The document provides information about interstitial lung disease (ILD), including: - ILD affects the interstitium, a lace-like network of tissue in the lungs. Common symptoms include shortness of breath. - There are many types of ILD, which can be caused by infections, autoimmune diseases, environmental exposures like asbestos, or idiopathic factors. - Diagnosis involves imaging tests and may require a lung biopsy. Treatment depends on the underlying cause but can include antibiotics, corticosteroids, oxygen therapy, immunosuppressants, or lung transplant in severe cases. Managing risk factors and lifestyle changes can also help.

1. AYESHA FAREED
PHARM D
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•Interstitial lung disease is a general category that includes many
different lung conditions.
•All interstitial lung diseases affect the interstitium, a part of
the lungs anatomic structure.
•The interstitium is a lace-like network of tissue that extends
throughout both lungs.
• The interstitium provides support to the lungs microscopic air sacs
(alveoli),Tiny blood vessels travel through the interstitium, allowing
gas exchange between blood and the air in the lungs.
•Normally, the interstitium is so thin it can't be seen on chest X-rays
or CT scans.

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Types of Interstitial Lung Disease:
•All forms of interstitial lung disease cause thickening of the
interstitium. The thickening can be due to inflammation, scarring, or
extra fluid (edema).
• Some forms of interstitial lung disease are short-lived; others are
chronic and irreversible.
Some of the types of interstitial lung disease include:
•1.Interstitial pneumonia: Bacteria, viruses, or fungi may infect the
interstitium of the lung. A bacterium called Mycoplasma pneumonia is
the most common cause.
•2.Idiopathic pulmonary fibrosis : A chronic, progressive form of fibrosis
(scarring) of the interstitium. Its cause is unknown.
•3.Nonspecific interstitial pneumonitis: Interstitial lung disease that's
often present with autoimmune conditions (such as rheumatoid
arthritis or scleroderma).

4. 4.Hypersensitivity pneumonitis: Interstitial lung disease caused by ongoing
inhalation of dust, mold, or other irritants.
5.Cryptogenic organizing pneumonia (COP): A pneumonia-like interstitial
lung disease but without an infection present. COP is also called bronchiolitis
obliterans with organizing pneumonia (BOOP).
6.Acute interstitial pneumonitis: A sudden, severe interstitial lung disease,
often requiring life support.
7.Desquamative interstitial pneumonitis: An interstitial lung disease that's
partially caused by smoking.
8.Sarcoidosis: A condition causing interstitial lung disease along with swollen
lymph nodes, and sometimes heart, skin, nerve, or eye involvement.
9. Asbestosis: Interstitial lung disease caused by asbestos exposure.
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5. Causes of Interstitial Lung Disease:
•Bacteria, viruses, and fungi are known to cause interstitial pneumonias.
•Regular exposures to inhaled irritants at work or during hobbies can also
cause some interstitial lung disease.
These irritants include:
Asbestos
Silica dust
Talc
Coal dust, or various other metal dusts from working in mining
Grain dust from farming
Bird proteins (such as from exotic birds, chickens, or pigeons)
Drugs such as nitrofurantoin, amiodarone, bleomycin, and many others
can rarely cause interstitial lung disease.
Men and women of any age can be affected.
 Interstitial lung disease is more common in people with autoimmune
disease, including lupus, rheumatoid arthritis, and scleroderma.
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6. The most common symptom of all forms of interstitial lung disease is
• shortness of breath, Nearly all people with interstitial lung disease will
experience breathlessness, which may get worse over time.
Other symptoms of interstitial lung disease include:
•Cough, which is usually dry and nonproductive.
•Weight loss, most often in people with COP or BOOP.
•In most forms of interstitial lung disease, the shortness of breath
develops slowly (over months).
• In interstitial pneumonias or acute interstitial pneumonitis, symptoms
come on more rapidly (in hours or days).
symptoms
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7. Risk factors:
Factors susceptible to interstitial lung disease include:
Age. Interstitial lung disease is much more likely to affect adults, although
infants and children are sometimes affected.
Exposure to occupational and environmental toxins. people work in
mining, farming or construction or for any reason are exposed to
environmental agents known to damage lungs.
Family history. There is evidence that some forms of interstitial lung
disease are heritable and risk of developing it is increased if close family
members have the disease.
Radiation and chemotherapy/immunomodulatory drugs: Having
radiation treatments to chest or using some chemotherapy or
immunomodulatory drugs
Smoking. Some forms of interstitial lung disease are more likely to occur in
people with a history of smoking, and active smoking may make the
condition worse, especially if there is associated emphysema.
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8. Complications
Acute exacerbation: Acute exacerbation can occur unexpectedly and often
requires hospitalization with supportive management. It is a serious complication
found in many types of interstitial lung disease.
Gastro esophageal reflux disease (GERD). Recent studies suggest that GERD
is associated with a more rapid progression of idiopathic pulmonary fibrosis, a
specific form of interstitial lung disease.
High blood pressures in the vessels of the lungs (pulmonary
hypertension. Pulmonary hypertension is a serious illness that becomes
progressively worse, leading to failure or dysfunction of the right side of the heart
(cor pulmonale).
Low oxygen (hypoxemia). As lung disease progresses, oxygen support may be
required both at rest and with exertion.
Respiratory failure. In the end stage of chronic interstitial lung disease,
respiratory failure occurs when severely low blood oxygen levels along with rising
pressures in the pulmonary arteries and the right ventricle cause heart failure.
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9. Diagnosis of Interstitial Lung Disease:
People with interstitial lung disease usually come to see a doctor due to
concern about shortness of breath or cough. Imaging tests of the lungs
are usually done to identify the problem.
Chest X-ray: Chest X-ray films in people with interstitial lung disease
may show fine lines in the lungs.
Computed tomography (CT scan): Interstitial lung disease can
usually be seen on a CT scan.
High-resolution CT scan: If interstitial lung disease is suspected,
using certain CT scanner settings can improve the images of the
interstitium. This increases the CT scan's ability to detect interstitial
lung disease.
Pulmonary function testing: People with interstitial lung disease may
have a reduced total lung capacity. They may also have a decreased
ability to transfer oxygen from their lungs into their blood
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10. Lung biopsy: Often, obtaining lung tissue to examine under a
microscope is the only way to determine which type of interstitial
lung disease a person has. There are several ways to collect lung
tissue, which is called a lung biopsy.
•Bronchoscopy: An endoscope is advanced through the mouth or
nose into the airways. Tiny tools on the endoscope can take a
sample of lung tissue.
•Video-assisted thoracoscopic surgery (VATS): Using tools
inserted through small incisions, a surgeon can sample multiple
areas of lung tissue.
•Open lung biopsy (thoracotomy): In some cases, traditional
surgery with a large incision in the chest is needed to obtain a lung
biopsy.
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11. Treatments for Interstitial Lung Disease
Treatments for interstitial lung disease vary according to the type of
interstitial lung disease and its cause.
Antibiotics: These are effective treatments for most interstitial
pneumonias.
•Azithromycin and levofloxacin eliminate the bacteria that cause most
interstitial pneumonias.
•Viral pneumonias usually resolve on their own. Fungal pneumonias
are rare, but can be treated with antifungal drugs.
Corticosteroids: In some forms of interstitial lung disease, ongoing
inflammation in the lungs causes damage and scarring.
Corticosteroids like prednisone and methylprednisolone reduce the
activity of the immune system. This reduces the amount of
inflammation in the lungs and the rest of the body.
Inhaled oxygen: In people with low oxygen blood levels due to
interstitial lung disease, inhaled oxygen may improve symptoms.
Regular use of oxygen might also protect the heart from damage
caused by low oxygen levels.
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12. Lung transplant: In advanced interstitial lung disease causing severe
impairment, a lung transplant may be the best option. Most people
undergoing lung transplant for interstitial lung disease make large gains in
quality of life and their ability to exercise.
Azathioprine (Imuran): This drug also suppresses the immune system. It
has never been proven to improve interstitial lung disease, but some
studies suggest it might help.
N-acetylcysteine (Mucomyst): This potent antioxidant may slow the
decline of lung function in some forms of interstitial lung disease. It does
not improve people's survival from interstitial lung disease.
Other less often-used treatments for interstitial lung disease include:
Cyclophosphamide (Cytoxan)
Methotrexate
Cyclosporine
These medicines suppress the immune system significantly. They may be
used in some cases of interstitial lung disease while monitoring for side
effects.
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13. Oxygen therapy
Using oxygen can't stop lung damage, but it can:
•Make breathing and exercise easier
•Prevent or lessen complications from low blood oxygen levels
•Reduce blood pressure in the right side of your heart
•Improve your sleep and sense of well-being
•You're most likely to receive oxygen when you sleep or exercise,
although some people may use it round-the-clock.
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Antibiotics:
Azithromycin: macrolide antibiotic
Dose : 500mg given orally once daily, then 250mg once daily for 4days.
Later, alternate 500mg per oral once daily for 3 days
ADR: diarrhea (52.8%) nausea(32.6%) abdominal pain(27%)
Monitoring : LFTs, ECG (QT prolongation)
Pharmacodynamics: t half: 70hrs
bioavailability: 37%
Levofloxacin: floroquinolones
Dose: 500mg per oral or IV once daily for 7-14 days
ADR: nausea, headache, diarrhea (1-10%)
Monitoring: ECG (QT prolongation)
Pharmacodynamics: t half: 6-8hrs
bioavailability: 99%

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Prednisolone:
Dose:0.5 mg/kg to start, tapering over 3 months to 10-20mg/day
maintenance dose
Monitoring: blood glucose levels, electrolytes
ADRs: adrenal suppression, glucose intolerance
Pharmacodynamics: t half: 3-4 hrs
Azathioprine: immunosuppressant
Dose: 2mg/kg/day to maximum of 150mg
Side effects: amaemia, bone marrow suppression, alopecia
Monitor: epistaxis, mouth ulcer bleeding
Withhold :if
White cell count:<4x 10⁹/L
Neutrophil count: <2x 10⁹/L
Platelet count: <150 x 10⁹/L
AST/ALT: >2 times the upper limit of normal
Urea and creatinine rising without any alternative explanation

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Cyclosporine: (immunosuppressant)
Dose:
4-12hr pre transplant : 15mg/kg per oral for 1 dose
1-2 week post transplant:15mg/kg/day per oral twice daily
Maintenance dose: 12.5mg/kg/day (↑FVC) in 10% cases
Pharmacodynamics: t half: 8.4- 27hrs
Methotrexate:
It is used in RA associated ILD but <70% chances of causing pulmonary
impairment
Drug induced ILD?

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Cyclophosphamide:
Dose: 2mg/kg/day 3-4 weekly regimen over at least 6 months.
In some cases it is continued up to a year
ADR: thrombocytopenia, bleeding, infertility, hair loss
Monitor: secondary malignancies, LFT, renal function, CBC
Pharmacodynamics: t half: 3-12hrs
bioavailability: 75%

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COMBINATION THERAPY:
•Start Prednisolone first at dose of 0.5mg/kg/day tapering over 3 months
to 10-20mg/day maintenance.
•After 1 month start azathioprine at a dose of 50mg daily with weekly blood
tests. if blood remain stable increase the dose by 50mg increments every
month to a maximum dose of 150mg daily.
•After a further month add in NAC at full dose (600mg daily)
•All 3 drugs should be continued for long time
•Monitoring: CBC,LFT, every week for first 4 weeks, then every 2 weeks for
the next month, reducing it to every 3 months once stable dosing has been
achieved.

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•Cyclosporine 7mg/kg/day and prednisolone 20mg/day theses doses are
maintained for 8-10 weeks and reduced over following 2months to
• maintenance dose of 3mg/kg/day of of cyclosporine and 10mg/day of
prednisolone.
This combination decreased the dysponea and increased FVC

20. Lifestyle and home remedies:
•Stop smoking. If you have lung disease, the best thing you can do
for yourself is to stop smoking.
•Eat well. People with lung disease may lose weight both because it's
uncomfortable to eat and because of the extra energy it takes to
breathe.
•These people need a nutritionally rich diet that contains adequate
calories. A dietitian can give you further guidelines for healthy eating.
•Remain active. As much as you can tolerate, continue to exercise
and remain active to avoid deconditioning.
•Get vaccinated. Respiratory infections can worsen symptoms of
interstitial lung disease. Make sure you receive the pneumonia
vaccine and an annual flu shot.
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21. •Mason, R. Murray and Nadel's ,Textbook of Respiratory
Medicine, 4th edition, Elsevier Saunders, 2005.
•National Jewish Health: "Interstitial Lung Disease (ILD): Overview."
FamilyDoctor.org: "Occupational Respiratory Disease.“ News
release, FDA.Reviewed by Varnada Karriem-Norwood, MDon July
15.
•J A moolman et al. cyclosporine as a treatment fot interstitial lung
disease of unknown aetiology:1991;46:592-595.
•Buckinghamshire hospitals NHS trust, department of respiratory
medicine,azathioprine,prednisolone,and Nacetyl cysteine in
interstitial lung disease; guidelines for prescribing and ss at
<<http://www.brit-thoracic.org.uk>>
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References:

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