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Interstitial pulmonary fibrosis

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What is interstitial pulmonary fibrosis ?

Interstitial pulmonary fibrosis is most common ILD of unknown cause where the lung undergoes irreversible damage in form of fibrosis which causes compromised functioning of the lung . Its prevalence increase with age and is generally diagnoses in 5th or 6th decade of life, being more common in men. Frequently associated with history of smoking or exposure to other environmental antigens.

Very poor prognosis with survival of 3-5 years.

Like in all other forms of ILD it generally begins with inflammation of the air sacs (alveolitis) , inflammation of small airways (bronchiolitis) and inflammation of small blood vessels (vasculitis).

How does it all happen?

It all starts with injury or damage to the lung parenchyma due to a known cause ( prolonged exposure to some allergen or part of occupational hazard, drug induced or auto-immune ) or unknown cause.

This leads to inflammatory changes in the air-sacs, air-ways and blood vessels which causes scarring and fluid retention. The scarring is seen in form of fibrosis of lung parenchyma, enlarged airspaces and thickened interstitum.

Lung parenchyma, air sacs ,space around the air sacs and the blood vessels show permanent damage as the disease progresses. This makes it difficult and decreases the efficacy of blood to absorb oxygen hence results in breathing difficulties.

Causes:

• Environmental & Occupational hazards: silica, asbestos, Beryllium, animal/bird antigens, farmers , Grain dust and indoor hot tubs.

• Drug induced from antibiotics like ethambutol and nitrofurantoin, heart medicines like amiodarone and propranolol, anti-inflammatory medicines like rituximab and sulfasalazine and chemotherapy drugs like methotrexate and cyclophosphamide.

• Connective tissue disease Scleroderma, Polymyositis, Systemic lupus erythematosus, Rheumatoid arthritis, mixed connective tissue disease, Ankylosing spondylitis, Primary Sjogren’s syndrome and Behcet’s syndrome.

• Infections Atypical pneumonias, Pneumocystiscarinii pneumonia and Tuberculosis.

• Idiopathic causes are Sarcoidosis, Eosinophilic granuloma, Bronchiolitis obliterans organizing pneumonia, Lymphocytic interstitial pneumonia, Lymphangioleiomyomatosis, Usual interstitial pneumonia, Nonspecific interstitial pneumonia, Desquamative interstitial pneumonia, Respiratory bronchiolitis with interstitial lung disease and Acute interstitial pneumonia.

• Malignant causes are lymphangitic carcinomatosis and bronchoalveolar cell carcinoma

Risk factors:

• Age: Though the disease can occur at any age, generally ~60 years of age

• Gender: More common in males than females.

• Genetics: plays an important role in some conditions/ causes.

• Occupation: Farmers, building constructors and birds/animals breeders have long term of exposure to the allergens hence leads of high risk of developing ILD

• Life style: Smokers are at higher risk of getting ILD and it exacerbates the condition once it has been established.

• Radiation and chemotherapies: They increase the risk of developing ILD in patients receiving radiation to the chest for chest wall or intrathoracic malignancies.

• GERD Gastroesophageal reflux increases the risk of developing ILD.

Symptoms:

• Dry cough

• Malaise

• Dyspnea

• Fatigue

• Weight loss

Alarming signs:

• Shallow and fast breathing

• Clubbing of digits (they become wide and rounded)

• Dyspnea with exertional fatigue (right heart failure)

Complications:

• Pulmonary hypertension results due to damage to the pulmonary blood vessels.

• Heart failure is a serious complication that occurs due to inflammation resulting in burdening the heart which fails to pump enough blood.

• Lung cancer

• Pulmonary embolism

• Pneumonia

Diagnosis:

Lung function test – Spirometer devices are used to assess the pulmonary functioning in form of how long the lung can hold, take in and move out the air.It is helpful in assessment of the the on going treatment and in grading the seriousness the disease. It helps in characterization of physiological impairment and knowing diffusion capacity for carbon monoxide in cases with lung fibrosis.

Prevention:

• Avoid cigarette smoke. Quit cigarette smoking and avoid being a passive smoker as well, since it exacerbates the condition.

• Wear a mask (N95 respirators ) to avoid environmental stimuli like birds, feathers, pollution , dust, fire smokes, to be also used by farmers, woodworkers, wine makers, animal and bird breeders.

• Maintaining hygiene: Simple steps like washing hands, use of hand sanitizers , and wearing a mask can prevent from exposure to the allergens to some extent.

• Flu shots: Getting flu vaccine can prevent from infections affecting the lung.

• Cleaning: deep cleaning the ventilation system, timely serving of AC- conditioners/ humidifiers and cleaning out the house and carpets of the dust, animal fur/ bird feathers .

Treatment:

• Cessation of smoking as it helps is stopping the condition from exacerbating.

• Get rid of the source: Stay away from the antigen/allergen, shift to new place or job if required.

Prognosis:

• It’s a severe form of ILD hence it is progressive in nature and require continuous treatments, therapies and lifestyle modifications to manage their symptoms but the prognosis is poor even with treatment as condition worsens with time.

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