Interstitial Lung Disease

Prepared by –Dharajiya Chandrika

 INTRODUCTION
• Interstitial lung disease is another term for
pulmonary fibrosis, or “scarring” and
“inflammation” of the interstitium (the tissue
that surrounds the lung’s air sacs, blood vessels
and airways).
• This scarring makes the lung tissue stiff, which
can make breathing difficult.
• Symptoms include shortness of breath and
coughing.
• Treatments include medications and oxygen
therapy.
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• ILD may be limited to the lung, or it can be
related to a condition that may affect other
parts of the body, such as rheumatoid
arthritis or sarcoidosis (Sarcoidosis is a disease
involving abnormal collections of
inflammatory cells that form lumps known as
granulomas.
• The disease usually begins in the lungs, skin,
or lymph nodes..)
Types acc. To causes of interstitial lung
disease
• There are more than 200 causes of ILD.
Because ILD includes many disorders, it is
categorized based on the cause. These types
of ILD include
• 1) ILD related to another health
disorder: Some people develop ILD as a result
of having an autoimmune disease (the
immune system harms the body). Examples of
autoimmune diseases include rheumatoid
arthritis, scleroderma, polymyositis/dermato
myositis, lupus and sarcoidosis.
 Conti..
• Scleroderma is a chronic but rare autoimmune
disease in which normal tissue is replaced
with thick tissue with extra collagen.
• Dermatomyositis occurs when polymyositis
(an inflammatory muscle disease) affects the
skin, causing a scaly rash and/or purple spots.
Steroids are usually the first treatment tried.
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• 2) ILD caused by breathing in harmful
substances: People who breathe harmful particles such
as coal dust, asbestos, tobacco smoke or hairdressing
chemicals may develop ILD.
• 3) Genetic ILD: A genetic ILD occurs when the disease
is passed down among family members. These
conditions include neurofibromatosis (a disease in
which tumors grow on nerves) and Gaucher
disease (marked by enlargement of internal organs,
including the spleen and liver, and lesions on the
bones).
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• 4) Idiopathic ILD: Idiopathic means the cause
is not known. Idiopathic ILD usually affects
people over 60 years old.
 symptoms of interstitial lung disease

• Shortness of breath
• Coughing, typically nonproductive
• Decreased exercise tolerance
• Fatigue
• Weight loss
 Diagnosis
• A physical exam. ask if you have any systemic
conditions (disorders that affect the entire
body) or have had any exposure to harmful
substances.
• A lung function test to check how well your
lungs are working
• Imaging tests such as X-ray and a high-
resolution computed tomography (CT scan)
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• Bronchoscopy, a test in which we inserts a device
called a bronchoscope through nose or mouth into
lungs to look inside your airways.
• , we will take a sample of lung tissue. A test of this
tissue, called a biopsy, can further identify the type
of ILD.
• During a biopsy, removes a small sample of tissue
from the lung and studies it under a microscope to
help identify the type of ILD.
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• Video-assisted thoracoscopic surgery (VATS).
make small cuts to insert tools that take
samples from multiple areas of lung tissue.
• Open lung biopsy (thoracotomy). In some
cases need traditional surgery with a large
incision in the chest to get a lung biopsy.
 Treatments for Interstitial Lung Disease
• Medications can help improve lung function by
reducing inflammation and/or fibrosis.
Medications to reduce inflammation include
steroids (prednisone) and other rheumatologic
drugs, including mycophenolate (CellCept),
azathioprine (Imuran), leflunomide (Arava),
rituximab (Rituxan), cyclophosphamide
(Cytoxan), tacrolimus (Prograf) and others.
Medications to stop further fibrosis include
pirfenidone (Esbriet) and nintedanib (Ofev).
 Conti..
• Oxygen therapy: Extra oxygen delivered through
a tube in the nose can make breathing easier. This
therapy raises the blood’s oxygen levels, so that
every breath is more productive.
• Pulmonary and exercise therapy: Breathing
exercises and increased physical activity can
improve lung fitness.
• Lung transplant: Some people with severe cases
of ILD have lung transplants to help prolong their
lives.
Interstitial Lung Disease Complications

• High blood pressure in your lungs, known as

pulmonary hypertension
• Respiratory failure
• Right-sided heart failure, known as cor
pulmonale