What are the treatment options for idiopathic pulmonary fibrosis in restrictive lung disease?

Updated: Sep 16, 2020
  • Author: Jonathan Robert Caronia, DO; Chief Editor: John J Oppenheimer, MD  more...
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The rate of progression of idiopathic pulmonary fibrosis (IPF) is highly variable, and controversy exists regarding the timing of treatment. The disease may be responsive to treatment in the early, so-called inflammatory stage. IPF always progresses insidiously, and documenting the changes over short periods is difficult. Initiate a trial of therapy for 6-12 weeks, starting as early as possible, with the hope of slowing disease progression. Discontinue therapy if no benefit is observed or if adverse effects develop.

The prognosis for patients with IPF who do not respond to medical therapy is poor. They usually die within 2-3 years. These and other patients with severe functional impairment, oxygen dependency, and a deteriorating course should be listed for lung transplantation.

Conventional therapies (corticosteroids, azathioprine, [45] cyclophosphamide) provide only marginal benefit to patients with IPF. Corticosteroids have never been studied against placebo. Retrospective studies have not demonstrated any benefit from steroid monotherapy. [46] Acute exacerbations may not respond to high-dose corticosteroid therapy. [47]

Intermittent intravenous cyclophosphamide given to IPF patients surviving 6 months improved pulmonary function and reduced prednisone dosage in one study. [48] However, current guidelines recommend against the use of combination immunosuppressant therapy, owing to limited efficacy data. [49]

Thalidomide has been shown to attenuate pulmonary fibrosis after a bleomycin challenge in animal models. [50] A randomized crossover design study has demonstrated a  significant reduction in cough and improved quality of life in patients with IPF. [51]

Pulmonary rehabilitation has been demonstrated to improve overall quality of life and can provide education and psychosocial support for patients with IPF. [52]

A retrospective cohort study found that treatment of gastroesophageal reflux disease was associated with an increased length of survival and reduced radiographic evidence of fibrosis. [53]

Supplemental oxygen can be provided for patients with hypoxemia (PaO2< 55 mm Hg or oxygen saturation [SaO2] < 88%) at rest or during exertion. However, rigorous studies of benefit or improvement in quality of life have not been demonstrated, as it has been in the COPD population.

Lung transplantation should be considered for patients with IPF refractory to medical therapy. [54] Transplantation has been reserved for patients at advanced stages of IPF. The 5-year mortality rate remains around 50%. However, bronchiolitis obliterans syndrome (BOS), a process of progressive fibrosis of the bronchioles, can occur post transplantation and has high mortality.

Because of a lack of response to available anti-inflammatory therapy, alternative approaches to therapy are being pursued. [55] Emerging strategies to treat patients with IPF include agents that inhibit epithelial injury or enhance repair, anticytokine approaches, agents that inhibit fibroblast proliferation or induce fibroblast apoptosis, and other novel approaches. [56]

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