Chest

Chest

Volume 113, Issue 1, January 1998, Pages 192-202
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Reviews
Diagnostic and Management Strategies for Diffuse Interstitial Lung Disease

https://doi.org/10.1378/chest.113.1.192Get rights and content

Patients with diffuse interstitial lung diseases (DILD) are challenging to treat. Many patients with DILD have inadequate information about the disease process, an imprecise diagnosis, unsatisfactory treatment or unacceptable side effects associated with therapy, and poorly controlled symptoms of progressive illness. Establishing an accurate diagnosis is necessary so that the patient and his/her family can be provided with reasonable expectations about prognosis and outcome from therapy. A pragmatic approach is presented that emphasizes diagnostic strategies and plans for therapy that are effective and resource efficient and that will help maintain patient satisfaction.

Section snippets

Patient Base and Spectrum of Diagnoses

Patients with suspected or already diagnosed DILD were evaluated on referral for further disease management. These patients were not enrolled in ongoing research protocols about underlying mechanisms of disease.1, 2, 3, 4, 5 In Table 1, 100 consecutively evaluated patients with various forms of DILD who had been seen over a prior 10-year interval were assembled as of 1988 (group 1) and contrasted with 60 similar referral patients (group 2) evaluated from 1989 through 1996. Group 1 included

Approach to Diagnosis

On referral, about half of the patients (Table 1) carried a diagnosis usually based on tissue histology. Even with this assurance, a brief reassessment was worthwhile, following the algorithm in Figure 1. Occasionally a diagnosis seemed flawed and needed to be challenged and even changed. Establishing a confident diagnosis for patients without one was essential, because lack of a precise diagnosis was often the basis of the patient's discontent. The absence of a firmly established diagnosis

Ancillary Diagnostic Tests

Although several serology and enzyme tests can be useful in confirming a diagnosis or for monitoring response to treatment, for example converting enzyme in sarcoidosis, these will not be discussed. Instead three procedures will be reviewed: high-resolution CT scanning (HRCT) of the chest, bronchoscopy to obtain endobronchial or transbronchial biopsy specimens and BAL fluid, and lung biopsy performed by video-assisted thoracoscopy (VATS).

Review of prior chest radiographs is indispensable, as

Management of Patients With DILD

Most forms of DILD rarely remit and feature periods of exacerbation superimposed on a chronic or worsening baseline of symptoms. Exceptions may be DILD caused by aerosolized organic antigens or a medication reaction. In these cases, avoidance constitutes therapy. Desquamative interstitial pneumonitis (DIP) may resolve spontaneously,18 or cessation of cigarette smoking may stabilize LCG.10 Other forms of DILD usually persist for many years. Add the dysfunction of other critical organs—especially

Therapy to Suppress Alveolitis, Bronchiolitis, and the Immunologic Processes Promoting Fibrosis

For the consultant, there can be a perceived expectation that the patient or referring physician wants a bold decision about therapy: either to initiate it, or change what is not working, or suggest that it be discontinued. About half of my referral patients (Table 1) were in this situation—already receiving treatment but finding results unsatisfactory and complications distressing. Those not yet started on a regimen of therapy were apprehensive that their illness was progressing, but feared

Ancillary Measures and Care

Treatment of other medical problems is almost as important as suppression of alveolitis with anti-inflammatory drugs. Good control of these makes a great deal of difference in the patient's quality of life and satisfaction. Nine frequently encountered problems are listed in Table 2. More information about DILD is often requested by the patient. This request can be met by illustrating the affected lung structures with a cartoon drawing and providing some articles about the illness. For the

Monitoring Response to Therapy

Controversy remains about which tests are most effective for judging natural progression of DILD or assessing therapeutic improvement. Unfortunately, monitoring disease activity does not have precise indicators. Many investigators had hoped that assessing inflammation by cellular characteristics in BAL fluid6, 7, 8, 9, 13 or by gallium scanning6, 86 would identify which patients were responding to therapy and objectively guide subsequent treatment. This did not occur, and more research is still

Acknowledgment

Susan Crawford assisted with manuscript preparation. Anne H. Hawkins, PhD, Department of Humanities, provided editorial review.

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    Presented at CHEST 1996, the 62nd Annual International Scientific Assembly of the American College of Chest Physicians, October 28, 1996, San Francisco.

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    Copyright © 1998 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.