Current Respiratory Medicine Reviews - Volume 9, Issue 4, 2013

Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upperlobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough. Chest pain because of pneumothorax may be the first symptom in some patients. Patients with PPFE are slender with a flat rib cage or abnormally narrowed anterior–posterior thoracic dimension. Decreases in forced vital capacity, total lung capacity, and diffusing capacity are respiratory-function characteristics of PPFE, similar to those seen in idiopathic pulmonary fibrosis (IPF). The most remarkable difference in clinical features between PPFE and IPF is imaging findings, with upper-lobe-predominant lesions in PPFE and lower-lobe-predominant lesions in IPF.

Pleuroparenchymal fibroelastosis (PPFE) is a distinct pattern of pulmonary fibrosis which often runs a rapidly progressive course with a poor prognosis, and it is likely to be introduced as a separate entity in the new classification scheme of idiopathic interstitial pneumonias. It is characterised by pleural fibrosis and subpleural fibroelastosis, with an upper lobe predominance. In addition to cases following lung and bone marrow transplantation, familial and idiopathic cases have been described. The literature on PPFE is fragmented, however, and primarily consists of small case series, lacking a uniform methodology of clinical, radiological and histopathological description. In this review article, most previously published reports of PPFE in the English-language literature will be discussed and the salient clinical and histopathological data analysed to arrive at a working definition of PPFE in daily histopathological practice, and to aid the generation of a unifying hypothesis regarding its potential aetiologies and pathogenesis.

Pleuroparenchymal fibroelastosis (PPFE) is a clinicopathologic entity characterized by clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, radiologic features of pleural and parenchymal involvement accentuated in the upper lobes, and a constellation of histologic findings including visceral pleural fibrosis and prominent fibroelastosis of the subpleural lung parenchyma. While the large majority of cases of PPFE have been considered idiopathic, development of PPFE in post-bone marrow transplant patients and in lung transplant recipients has recently been reported. Further, with the growing number of idiopathic PPFE cases reported in the literature, interesting patterns of association between PPFE and various clinical conditions – including prior treatment for malignancy, autoimmunity, recurrent infections, and vascular compromise/ischemia – are beginning to emerge. These associations, reviewed here, may offer clues into the pathogenesis of this rare condition.

Air leaks after thoracic surgeries have been traditionally managed by the closed thoracotomy tube drainage. In some cases, the leak remains persistent requiring repeated surgical procedures. The IBV® valve has initially been investigated as an alternative to lung volume reduction surgery (LVRS). Recently the Food and Drug Administration has approved it in the control of prolonged air leaks, or persistent leaks following lobectomy, segmentectomy, or LVRS. We present a case of a persistent air leak following LVRS that underwent successful placement of IBV® valve via flexible bronchoscopy under conscious sedation with rapid resolution of the air leak.

The chronic inflammation associated with asthma leads to remodeling of the lung resulting in bronchoconstriction from airway hyperresponsiveness and progressive airway narrowing. Although the inflammation related to asthma affects the epithelial lining, basement membrane and vascular supply of the airway, its affect on the airway smooth muscle ultimately leads to both acute bronchoconstriction and progressive airway obstruction. Current therapies, including inhaled corticosteroids and long-acting &bgr2-adrenergic agonists, do not conclusively alter the remodeling of the lung tissue related to asthma. The direct application of thermal energy to the airway, termed bronchial thermoplasty, has been demonstrated to reduce the thickness and responsiveness of airway smooth muscle, as well as reduce the incidence of acute exacerbations related to bronchoconstriction and improve progressive airway obstruction.

Asthma is a pathophysiological condition associated with eosinophilia and heightened inflammatory reactions, epithelial desquamation, hyper responsiveness, recurring episodes of reversible broncho-constriction, and mucus hyper secretion from the respiratory organs. Asthma interludes can be triggered by a variety of factors most notably allergens, infectious agents, pollutants, and nonspecific stimuli such as exercise, nutritional factors and emotional stress. Certain genes and their polymorphism may also have effects in the initiation and progression of asthma. Thus asthma may also be classified as atopic (extrinsic) or non-atopic (intrinsic). Here atopy refers to a predisposition towards developing type 1 hypersensitivity reactions. In a present day scenario asthma is termed as a complex genetic disorder with a heterogeneous phenotype, largely attributed to the interactions between genes and/or environment. Hence, pharmacogenomics is an important area to understand the influence of genetic variations on drug response in asthmatics by correlating gene expression or single-nucleotide polymorphisms (SNPs) with drug's efficacy. This article reviews the role of various genes and their polymorphism in initiation, complication of asthma and its treatment strategies focussing on disease causing polymorphic and novel genes.

Thickening of the basement membrane zone (BMZ) is a characteristic feature of airway remodeling in the lungs of asthmatics. However the significance of a thickened BMZ in the pathology of the asthmatic airway is not known. In this review we show that the columnar epithelium is linked to the reticular BMZ through the supracellular anchoring network. We discuss the evidence that changes in the width of the BMZ in control airways are part of a supracellular anchoring mechanism for increasing the strength of attachment between the airway epithelium and the extracellular matrix (ECM). We then review the effects of asthma on this anchoring mechanism. We conclude that both thickening of the BMZ and sloughing of columnar epithelium (creola bodies) in asthma represent abnormalities in the supracellular anchoring network attaching the airway epithelium to the ECM. Future research directed toward studying the regulation and development of the supracellular anchoring network may help better understand sloughing of columnar epithelium and the significance of reticular BMZ thickening in the asthmatic airway.

Background: OSA is a common disorder with significant neuropsychiatric morbidity. Studies have shown a 20% prevalence of depression in OSA patients. Prevalence of anxiety disorders in OSA has not been well characterized. Objective: The objective of this study was to examine the prevalence of depression and anxiety disorders in a cohort of patients with OSA, and evaluate the influence of gender and ethnicity. Methodology: A retrospective study was carried out on consecutive patients referred for polysomnography at our tertiary care county hospital sleep center. Patients were administered the Hospital Anxiety and Depression Scale (HADS) in addition to routine sleep questionnaires. Scores of >10 were considered positive for both anxiety, and for depression respectively. Patients over age 18 years and AHI > 5 on the sleep study were included in the analysis. Results: 51 patients meeting the inclusion criteria filled out a valid questionnaire. The prevalence of both anxiety, as well as depression, was very high (52.9 % and 39.2% respectively). There was a significantly higher prevalence of anxiety in females as compared to males. (62.5% vs 36.8% respectively; p < 0.05). African-American patients had higher rates of anxiety than Caucasians (66.7% vs 30.7%; p < 0.05). No gender or ethnic variance was seen in prevalence of depression. Conclusions: Anxiety, as well as depression, are extremely prevalent in patients with OSA, and are significantly underappreciated. Our data suggest that both gender and ethnicity may influence the prevalence of anxiety in OSA.