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Interstitial lung disease (ILD) consist of a group of diseases that causes inflammation and scarring of the lung tissue. Typically these diseases affects interstitium which provides support to the lung tissue and maintains its structure. Interstitium consists of supportive tissue that surrounds the air filled spaces within the lung.
There are many forms of ILD but, generally all of them begins with inflammation of the air sacs (alveolitis), small airways (bronchiolitis) and small blood vessels (vasculitis).
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 in people with ILD.
• 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: 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: Lymphangiticcarcinomatosis and bronchoalveolar cell carcinoma
• Age: Though the disease can occur at any age, many patients tend to get it in adulthood.
• 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 recieving radiation to the chest for chest wall or intrathoracic malignancies.
• GERD Gastroesophageal reflux increases the risk of developing ILD.
ILD has been categorized into acute, subacute and chronic forms.
Acute occurs within days to weeks
Subacute occurs from weeks to months.
Chronic months to year
• Shortness of breath, especially with exertion or activity chills
• Dry cough
• Chest tightness
• Weight loss
There is generally an irreversible component to the respiratory symptoms even when the antigen is removed due to permanent lung damage (lung fibrosis) which cause hypoxemic (lack of oxygen) respiratory failure.
• Shallow and fast breathing
• Clubbing of digits (they become wide and rounded)
• Dyspnea with exertional fatigue (right heart failure)
• 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.
• Pulmonary fibrosis cause irreversible lung parenchyma damage resulting in permanently damage to functioning of the lung reducing its ability to oxygenate the blood.
• 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 ongoing 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.
• 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. It is advisable to be 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 .
• Remove, replace, change and stay away from the antigen/allergen, shift to new place or job if required.
• Cessation of smoking as it helps is stopping the condition from exacerbating.
• Mild cases the patient can live normally without treatment with some lifestyle modifications
• Mild to moderate cases require treatment, pulmonary rehabilitation and the condition may get stabilized
• Severe forms are generally progressive in nature and require continuous treatments, therapies and life style modifications to manage their symptoms. In certain cases , like Usual interstitial pneumonia (UIP) the prognosis is poor even with treatment as condition worsens with time.
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