Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 3

Long Term Oxygen Therapy (LTOT):
In 20%, IPF guidelines ‘Oxygen Therapy’, or supplementary oxygen for home use, became a strong recommendation for use in those patients with clinically significant resting hypoxemia.

Although oxygen therapy has not been shown to improve survival in IPF some data indicate an improvement in exercise capacity.

Pulmonary Rehabilitation:
Fatigue and loss of muscular mass are common and disabling problems for patients with IPF.

Pulmonary rehabilitation may alleviate the over symptoms of IPF ad improve functional status by stabilizing and reversing the extra-pulmonary features of the disease.

Palliative Care:
It 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 hypoxemia patients.

Palliative care also includes relief of physical and emotional suffering and psychosocial support for patients and care givers.

With disease progression patients may experience fear, anxiety and depression.
Psychological counseling should be considered.

In selected cases of particularly severe dyspnea morphine could be considered. It reduces dyspnea, anxiety and cough.

Management and follow up:
IPF is often misdiagnosed when physiological or imagine data suggest the presence of ILD leading to delay in accessing appropriate care.

After diagnosis of IPF, and appropriate treatment choice according to symptoms and stage of diseases, a close follow-up should be applied.

Due to the high variable course of disease, the higher incidence of complications like lung cancer, a routine evaluation every 3 to 6 months, including spirometry (body plethysmography) diffusion capacity testing, chest x-ray, 6MWT, assessment of dyspnea, quality of life, oxygen requirement is mandatory.

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IPF : Pulmonary Function Test

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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History of IPF (Idiopathic Pulmonary Fibrosis)

In the absence of UIP pattern on HRCT a surgical lung biopsy is required for confident diagnosis.

Histologic specimens for the diagnosis of IPF must be taken at least in three different places and be large enough that the pathologist can comment on the underlying lung architecture.
Small biopsies, such as those obtained via transbronchial lung biopsy (performed during bronchoscopy) are usually not sufficient for this purpose.

Hence larger biopsies obtained surgically via a ‘Thoracotomy’ or ‘Thoracoscopy’ are usually necessary.
Lung tissue from people with IPF usually show a characteristic histopathologic UIP pattern and so is the pathologic counterpart of IPF.

Although a pathologic diagnosis of IPF, a UIP often corresponds to a clinical diagnosis of IPF, a UIP histological pattern can be seen in other diseases as well, and fibrosis of known origin (e.g. Rheumatic diseases).
There are four key features of UIP including interstitial fibrosis in a ‘patch work pattern’ interstitial scarring, honeycomb changes and fibroblast foci.

Fibroblastic foci are dense collections of fibroblasts and scar tissue and together with honeycombing, are the main pathological findings that allow a diagnosis of UIP.

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Classification of IPF – There are more than 200 lung diseases

Idiopathic Pulmonary Fibrosis

IPF stands Idiopathic Pulmonary Fibrosis.

 

 

It is chronic decline in lungs function. Pulmonary Fibrosis means scarring of lung’s tissues & it is a cause of worsening dyspnoea (shortness of breathing).

The type IIP it means that Idiopathic Interstitial Pneumonia. It is type of ILD. It is known as disease Parenchymal Lung Disease.

Different types of diseases : 

1. IPF           2. NSIP             3. RB             4. JLD            5. DIP           6. COP-ATP

Associated Symptoms :

– Hypersensitivity pneumonitis,

– Pulmonary Langerhan’s Cell History cytosis,

– Asbestosis,

– Collagen,

– Vascular Diseases

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