General: Treatment and prognosis vary depending on the particular lung disease. Patients who smoke tobacco are encouraged to quit. Patients should discuss treatment options with their qualified healthcare providers.
Antifibrotics: Antifibrotics have been
used to help reduce the development of scar tissue. It has been suggested that the medication may help slow the progression of lung damage without suppressing the immune system. Colchicine and D-penicillamine have not shown positive results in clinical trials. However, pirfenidone was recently shown to have some potential benefit in a small phase II trial, and follow-up studies are ongoing. Further research is needed to determine whether antifibrotics can effectively reduce scar tissue in ILD patients.
Corticosteroids: Patients are initially treated with corticosteroids to reduce inflammation in the lungs. However, these medications only help about 20% of ILD patients. Individuals who have a non-idiopathic disorder are most likely to benefit from anti-inflammatory treatment. Steroids rarely alleviate ILD symptoms with idiopathic pulmonary fibrosis, and if they do, the benefits are usually temporary. In general, steroids have to be taken for several months until symptoms improve. The patient is tapered off treatment once the symptoms improve. If corticosteroids are taken for long periods of time or in large doses, side effects may include glaucoma, bone loss (which can may lead to osteoporosis), high blood sugar levels (which may lead to type 2 diabetes), poor wound healing and increased susceptibility to infection.
Cytotoxic drugs: Cytotoxic drugs like azathioprine and cyclophosphamide have been used to treat ILD. The drugs are prescribed when steroid treatment is unsuccessful, or they are used in combination with steroids as a first-line treatment. Serious side effects, including reduced production of red blood cells, skin cancer and lymphoma, have been reported with cytotoxic drugs.
Lung transplant: A lung transplant may be necessary for patients who have severe ILD and do not respond to other treatment options. In order to be considered for a transplant, patients must not smoke or agree to quit smoking. They must be healthy enough to undergo surgery and post-transplant treatments. In addition, they must be willing to follow the medical program outlined by the rehabilitation and transplant team.
In most cases, single-lung transplants are more successful in people with ILD than double-lung transplants are.
The survival rate for a lung transplant is lower than it is for other types of transplants. Organ transplant recipients must take immunosuppressive (anti-rejection) drugs (like cyclosporine) for the rest of their lives. These agents make them more susceptible to infections and diseases.
Oxygen therapy: While oxygen therapy cannot prevent lung damage, it can make breathing and exercise easier for ILD patients. Oxygen therapy may also prevent or reduce complications associated with low oxygen levels and reduce blood pressure on the right side of the heart. Most patients receive oxygen during sleep or exercise. However, some patients may use it continually.
Pulmonary rehabilitation: Pulmonary rehabilitation is a program for patients with chronic lung disease. It includes both medical care and support. The goal is to help patients with ILD live full, satisfying lives. Programs focus on exercise, including breathing techniques, as well as education, emotional support and nutritional counseling. In most cases, rehabilitation programs include a team of healthcare professionals, including doctors, nurses, rehabilitation specialists, dietitians and social workers. Programs vary widely.
Unclear or conflicting scientific evidence
Ginkgo biloba: Based on early study, ginkgo may be effective in treating pulmonary interstitial fibrosis. Further research is needed to confirm these results.
Avoid if allergic or hypersensitive to members of the Ginkgoaceae family.
If allergic to mango rind, sumac, poison ivy or oak or cashews, then allergy to ginkgo is possible. Avoid with blood-thinners (like aspirin or warfarin) due to an increased risk of bleeding. Ginkgo should be stopped two weeks before surgical procedures. Ginkgo seeds are dangerous and should be avoided. Skin irritation and itching may also occur due to ginkgo allergies. Do not use ginkgo in supplemental doses if pregnant or breastfeeding due to insufficient scientific evidence of safety. The risk of bleeding associated with ginkgo may be dangerous during pregnancy.
Physical therapy: Lung hyperinflation is a technique used by physiotherapists to mobilize and remove excess lung secretions, reinflate areas of pulmonary collapse and improve oxygenation. Studies have compared manual vs. mechanical interventions and found no differences between the two. However, studies are lacking which compare physical therapy to placebo or other interventions. Additional research is needed in this area.
Not all physical therapy programs are suited for everyone, and patients should discuss their medical history with a qualified healthcare professional before beginning any treatments. Based on the available literature, physical therapy appears generally safe when practiced by a qualified physical therapist, however complications are possible. Treatment options should be considered carefully. Physical therapy may aggravate pre-existing conditions. Persistent pain and fractures of unknown origin have been reported. Physical therapy may increase the duration of pain or cause limitation of motion. Pain and anxiety may occur during the rehabilitation of patients with burns. Both morning stiffness and bone erosion have been reported in the physical therapy literature although causality is unclear. Erectile dysfunction has also been reported. Physical therapy has been used in pregnancy, specifically to treat women with pelvic girdle pain during pregnancy and at 3, 6, and 12 months postpartum. Reports of major adverse effects are lacking the available literature, but caution is advised nonetheless. All therapies during pregnancy and breastfeeding should be discussed with a licensed obstetrician/gynecologist before initiation.
Avoid routine exposure to occupational or environmental factors that may cause ILD.
Avoid tobacco cigarettes because smoking is strongly associated with idiopathic ILD.
Immunizations with the influenza (flu) vaccine (yearly) and pneumococcal pneumonia vaccine (every five to seven years) are recommended for individuals who are at risk for developing ILD because they may help prevent infection.
It is best to treat underlying medical conditions, such as respiratory infections, as quickly and efficiently as possible to avoid developing ILD or secondary complications.