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Pulmonary Fibrosis, Interstitial (Nonidiopathic): Treatment & Medication
Updated: Jun 20, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Treatment is best determined by the specific diagnosis. Unfortunately, a specific etiology often is not determined. General supportive measures include the following:
- Smoking cessation should be counseled.
- Good pulmonary hygiene is important.
- Supplemental oxygen therapy may be useful for any patients who demonstrate significant hypoxemia (oxygen saturation [SaO2] <89% or PaO2 <55 mm Hg while breathing room air).
- If inhalational exposures are thought to be the etiology, as in HP and occupational forms of interstitial lung disease, removal from exposure is mandatory.
- If a toxic medication is suspected, its discontinuation is mandated.
- Respiratory infections should be promptly treated.
- Pharmacologic therapy with corticosteroids (eg, prednisone) and/or cytotoxic agents for their potential steroid-sparing effect (eg, cyclophosphamide, azathioprine, methotrexate) may be indicated for specific diagnoses.
- Other immunosuppressive or antifibrotic agents such as colchicine, cyclosporine, and D-penicillamine may have a role in specific cases. Empiric use of these medications without a specific diagnosis should be discouraged because they have significant toxicities.
- Interferon-gamma-1b,8 pirfenidone,9 and acetylcysteine10 have been studied for the treatment of IPF. Interferon-gamma-1b initially appeared to have a favorable effect. This, however, was not supported in a larger follow-up study. Some evidence suggests that pirfenidone and acetylcysteine may have some benefit in IPF. Further investigation is still needed, particularly in other forms of DPLD.
- A 2008 multisystem, randomized, controlled study of bosentan over a period of 4 weeks did not show superiority over placebo; however, a trend toward superiority was noted, which was more pronounced in patients with UIP documented by surgical biopsy.11
Surgical Care
- Surgery in the form of either thoracoscopic (preferred) or open lung biopsy is indicated to obtain tissue specimens for definitive diagnosis.
- More recently, lung transplantation has become a treatment option for selected patients with advanced disease.12
- Survival rates worldwide after single lung transplantation are approximately 74% at 1 year, 58% at 3 years, 47% at 5 years, and 24% at 10 years.
- Survival rates are lower for bilateral lung transplantation.
- Following transplantation, patients overall report improved quality of life with better physical, social, and general health functioning.
Consultations
- Consider consultation with a pulmonary or occupational disease specialist for patients with suspected DPLD.
Diet
- No specific dietary restrictions are warranted for affected patients.
- Some suggest that antioxidants have a therapeutic benefit.
Activity
- Encourage exercise and pulmonary rehabilitation because they may improve a patient's functional status. However, these activities generally have no effect on disease progression.
Medication
Medications are best used for specific diagnoses. However, corticosteroids, cytotoxic agents, and, more recently, antifibrotics, antioxidants, and other immunosuppressive agents have been used with varying success in some forms of DPLD.
In general, NSIP, DIP, and COP have been found to be more responsive to corticosteroids and immunosuppressive therapies. UIP is generally thought to be unresponsive to these modalities, and thus, additional research in the form of clinical trials evaluating potentially promising agents continues. RBILD responds to smoking cessation.
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, modify body's immune response to diverse stimuli.
Prednisone (Deltasone, Meticorten, Orasone, Sterapred)
Used as immunosuppressant in treatment of autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation. Oral corticosteroid with relatively less mineralocorticoid activity.
Best prescribed in consultation with a pulmonary disease specialist.
Adult
1-1.5 mg/kg/d PO up to 100 mg/d to start, followed by a taper after 2-4 wk to a maintenance dose of 0.25-0.5 mg/kg/d PO
Pediatric
Not established
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity, viral infection, peptic ulcer disease, hepatic dysfunction, connective-tissue infections, fungal or tubercular skin infections, GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur
Cytotoxics
Used for some of their immunosuppressant properties.
Cyclophosphamide (Cytoxan, Neosar)
Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells of immune system. Possibly a steroid-sparing medication.
Adult
20-125 mg/d IV
Pediatric
Not established
Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones
Chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Documented hypersensitivity, severely depressed bone marrow function
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; opportunistic infections may occur
Azathioprine (Imuran)
Inhibits mitosis and cellular metabolism by antagonizing purine metabolism and inhibiting synthesis of DNA, RNA, and proteins. These effects may decrease proliferation of immune cells and result in lower autoimmune activity. Possibly a steroid-sparing medication.
Adult
3 mg/kg/d PO; not to exceed 200 mg/d
Pediatric
Not established
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; fever and drug-induced pulmonary fibrosis may occur; opportunistic infections may occur
Anti-inflammatories
Immunosuppressive effects may inhibit cellular division and fibrosis.
Colchicine
Decreases leukocyte motility and phagocytosis observed in inflammatory responses.
Adult
0.6 mg/d PO
Pediatric
Not established
Sympathomimetic agent toxicity and effect of CNS depressants are significantly increased
Documented hypersensitivity; severe renal, hepatic, GI, or cardiac disorders; blood dyscrasias
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Adverse effects include risk of renal failure, hepatic failure, permanent hair loss, bone marrow suppression, numbness or tingling in hands and feet, disseminated intravascular coagulopathy, and decreased sperm count
More on Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
| Overview: Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
| Differential Diagnoses & Workup: Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
 Treatment & Medication: Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
| Follow-up: Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
| Multimedia: Pulmonary Fibrosis, Interstitial (Nonidiopathic) |
| References |
| « Previous Page | Next Page » |
References
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Further Reading
Keywords
interstitial lung disease, ILD, diffuse parenchymal lung disease, DPLD, diffuse lung disease, DLD, fibrotic lung disease, occupational interstitial lung disease, autoimmune pulmonary fibrosis, desquamative interstitial pneumonia, DIP, eosinophilic pneumonia, EP, respiratory bronchiolitis interstitial lung disease, RBILD, acute interstitial pneumonitis, AIP, Hamman-Rich syndrome, nonspecific interstitial pneumonia, NSIP, bronchiolitis obliterans-organizing pneumonia, BOOP, cryptogenic organizing pneumonia, COP, lymphocytic interstitial pneumonia, LIP, giant cell pneumonitis, granulomatous pneumonitis, sarcoidosis, hypersensitivity pneumonia, HSP, extrinsic allergic alveolitis, tuberous sclerosis, pulmonary Langerhans cell histiocytosis, PLCH, lymphangioleiomyomatosis, LAM
