eMedicine Specialties > Pulmonology > Interstitial Lung Diseases
Lymphocytic Interstitial Pneumonia: Treatment & Medication
Updated: Jan 17, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
Medical Care
- Asymptomatic and physiologically unaffected patients may not require treatment.
- Symptomatic patients may require supportive care and immunosuppressives, chiefly corticosteroids. Occasionally, cytotoxic therapy has been used. No controlled treatment trials have been reported. Consider oxygen supplementation based on blood gas and/or exercise oximetry findings.
- Antibiotics are used for associated pulmonary infections.
- In pediatric patients with HIV, empiric treatment for LIP often is initiated based on the findings of subacute dyspnea, mild hypoxemia, and clubbing.
- LIP has been reported to improve with the use of zidovudine alone. Instances of resolution with highly active antiretroviral therapy (HAART) have been reported.2
- Bronchodilators may be used for associated wheezing.
Consultations
- Consultation with a pulmonologist or thoracic surgeon may be necessary to obtain transbronchial biopsy or open lung biopsy, respectively.
- In cases associated with HIV infection, consultation with a specialist familiar with HIV care is recommended.
Activity
- Activity may be limited by exercise-induced oxygen desaturation.
- Perform exercise oximetry to determine if supplementary oxygen is needed.
Medication
Medications should be used in patients who are symptomatic or physiologically compromised. Carefully weigh risks and benefits of immunosuppressive therapy in these patients.
HAART may result in improvement or resolution of LIP in some instances.2
One report describes dramatic improvement in LIP associated with common variable immunodeficiency treated with intravenous immunoglobulin without steroids.8
Corticosteroids
Used when patient is symptomatic and/or has physiologic compromise due to LIP. Risks of infection, osteoporosis, hyperglycemia, weight gain, dermatologic changes, and other potential toxicities should be weighed against any potential benefit.
Prednisone (Deltasone, Orasone, Sterapred)
For use as immunosuppressant in autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.
Doses on lower end of range are prescribed for at least 1 mo and tapered as tolerated. More than 50% respond. In some instances, chronic low-dose suppressive therapy required.
Adult
0.5-1 mg/kg/d PO
Pediatric
Administer as in adults
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 of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Alkylating agents
Are used when disease is refractory to corticosteroid therapy. Should be considered only in cases clearly unresponsive to corticosteroids used in high dosage. Should only be prescribed by physicians familiar with usage and toxicities. Generally prescribed for several weeks at a time; disease manifestations and complete blood count should be monitored.
Chlorambucil (Leukeran)
Reportedly given in pre-HIV era to patients with LIP refractory to corticosteroids. Anecdotal results, efficacy unclear.
Adult
0.1 mg/kg/d PO
Pediatric
Not established
None reported
Documented hypersensitivity; previous resistance to medication
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Causes infertility, is carcinogenic, and causes myelosuppression; lowest possible doses should be used; monitor CBC for neutropenia, lymphopenia, anemia, and thrombocytopenia; dosage is decreased or discontinued if counts fall below normal values; if rash develops, discontinue drug because it may cause severe Stevens-Johnson syndrome; potentially epileptogenic medication and should be administered with care to patients with history of seizure disorder, head trauma, or who receive other epileptogenic agents; monitor for GI bleeding or symptoms
More on Lymphocytic Interstitial Pneumonia |
| Overview: Lymphocytic Interstitial Pneumonia |
| Differential Diagnoses & Workup: Lymphocytic Interstitial Pneumonia |
Treatment & Medication: Lymphocytic Interstitial Pneumonia |
| Follow-up: Lymphocytic Interstitial Pneumonia |
| Multimedia: Lymphocytic Interstitial Pneumonia |
| References |
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References
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Further Reading
Keywords
lymphocytic interstitial pneumonia, LIP, lymphoid interstitial pneumonitis, lymphoid pneumonitis, plasma cell interstitial pneumonitis, pulmonary interstitial infiltration, pseudolymphoma, autoimmune disorders, lymphoproliferative disorders, human immunodeficiency virus, HIV-related LIP, HIV-associated LIP, HIV, AIDS, Epstein-Barr virus, EBV, human T-cell leukemia virus, HTLV type 1, HIV type 1, rheumatoid arthritis, Hashimoto thyroiditis, myasthenia gravis, pernicious anemia, autoerythrocyte sensitization syndrome, chronic active hepatitis, common variable immunodeficiency, Sjögren syndrome, allogeneic bone marrow transplantation, lupus, lymphoma, B-cell CLL/lymphoma 6, BCL-6, zinc finger protein 51
Treatment & Medication: Lymphocytic Interstitial Pneumonia