Idiopathic interstitial pneumonia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Zaghw, M.D. [2];

Overview

As IIPs are heterogenous group of unknown interstitial lung disease with different natural history and clinical course, their management should be based on the clinical subtype. Optimal therapy for IPF is controversial as all currently available medications for IPF are severely limited by the lack of clear understanding of the natural history of IPF, the presence of various forms of study designs; heterogeneous patient groups, disputable diagnostic certainty; variable study duration; differences in medication formulation, dosage, route of administration, and duration of treatment; lack of placebo controls; variable intervals between evaluations and differing types of non quantitative assessment criteria. To date, most of treatment strategies have been based on eliminating or suppressing the inflammatory component. As no pharmacological therapy has been proven a clinical efficacy in altering or reversing the inflammatory process of IPF.

Medical Therapy

The following points are a general summary for the responsiveness of each subtype of IIPs

Chronic Fibrosing IIPs

Idiopathic Pulmonary Fibrosis

• poor response to corticosteroids and cytotoxic drugs

Idiopathic Nonspecific Interstitial Pneumonia

• good response to corticosteroids

Acute/Subacute IIPs

Cryptogenic Organizing Pneumonia

• good response to corticosteroids

Acute Interstitial Pneumonia (Hamman-Rich Syndrome)]

• unknown response to corticosteroids

Smoking-related IIPs

Respiratory Bronchiolitis-Interstitial Lung Disease

• good response to smoking cessation but unknown response to corticosteroids

Desquamative Interstitial Pneumonia

• good response to smoking cessation but unknown response to corticosteroids

General outline of chronic fibrosing IIPs

• Establishment of the diagnosis is the first critical step in management as misdiagnosis can lead to inappropriate initial therapy. The second step is severity stage to guide treatment choices. Finally, a disease management plan is tailored to the disease severity and patient’s preferences. Follow up assessment is needed to refine treatment options with the disease progression. As no therapy has been proven to be efficacious in this disease, management generally includes some combination of supportive care such as supplemental oxygen and pulmonary rehabilitation, consideration for participation in clinical trials, referral for lung transplant evaluation if possible and early detection and management of comorbidities.