The goals of treatment in<unk>  are essentially to reduce the symptoms, stop disease progression, prevent acute exacerbations, and prolong survival. Preventive care (e.g. vaccinations) and symptom-based treatment should be started early in every patient. Oxygen therapy In the 2011<unk>  guidelines, oxygen therapy, or supplementary oxygen for home use, became a strong recommendation for use in those patients with significantly low oxygen levels at rest. Although oxygen therapy has not been shown to improve survival in<unk> , some data indicate an improvement in exercise capacity.<unk>  rehabilitation Fatigue and loss of muscular mass are common and disabling problems for patients with<unk> .<unk>  rehabilitation may alleviate the overt symptoms of<unk>  and improve functional status by stabilizing and/or reversing the extrapulmonary features of the disease. The number of published studies on the role of<unk>  rehabilitation in<unk>  is small, but most of these studies have found significant short-term improvements in functional exercise tolerance, quality of life, and dyspnea on exertion. Typical programs of rehabilitation include exercise training, nutritional modulation, occupational therapy, education and psychosocial counseling. In the late phase of disease,<unk>  patients tend to discontinue physical activity due to increasing dyspnea. Whenever possible, this should be discouraged. Medications A number of treatments have been investigated in the past for<unk> , including interferon gamma-1β, bosentan, ambrisentan, and anticoagulants, but these are no longer considered effective treatment options. Many of these earlier studies were based on the hypothesis that<unk>  is an inflammatory disorder. Pirfenidone A Cochrane review comparing pirfenidone with placebo, found a reduced risk of disease progression by 30%. FVC or VC was also improved, even if a mild slowing in FVC decline could be demonstrated only in one of the two CAPACITY trials. A third study, which was completed in 2014 found reduced decline in lung function and<unk>  disease progression. The data from the ASCEND study were also pooled with data from the two CAPACITY studies in a pre-specified analysis which showed that pirfenidone reduced the risk of death by almost 50% over one year of treatment. N-acetylcysteine and triple therapy N-Acetylcysteine (NAC) is a precursor to glutathione, an antioxidant. It has been hypothesized that treatment with high doses of NAC may repair an oxidant–antioxidant imbalance that occurs in the lung tissue of patients with<unk> . In the first clinical trial of 180 patients (IFIGENIA), NAC was shown in previous study to reduce the decline in VC and DLCO over 12 months of follow-up when used in combination with prednisone and azathioprine (triple therapy). More recently, a large randomized, controlled trial (PANTHER-IPF) was undertaken by the National Institutes of Health (NIH) in the US to evaluate triple therapy and NAC monotherapy in<unk>  patients. This study found that the combination of prednisone, azathioprine, and NAC increased the risk of death and hospitalizations and the NIH announced in 2012 that the triple-therapy arm of the PANTHER-IPF study had been terminated early. This study also evaluated NAC alone and the results for this arm of the study were published in May 2014 in the New England Journal of Medicine, concluding that "as compared with placebo, acetylcysteine offered no significant benefit with respect to the preservation of FVC in patients with<unk>  with mild-to-moderate impairment in lung function". Nintedanib Nintedanib is a triple angiokinase inhibitor that targets receptor tyrosine kinases involved in the regulation of angiogenesis: fibroblast growth factor receptor (FGFR), platelet-derived growth factor receptor (PDGFR), and vascular endothelial growth factor receptor (VEGFR), which have also been implicated in the pathogenesis of<unk>  and<unk> . In both phase III trials, nintedanib reduced the decline in lung function by approximately 50% over one year. It was approved by the US FDA in October 2014 and authorised in Europe in January 2015. Lung transplantation Lung transplantation may be suitable for those patients physically eligible to undergo a major transplant operation. In<unk>  patients, lung transplant has been shown to reduce the risk of death by 75% as compared with patients who remain on the waiting list. Since the introduction of the lung allocation score (LAS), which prioritizes transplant candidates based on survival probability,<unk>  has become the most common indication for lung transplantation in the USA. Symptomatic patients with<unk>  younger than 65 years of age and with a body mass index (BMI) ≤26 kg/m2 should be referred for lung transplantation, but there are no clear data to guide the precise timing for LTx. Although controversial, the most recent data suggest that bilateral lung transplantation is superior to single lung transplantation in patients with<unk> . Five-year survival rates after lung transplantation in<unk>  are estimated at between 50 and 56%. Palliative care Palliative care focuses on reducing symptoms and improving the comfort of patients rather than treating the disease. This may include treatment of worsening symptoms with the use of chronic opioids for severe dyspnea and cough. Further, oxygen therapy may be useful for palliation of dyspnea in hypoxemic patients. Palliative care also includes relief of physical and emotional suffering and psychosocial support for patients and caregivers. With disease progression, patients may experience fear, anxiety and depression and psychological counseling should therefore be considered. In a recent study of outpatients with ILDs, including<unk> , depression score, functional status (as assessed by walk test), as well as<unk>  function, all contributed to the severity of dyspnea. In selected cases of particularly severe dyspnea morphine could be considered. It can reduce dyspnea, anxiety and cough without significant decrease in oxygen saturation. Follow-up<unk>  is often misdiagnosed, at least until physiological and/or imaging data suggest the presence of an ILD leading to delay in accessing appropriate care. Considering that<unk>  is a disease with a median survival of three years after diagnosis, early referral to a center with specific expertise should therefore be considered for any patient with suspected or known ILD. On the basis of the complex differential diagnostic, multidisciplinary discussion between pulmonologists, radiologists, and pathologists experienced in the diagnosis of ILD is of the utmost importance to an accurate diagnosis. After diagnosis of<unk> , and the appropriate treatment choice according to symptoms and stage of disease, a close follow-up should be applied. Due to the high variable course of disease, the higher incidence of complications such as lung cancer (up to 25% of patients has been reported in<unk>  a routine evaluation every 3 to 6 months, including spirometry (body plethysmography), diffusion capacity testing, chest X-rays, 6MWT, assessment of dyspnea, quality of life, oxygen requirement is mandatory. In addition, the increasing awareness of complications and common concomitant conditions frequently associated with<unk>  requires a routinely evaluation of comorbidities, most of them simply reflecting concurrent diseases of aging, and medications with their interaction and side effects. Acute exacerbations Acute exacerbations of<unk>  (AE-IPF) are defined as an unexplained worsening or development of dyspnea within 30 days with new radiological infiltrates at HRCT abnormality often superimposed on a background consistent with UIP pattern. The yearly incidence of AE-IPF is between 10 and 15% of all patients. The prognosis of AE-IPF is poor, with mortality ranging from 78% to 96%. Other causes of AE-IPF such as<unk>  embolism, congestive heart failure, pneumothorax, or infection need to be excluded.<unk>  infection have to be ruled out by endotracheal aspirate or BAL. Many patients experiencing acute deterioration require intensive care treatment, particularly when respiratory failure is associated with hemodynamic instability, significant comorbidities or severe hypoxemia. However, mortality during hospitalization is high. Mechanical ventilation should be introduced only after carefully weighing the person's long-term prognosis and, whenever possible, the person's wishes. However, current guidelines discourage the use of mechanical ventilation in patients with respiratory failure secondary to<unk> .
Idiopathic pulmonary fibrosis
Sarcoidosis
Mineral dust airway disease
Hypersensitivity pneumonitis
Allergic bronchopulmonary aspergillosis
Interstitial lung disease
Rheumatoid lung disease
Idiopathic interstitial pneumonia