Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Jun 2016
ReviewNonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?
We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. ⋯ Second, the "highly fibrotic" subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into "full-blown idiopathic pulmonary fibrosis." The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients.
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Semin Respir Crit Care Med · Jun 2016
ReviewLymphoid Interstitial Pneumonia and Other Benign Lymphoid Disorders.
Benign pulmonary lymphoid disorders include a variety of rare lymphoid abnormalities characterized by a polyclonal lymphoid infiltrate with differing histopathologic patterns and clinicoradiologic features that may overlap. Histological examination is essential to reach a correct diagnosis and to exclude alternative causes, although this task can at times prove difficult. Further studies and clinical trials are needed to provide additional insights on pathogenesis and to guide the therapeutic management of these disorders. The purpose of this article is to review the histopathological, epidemiological, and clinicoradiologic features of several benign pulmonary lymphoid disorders, including lymphoid interstitial pneumonia, follicular bronchiolitis, nodular lymphoid hyperplasia (pulmonary pseudolymphoma), inflammatory pseudotumor, immunoglobulin G4-related disease, Castleman disease, posttransplantation lymphoproliferative disease, and drug-induced lymphoid disorders.
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Semin Respir Crit Care Med · Jun 2016
ReviewRecent Advances and Future Needs in Interstitial Lung Diseases.
Interstitial lung diseases (ILDs) are a diverse range of conditions affecting the lung interstitium. The prototypic ILD, idiopathic pulmonary fibrosis (IPF), is a chronic progressive fibrotic lung disease with a median survival of only 3 years from the time of diagnosis. Recently significant progress has been made in both our understanding of the pathogenesis and of the therapeutic targeting of IPF. ⋯ While an important first step, patients continue to progress and better therapies are urgently required. The aim of this article is to highlight some of the recent advances that have been made in our understanding of genetics, disease classification, clinical trial design, and novel antifibrotic therapy in IPF. It discusses future priorities if we are to continue to increase the length and quality of life of patients with IPF, and considers possible approaches to translate the progress made in IPF to other progressive fibrotic lung diseases where our understanding remains limited.
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Semin Respir Crit Care Med · Jun 2016
ReviewWhat We Know About the Pathogenesis of Idiopathic Pulmonary Fibrosis.
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease of unknown cause, occurring in adults, limited to the lungs and associated with the pathologic and radiologic pattern of usual interstitial pneumonia. Prognosis is poor, and most patients die of respiratory failure within 3 to 6 years from the onset of symptoms. Although our understanding of the pathogenesis of IPF has improved over the past two decades, the mechanisms responsible for this disorder have not been clearly defined. ⋯ Unfortunately, improvement or cure has not yet been achieved with pharmacological agents. The challenge for the future is to improve the comprehension of the mechanisms involved in the inception and evolution of IPF and their articulated interactions. This is fundamental not only to conceive and develop new drugs against this dreadful disease but also to apply different therapeutic approaches such as drug repositioning and personalized therapies in the management of IPF.
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Semin Respir Crit Care Med · Jun 2016
ReviewHypersensitivity Pneumonitis: Challenges in Diagnosis and Management, Avoiding Surgical Lung Biopsy.
This review presents an update of the currently available information related to hypersensitivity pneumonitis, with a particular focus on the contribution of several techniques in the diagnosis of this condition. The methods discussed include proper elaboration of a complete medical history, targeted auscultation, detection of specific immunoglobulin G antibodies against the most common antigens causing this disease, skin tests, antigen-specific lymphocyte activation assays, bronchoalveolar lavage, and cryobiopsy. Special emphasis is placed on the relevant contribution of specific inhalation challenge (bronchial challenge test). Surgical lung biopsy is presented as the ultimate recourse, to be used when the diagnosis cannot be reached through the other methods covered.