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Current approach to connective tissue disease-associated interstitial lung disease

imagePurpose of review : To review current approaches to the complex intersection of interstitial lung disease (ILD) with the spectrum of connective tissue disease (CTD).
Recent findings : There is a growing appreciation that the approach to CTD-associated ILD (CTD-ILD) can be enhanced by a multidisciplinary evaluation that often incorporates the rheumatologist. Determining that ILD is associated with an established CTD requires the exclusion of alternative causes and thorough assessments of the clinical features of both the CTD and ILD. The detection of occult CTD in patients with presumed ‘idiopathic’ disease requires careful attention to the demographic profile, historical clues, subtle physical examination findings, specific autoantibody positivity, radiologic, and histopathologic features. A comprehensive treatment program for CTD-ILD should address comorbid conditions and consider implementation of adjunctive therapeutic strategies. Pharmacologic intervention for CTD-ILD is reserved for those with progressive, clinically significant disease and typically involves use of immunosuppressive therapies.

Summary : A multidisciplinary approach can be helpful for CTD-ILD. Further research and controlled trials are needed to determine how to best manage the diverse spectrum of CTD-ILD.

Treatment strategies for idiopathic interstitial pneumonias

imagePurpose of review : With recent changes in diagnostic algorithms in idiopathic pulmonary fibrosis (IPF) guidelines and treatment advances in IPF, it is now necessary to reappraise the way in which clinicians should formulate treatment strategies in the idiopathic interstitial pneumonias.

Recent findings : The idiopathic interstitial pneumonias can usefully be subdivided into the following categories: first, definite IPF, second, probable IPF with major differential diagnoses of fibrotic nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis and third, apparently idiopathic interstitial pneumonias other than IPF. In definite IPF, the therapeutic landscape has irrevocably changed with the identification of robust treatment effects with pirfenidone and nintedanib, consisting of the prevention of approximately 50% of disease progression (as judged by serial forced vital capacity trends). In probable IPF, generally equating with high resolution computed tomography findings of ‘possible usual interstitial pneumonia’ and the nonperformance of a diagnostic surgical biopsy, management is based on multidisciplinary evaluation, integrating all available information, with a final ‘working diagnosis’ made for treatment purposes. In other idiopathic interstitial pneumonias and their major differentials, removal of potential triggers and immunomodulation remain the cornerstones of therapy, with treatment goals usefully designated using a disease behaviour classification. In mild disease, an initial policy of observation is often appropriate.

Summary : The striking recent treatment effects reported in IPF will have major management implications in the idiopathic interstitial pneumonia in general, whenever IPF is in the differential diagnosis.

Acute exacerbations complicating interstitial lung disease

imagePurpose of review : The purpose of this review is to provide an update on acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), with a specific focus on new data regarding the cause, clinical features, management and prognosis of AE-IPF. In addition, the limitations of the current definition of AE-IPF are discussed and a novel classification schema is proposed.
Recent findings : AE-IPF occurs in up to 15% of IPF patients annually and has a mortality of approximately 50%. The incidence of AE-IPF is higher in patients with worse lung function and may be increased in some populations. Emerging data suggest that exacerbations may be secondary to subclinical triggers such as infection, aspiration, mechanical injury and air pollution. Management of AE-IPF typically includes high-dose corticosteroids and antimicrobials; however, there are limited data to support these or other therapies. Prevention of AE-IPF with antifibrotic medications may be feasible and warrants further study.
Summary : AE-IPF is associated with significant morbidity and mortality; however, there remains a paucity of clinical data. The current definition of AE-IPF has limitations and a new classification schema should be considered.

Genetic susceptibility and pulmonary fibrosis

imagePurpose of review : Recent genetic findings have identified new targets of investigation in the field of pulmonary fibrosis and have the potential to change clinical care.
Recent findings : These findings implicate alterations in host defense, cell-to-cell adhesion, and aging and senescence in the pathophysiology of pulmonary fibrosis. At least one common genetic variant strongly associated with pulmonary fibrosis appears to have prognostic implications for patients.
Summary : The inherited risk for pulmonary fibrosis is substantial, and recent data suggest that genetic risk for familial and sporadic forms of the disease are similar.
Further characterizing this genetic risk will influence clinical practice in terms of categorization, diagnosis, and screening of individuals for this disease.

IPF clinical trial design and endpoints

ILDPurpose of review : There remains a dire need for therapies that impact the clinical course of patients with idiopathic pulmonary fibrosis (IPF). Indeed, there is a surge of interest in IPF therapeutics, with many candidate agents in various stages of development. Optimal design and implementation of the appropriate prospective clinical trials are essential to demonstrate clinical efficacy of promising drugs for the treatment of IPF. A key element in the success of such clinical trials is the choice of the best endpoint(s) to match the design of the study.

Recent findings : Although the results of many IPF clinical trials have been disappointing, these trials have provided valuable insights into the epidemiology and natural history of the disease and have sparked debate into the best clinical trial designs and endpoints.

Summary : This review will discuss the various clinical trial endpoints that have been used or proposed with a focus on their potential utility, as well as possible pitfalls that investigators should consider in the design of such studies.

Video abstract : http://links.lww.com/COPM/A13

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