Prednisone is one of the most commonly used types of steroid medications used to treat ILD patients who have inflammation in their lungs. Prednisone is effective in quickly suppressing the immune system from creating inflammation in the lungs, thereby decreasing the potential for scarring of the lungs. 

When the immune system response is decreased, though, the body cannot fight bacteria or viruses as well, so a person is more vulnerable to infections.  It is important for a person taking prednisone to pay attention to any new signs of illness, such as fever, sweats, chest congestion, change in sputum color or amount, or new or worsening shortness of breath or fatigue, because these may be signs of infection.  These new symptoms should be reported to your provider. People taking prednisone can also experience significant side effects. These can include:

• cataracts    
• easy bruising
• thinning of the skin
• mood chinsomnia
• weight gain
• water retention
• thinning of the bones
• anges
• glaucoma
• diabetes  

In some forms of ILD, prednisone works well to reduce tissue damage and scarring of the lungs.  But because of potential side effects from this medication, the general rule is to take the least amount, for the least amount of time, to do the job.  

Your provider will work closely with you and monitor you to make sure you are taking the right amount of prednisone.

What special precautions should I follow?

Before taking prednisone:

• Tell your provider and pharmacist if you are allergic to prednisone, other medications or any of the inactive ingredients in prednisone tablets or solutions. Ask your provider or pharmacist for a list of the inactive ingredients.
• Tell your provider and pharmacist what prescription and nonprescription medications, vitamins and nutritional supplements you are taking or plan to take. Be sure to mention any of the following:
  • Amiodarone (Cordarone, Pacerone)
  • Anticoagulants ('blood thinners') such as warfarin (Coumadin) 
  • Certain antifungals such as fluconazole (Diflucan), itraconazole (Sporanox), ketoconazole (Nizoral) and voriconazole (Vfend)
  • Aprepitant (Emend)
  • Aspirin
  • Carbamazepine (Carbatrol, Epitol, Tegretol)
  • Cimetidine (Tagamet)
  • Clarithromycin (Biaxin, in Prevpak)
  • Cyclosporine (Neoral, Sandimmune)
  • Delavirdine (Rescriptor)
  • Diltiazem (Cardizem, Dilacor, Tiazac, others)
  • Dexamethasone (Decadron, Dexpak)
  • Diuretics ('water pills')
  • Efavirenz (Sustiva)
  • Fluoxetine (Prozac, Sarafem)
  • Fluvoxamine (Luvox)
  • Griseofulvin (Fulvicin, Grifulvin, Gris-PEG)
  • HIV protease inhibitors including atazanavir (Reyataz), indinavir (Crixivan), lopinavir (in Kaletra), nelfinavir (Viracept), ritonavir (Norvir, in Kaletra), and saquinavir (Fortovase, Invirase)
  • Hormonal contraceptives (birth control pills, patches, rings, implants and injections)
  • Lovastatin (Altocor, Mevacor)
  • Diabetes medications
  • Nefazodone
  • Nevirapine (Viramune)
  • Phenobarbital
  • Phenytoin (Dilantin, Phenytek)
  • Rifabutin (Mycobutin), rifampin (Rifadin, Rimactane, in Rifamate)
  • Sertraline (Zoloft)
  • Troleandomycin (TAO)
  • Verapamil (Calan, Covera, Isoptin, Verelan)
  • Zafirlukast (Accolate)
• Tell your provider what herbal products you take or plan to take, especially St. John's wort.
• Tell your provider if you have:
  • An eye infection now or have ever had eye infections that come and go
  • Diabetes
  • Emotional problems
  • Heart disease
  • High blood pressure
  • Intestinal disease
  • Kidney disease
  • Liver disease
  • Mental illness
  • Myasthenia gravis, a condition in which the muscles become weak
  • Osteoporosis, a condition in which the bones become weak and fragile and can break easily
  • Seizures
  • Threadworms — a type of worm that can live inside the body — currently or in the past
  • Thyroid disease
  • Tuberculosis (TB)
  • Ulcer 
• Your provider may need to change the doses of your medications or monitor you carefully for side effects.
• Tell your provider if you are pregnant, plan to become pregnant or are breast-feeding. If you become pregnant while taking prednisone, call your provider.
• If you are having surgery, including dental surgery, or need emergency medical treatment, tell the provider, dentist or medical staff that you are taking or have recently stopped taking prednisone. You should carry a card or wear a bracelet with this information in case you are unable to speak in a medical emergency.
• Prednisone may decrease your ability to fight infection and can prevent you from developing symptoms if you get an infection. Stay away from people who are sick and wash your hands often while you are taking this medication. Be sure to avoid people who have chicken pox or measles. Call your provider immediately if you think you may have been around someone who had chicken pox or measles.

What side effects can this medication cause?

Prednisone may cause side effects. Tell your provider if any of these symptoms are severe or do not go away:

• Acne
• Bulging eyes
• Changes in personality
• Changes in the way fat is distributed on the body
• Decreased sexual desire
• Difficulty falling asleep or staying asleep
• Dizziness
• Extreme changes in mood
• Extreme tiredness
• Headache
• Heartburn
• Inappropriate happiness
• Increased hair growth
• Increased sweating
• Irregular or absent menstrual periods
• Red or purple blotches or lines under the skin
• Slowed healing of cuts and bruises
• Thin, fragile skin
• Weak muscles

Some side effects can be serious. If you experience any of the following symptoms, call your provider immediately:

• Confusion
• Depression
• Difficulty breathing or swallowing
• Dry, hacking cough
• Eye pain, redness or tearing
• Hives
• Irregular heartbeat
• Itching
• Lightheadedness
• Loss of contact with reality
• Muscle twitching or tightening
• Numbness, burning, or tingling in the face, arms, legs, feet or hands
• Rash
• Seizures
• Shaking of the hands that you cannot control
• Shortness of breath, especially during the night
• Sore throat, fever, chills, cough or other signs of infection
• Swelling of the eyes, face, lips, tongue, throat, arms, hands, feet, ankles or lower legs
• Swelling or pain in the stomach
• Upset stomach
• Vision problems
• Vomiting

Prednisone may increase the risk that you will develop osteoporosis. Talk to your provider about the risks of taking prednisone and about things that you can do to decrease the chance that you will develop osteoporosis.

As you can see, prednisone can cause a number of side effects and affect many different organs in the body, not just the lungs. This is why your provider will be monitoring your symptoms closely.  It is important that you also call your provider if you have any new and unusual problems while you are taking this medication.

What monitoring will I need?

Your provider will order regular lab tests to check your response to prednisone and monitor for toxicity. You will need to have your blood sugar checked regularly. Your provider may order additional tests depending on the results.

For additional information on prednisone, please visit MedlinePlus.gov.

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These are medications that also work to suppress the immune system and decrease inflammation in your lungs, but don’t have the same side effects as prednisone. Your provider may recommend one of these as a replacement to prednisone or to minimize the amount of prednisone you have to take.

Examples of these types of medications, their typical uses, and associated potential side effects include:

• Cyclophosphamide (Cytoxan) – an immunosuppressive medication that can be administered orally or through an IV that is used to help suppress inflammation in the lungs; not as commonly used as before given the short and long-term potential side effects and toxicities such as nausea, vomiting, changes in skin and nails, missed menstrual periods. Though less commonly used these days, this medication can be used in conjunction with steroids in patients whose condition is worsening despite treatment.

• Mycophenolate mofetil (Cellcept) – immunosuppressive medication, commonly used to treat autoimmune/connective tissue disease related ILD or hypersensitivity pneumonitis; can be used alone or in conjunction with prednisone; side effects may include nausea, vomiting, stomach upset, diarrhea, increased risk for skin cancers.
• Rituximab (Rituxan) – monoclonoal antibody medication that lowers the B cells in the immune system that are responsible for producing certain harmful antibodies in autoimmune/connective tissue diseases. The medication is administered intraveneously in doses given a few weeks apart from each other. Side effects may include headache, nausea, diarrhea, muscle or joint pains, flushing.
• Azathioprine (Imuran) – immunosuppressive medication, commonly used to treat autoimmune/connective tissue disease related ILD or hypersensitivity pneumonitis; like Cellcept, can be used in conjunction with prednisone; side effects may include nausea, vomiting, delayed healing.
• Methotrexate – This medication reduces inflammation and suppresses the immune system and is commonly used in the treatment of autoimmune disease or certain cancers.
• Hydroxychloroquine (Plaquenil) – These medications, known as immunosuppressive and anti-parasitic medications, are used to treat sarcoidosis of the skin and lungs.
• Colchicine – This medication is most commonly used to treat gout and is sometimes prescribed to treat sarcoidosis-related arthritis for its anti-inflammatory effects.

Some important things to note is that all of these medications require periodic blood tests and close monitoring by your provider. Given that these medication suppress your immune system, you are at an increased risk for infection. This is why it is also important to work with your primary care providers to ensure all vaccinations are up to date.

NOTE: Insurances may require prior authorization before they agree to pay for some of these immunosuppressants, and some insurances do not pay at all for immunosuppressants used in interstitial lung disease.

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Pirfenidone (Esbriet) is an antifibrotic medication used to treat IPF. The recommended dosage is 801 mg three times daily, with food. Upon initiation of treatment, the daily dosage is usually titrated to the full dosage of nine capsules daily over a 14-day period. Taking nine capsules a day may sound like a lot, but once you achieve the full daily dose, the capsules can be combined into a total of 3 capsules a day.

Prior to starting treatment, you will need to get your labs checked for your liver function. These tests should be checked every 2 weeks during dose titration, and then once monthly when you get to the final dose. Dosage modification, interruption, or discontinuation may be necessary if liver enzymes are elevated.

Side effects primarily include photosensitivity and rash as well as gastrointestinal side effects such as nausea, vomiting, and diarrhea. Other side effects include elevations in liver enzymes, abdominal pain, upper respiratory tract infection, fatigue, headache, dyspepsia, dizziness, anorexia, gastroesophageal reflux disease, sinusitis, insomnia, weight loss, and arthralgia.

Drug interactions have been noted with medications that inhibit an enzyme called CYP1A2 (e.g. Ciprofloxacin and fluvoxamine) and may increase the side effect profile of Esbriet. Make sure that your provider is aware if you have any medication changes while taking pirfenidone.

Finally, it is important to remember that you cannot smoke while taking pirfenidone, as smoking may affect the efficacy of the medication.

Side effect management tips for pirfenidone (Esbriet)

• Gastrointestinal-related adverse events prevention:

1. Take pirfenidone during or after a meal.
2. Take each of the three capsules separately throughout the meal (rather than simultaneously).
3. Increase your dosage more slowly – for example, over 4 weeks instead of 2 weeks.
4. If side effects are not tolerable, dose reductions may be helpful with re-escalation to recommended daily dose as tolerated.
5. If side effects persist despite dose reduction, please consult your provider. Temporary treatment discontinuation can be done until symptoms become tolerable (typically 1-2 weeks) and can be reintroduced with a slower re-escalation scheme.
6. Use of proton pump inhibitors may be helpful for managing indigestion, heartburn, or reflux.
7. Many patients have been successful in managing these side effects with adjustments to their diet. Please also check out our nutrition section for additional tips on managing nausea and diarrhea. Please also see our nutrition section for tips on nausea.

• Skin-related adverse events prevention:

1. Sun exposure should be avoided for a few hours following pirfenidone intake.
2. Avoid exposure to direct sunlight.
3. Use sunscreen active against both UVA and UVB.
4. Use protective clothing (e.g. long-sleeved tops, hats, gloves, etc).
5. Avoid other medicinal products known to cause.

• Mild to moderate photosensitivity reaction or rash – reduce dose to one capsule three times daily for 7 days (or until symptom resolution).

1. If the rash persists for more than 7 days despite dose reduction, discontinue therapy for 15 days.
2. After the rash is resolved, re-introduce and re-escalate to recommended daily dose, as tolerated.

• Severe photosensitivity reaction

1. Discontinue medication.
2. Seek medical advice.
3. It is important to distinguish a photosensitivity reaction from an allergic reaction. In cases of an allergic reaction, pirfenidone should be permanently discontinued. The expertise of a dermatologist may be helpful.

• Liver Enzyme Elevation

1. It is important that you keep a schedule of when your labs are due while taking pirfenidone given the potential side effect of liver toxicity.
2. If your liver lab results are high, you will need to discuss this with your provider. Depending on how elevated the results are, you may be asked to decrease the dosage of pirfenidone, stop the medication temporarily, switch medications, or repeat the lab tests. Decreasing the dosage of pirfenidone temporarily for up to a few months, as far as we know, has not shown to result in a difference of outcomes compared to those people who do not need to decrease the dose.

For more information about pirfenidone and to sign up for the Esbriet Inspiration Program to help you take pirfenidone and manage side effects, you can check out their website here.

Nintedanib (Ofev) is an antifibrotic used to treat IPF. The recommended dosage is 150 mg (one pill) twice daily with food.

Prior to starting treatment, you will need to check your liver function with a lab draw. These labs should be checked after 2 weeks of starting the medication, and then once monthly for the next 3 months. If the results are normal, you can switch to labs every 3 months. Dosage modification, interruption, or discontinuation may be necessary if your liver function is elevated.

Side effects primarily include diarrhea and nausea/vomiting. Mild to moderate diarrhea should be treated with loperamide (Lomotil), and if persistent, should prompt dose reduction, interruption, or discontinuation. For nausea/vomiting that persists despite taking the drug with food and anti-emetic therapy, dose reduction, interruption, or discontinuation may be necessary. Your nintedanib prescription should have included free tablets of loperamide for the first few months of starting the prescription. If you haven’t received these, you can call the patient assistance program, Open Doors, at 1-866-OPENDOORS to request this. Many patients have been successful in managing these side effects with adjustments to their diet. Please also check out our nutrition section for additional tips on managing nausea and diarrhea.

Nintedanib is pregnancy category D meaning it can cause fetal harm. Women of child-bearing potential should be advised to avoid becoming pregnant while receiving treatment and to use adequate contraception.

There may be an increased risk of arterial thrombotic events (including myocardial infarction) and caution should be used when treating patients at higher cardiovascular risk.

Side effect management tips for nintedanib (Ofev):

• Diarrhea:

1. 4-6 extra stools per day, use loperamide as needed. Other things that may help with diarrhea include bulking agents such as Banatrol.
2. If >8 days, discontinue or reduce medication until less than 4 extra stools per day.
3. >6 stools or incontinence, hospitalization or decreased ADLs, discontinue medication until less than 4 extra stools per day then restart the medication at a reduced dose.
4. See our nutrition section for more tips on managing diarrhea.

• Liver Enzyme Elevation:

1. It is important that you keep a schedule of when your labs are due while taking nintedanib given the potential side effect of liver toxicity.
2. If your liver lab results are high, you will need to discuss this with your provider. Depending on how elevated the results are, you may be asked to decrease the dosage of nintedanib, stop the medication temporarily, switch medications, or repeat the lab tests. Decreasing the dosage of nintedanib temporarily for up to a few months, as far as we know, has not shown to result in a difference of outcomes compared to those people who do not need to decrease the dose.
3. For all other intolerable adverse events, consider a dose reduction or an interruption of medication. Going back up to the full dose is always possible once those intolerable symptoms resolve. If you needed to stop the medication due to side effects, resuming nintedanib at the lower dose of 100mg tablets twice a day is an option which people generally are able to tolerate better.
4. For more information about nintedanib and for additional support while you are taking the medication, we encourage you to sign up for the patient assistance program, Open Doors, which will give you access to a clinical nurse educator and the opportunity to talk to others who are also taking nintedanib.

Remember, this is not a complete list of all medications used to treat ILDs. New therapies are being tested in clinical trials all the time. You can ask your provider about current clinical trials, or jump to our Clinical Trials page for more information.