The goals of treatment in IPF 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 shared early in every patient.
A number of treatments have been investigated in the past for IPF, including interferon gamma-1β, bosentan, ambrisentan and anticoagulants, but there are no longer considered effective treatment options.
Many of these studies were based on the hypothesis that IPF is an inflammatory disorder.
Pirfenidone is a small molecule that combines both the anti-inflammatory, anti-oxidant, and anti-fibrotic effects in experimental models of fibrosis.
Pirfenidone marketed under the trade name ‘Esbriet’ is approved in Europe for the treatment of patients with mild-to-moderate IPF.
N-Acetylcysteine and Triple Therapy:
N-Acetylcysteine (NAC) is a precursor to glutathione, an antioxidant.
Treatment with high doses of NAC may repair an oxidant-antioxidant imbalance that occurs in the lung tissue of the patients with IPF.
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Pulmonary Function Test:
Spirometry reveals a reduction in the vital capacity (VC) with a proportionate reduction in airflows or increased airflows for the observe vital capacity.
The latter findings reflects the increased lung stiffness (reduced lung compliance) associated with pulmonary fibrosis, which leads to increased lung elastic recoil.
Measurement of static lung volumes using body plethysmography or other techniques typically reveals reduced lung volumes (restrictions). This reflects the difficulty encountered in inflating the Fibrotic lungs.
The diffusing capacity for carbon monoxide (DLCO) is invariably reduced in IPF and may be only abnormality in mild or early disease.
Its impairment underlies the propensity of patients with IPF to exhibit oxygen desaturation with exercise which can also be evaluated using the 6 minutes’ walk test (6 MWT).
Terms such as mild moderate, severe are used for staging diseases and are commonly based on resting pulmonary function test ‘measurements’. There is no clear consensus regarding the staging of IPF patients and what are the best criteria and values to use.
Mild to moderate IPF has been characterized by the following functional criteria:
– Forced vital capacity (FVC) of ≥ 50%
– DTOC of ≥ 30%
– 6 MWT distance ≥ 150 meters
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Broncho-alveolar lavage(BAL) is a well tolerated diagnostic procedure in ILD.
Broncho-alveolar lavage(BAL) cytology analysis (differential cell counts) should be
considered in the evaluation of patients with IPF at the discretion of the treating physicians:
Histio-cytosis x, or
In the evaluation of patients with suspected IPF, the most important application of Broncho-alveolar lavage(BAL) is in the exclusion of other diagnosis.
Prominent lympho-cytosis allows excluding a diagnosis of IPF.
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