Diagnosis and management of interstitial lung disease.

Keith C Meyer

doi:10.1186/2213-0802-2-4

Abstract

The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life. A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies. However, ILD accompanied by extensive fibrosis may be difficult to treat, and the identification of an effective pharmacologic therapy for idiopathic pulmonary fibrosis (IPF) has remained elusive despite the completion of many phase 3 clinical trials over the past decade. Nonetheless, patients with IPF or advanced forms of non-IPF ILD can benefit significantly from detection and treatment of various co-morbid conditions that are often found in patients (especially the elderly patient), and supportive care (oxygen therapy, pulmonary rehabilitation) can have a beneficial impact on quality of life and symptom palliation. Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation.

Highlights

Well over one hundred different forms of interstitial lung disease (ILD) have been described
Some forms of ILD have been linked to specific genetic abnormalities (e.g. Hermansky-Pudlak syndrome, familial pulmonary fibrosis), and a number of gene variants have been associated with an increased risk to develop ILD disorders such as idiopathic pulmonary fibrosis (IPF), sarcoidosis, or chronic beryllium disease (CBD)
The diagnosis and treatment of the various types of ILD present a considerable challenge to clinicians

Summary

Introduction

Well over one hundred different forms of interstitial lung disease (ILD) have been described (see Table 1 for major categories). Treatment of any form of ILD with immunosuppressive therapy is off-label in the U.S and anti-inflammatory/ immunosuppressive pharmacologic therapy has not been validated in placebo-controlled clinical trials, there is reasonably compelling evidence that the administration of agents such as corticosteroids is strongly associated with improvement or even clearing of lung pathology for many forms of ILD This is the case for disorders such as cryptogenic organizing pneumonia (COP), eosinophilic pneumonia, sarcoidosis, or cellular non-specific interstitial pneumonia (NSIP) [36]. An improved understanding of the genetics and genomics of ILD will likely lead to the identification of new therapies that may have a significant treatment effect that relieves symptoms and restores quality of life for patients with significant, progressive ILD, but such therapies should have minimal risk of precipitating adverse reactions that can abrogate the benefits of pharmacotherapy

Conclusions

45. American Thoracic Society

Findings

67. Meyer KC