Seminars in respiratory and critical care medicine
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Significant progress has been made in elucidating the genetics of parenchymal lung diseases, particularly idiopathic interstitial pneumonias (IIPs). IIPs are a heterogeneous group of diffuse interstitial lung diseases of uncertain etiology, diagnosed only after known causes of interstitial lung disease have been excluded. ⋯ Through candidate gene approaches and genome wide association studies, much light has been shed on the genetic origins of IIPs, enhancing our understanding of risk factors and pathogenesis. However, significant work remains to be accomplished in identifying novel genetic variants and characterizing the function of validated candidate genes in lung pathobiology, their interplay with environmental factors, and ultimately translating these discoveries to patient care.
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Medical therapy for idiopathic fibrosis remains controversial. Idiopathic pulmonary fibrosis (IPF) was uniformly a disease that progressed inexorably, typically leading to death within 3 to 5 years from onset of symptoms. Until recently, lung transplantation was the only effective transplant option. ⋯ Nonetheless, these agents bring a glimmer of hope to patients with this deadly disease. The appropriate indications for initiating therapy, best candidates for therapy, and possible role for combination therapy remain controversial. Additional studies using agents that attenuate or abrogate profibrotic cytokines and chemokines may provide even further improvement in the future.
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Semin Respir Crit Care Med · Jun 2016
ReviewIdiopathic Pulmonary Fibrosis: Epidemiology, Clinical Features, Prognosis, and Management.
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic interstitial lung pneumonia associated with the histologic pattern of usual interstitial pneumonia (UIP). Although UIP is a distinct histologic lesion, this histologic pattern is not specific for IPF and can also be found in other diseases (e.g., connective tissue disease and asbestosis). Clinical features of IPF include progressive cough, dyspnea, restrictive ventilatory defect, and progressive fibrosis and destruction of the lung parenchyma. ⋯ Mean survival from the onset of symptoms approximates 3 to 5 years. Medical treatment is only modestly effective, primarily by slowing the rate of disease progression. Lung transplantation is the best therapeutic option.
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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.