tomography (HRCT) scan of the lungs showing a usual interstitial pneumonia (UIP) pattern, a definitive diagnosis of IPF can be made, provided all other causes of a radiological UIP pattern are excluded. Patients who present with atypical clinical features or an HRCT pattern classified as "possible" UIP, should be referred for a surgical lung biopsy. Once the diagnosis of IPF is confirmed, a patient-centred approached should be followed, as the stage of the disease, degree of impairment, rate of disease progression, comorbid illnesses and patient preferences all impact on long-term management. The South African Thoracic Society (SATS) suggests that anti-fibrotic treatment should be offered to appropriate candidates [confirmed IPF with a forced vital capacity (FVC) of 50-80%], but discontinued should there be evidence of disease progression (a decline in FVC of ≥10% within any 12-month period). The routine use of high dose oral steroids, immunosuppressive drugs and anticoagulants is not recommended whilst anti-acid therapy may be considered in patients without advanced disease.