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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls. The aim of this study was to review cases fulfilling published imaging and histological criteria, and identify any common clinical features that may suggest an underlying aetiology for a condition that has previously been regarded as idiopathic. Of 12 patients (seven females, median age 57 yrs), the presenting symptoms were shortness of breath (11 out of 12 patients) and dry cough (six out of 12 patients). Seven patients reported recurrent infections during the course of their disease. Five demonstrated nonspecific autoantibody positivity. Two patients had a family history of interstitial lung disease (ILD). High-resolution computed tomography features of lung disease remote from the pleuroparenchymal changes were present in six out of 12 patients (coexistent fibrosis, n=5; bronchiectasis, n=1). Of seven patients with tissue sampled from the lower lobes, four patients showed less intense PPFE changes (one with additional features of hypersensitivity pneumonitis) and three showed usual interstitial pneumonia. PPFE is a distinct clinicopathological entity, with clinical data suggesting a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms may also contribute to the development of these changes. PPFE may also present with more diffuse involvement than previously reported, and coexist with different patterns of ILD.
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PMID:Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. 2244 48

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.
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PMID:Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. 2457 77

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare, recently classified entity that consists of pleural and subjacent parenchymal fibrosis predominantly in the upper lungs. In an official American Thoracic Society/European Respiratory Society statement in 2013, this disease is introduced as a group of rare idiopathic interstitial pneumonias. We describe a case of a 76-year-old woman with cough and recurrent pneumothorax. She was admitted to our hospital with severe cough at first. High resolution computed tomography (HRCT) disclosed multifocal subpleural consolidations with reticular opacities in both lungs, primarily in the upper lobes, suggesting interstitial pneumonia. Rheumatoid lung was diagnosed initially through an elevated rheumatoid factor, HRCT and surgical biopsy at the right lower lobe. However, one month later, pneumothorax recurred. Surgical biopsy was performed at the right upper lobe at this time. The specimens revealed typical subpleural fibroelastosis. We report this as a first case of idiopathic PPFE in Korea after reviewing the symptoms, imaging and pathologic findings.
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PMID:Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax: a case report. 2536 65

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management.
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PMID:Pleuroparenchymal fibroelastosis: is it also an idiopathic entity? 2609 64

Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.
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PMID:Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias. 2638 Jan 41

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit.
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PMID:Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report. 2681 Jul 62

We report the first patient with pleuroparenchymal fibroelastosis (PPFE) to undergo living donor bilateral lobar lung transplantation. The patient was diagnosed with secondary PPFE as a late complication of chemotherapy that included high-dose cyclophosphamide for mature B-cell lymphocytic leukemia. Although the patient maintained complete remission, dry cough and back pain appeared 8 years after the chemotherapy. He had repeated bilateral pneumothoraces, and his respiratory condition gradually deteriorated because of progressive pleural thickening and parenchymal fibrosis. He underwent living-donor bilateral lobar lung transplantation with an inverse transplant on the left side.
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PMID:Living Donor Lung Transplantation for Pleuroparenchymal Fibroelastosis. 2710 30

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recognized as a rare new entity. We report the case of a 63 years old female suffering from progressive dyspnea and dry cough for three years. Two years before admission to our hospital, idiopathic pulmonary fibrosis (IPF) was diagnosed in another hospital and treatment with prednisolone and N-acetylcysteine (NAC) was commenced. At admission HRCT showed upper lobe dominant fibrosis and associated pleural thickening. Surgical biopsies were re-evaluated and revealed fibroelastosis with pleural thickening and a probable UIP pattern, consistent with idiopathic PPFE. Treatment with pirfenidone was initiated due to progression under prednisolone and NAC. Upper lobe predominant pleural thickening with associated subpleural fibrotic changes should raise suspicion of PPFE.
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PMID:Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity. 2878 Sep 89

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. The patient was admitted due to a 9-year history of progressive cough and exertional dyspnea, as well as a history of Hodgkin's lymphoma treated by autologous hematopoietic stem cell transplantation (HSCT). The patient had been initially diagnosed with tuberculosis and received regular anti-tuberculosis therapy for 18 months; however, the symptoms progressed. Serial chest computed tomography scans indicated a gradually worsening diffuse pleural thickening, dense subpleural opacification and volume loss, associated with evidence of fibrosis in the right lung. On physical examination the patient was cachectic, with a body mass index of 18.5 kg/m2, and he had a flattened thoracic cage. Arterial blood gas analysis revealed hypoxia. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. The microbiological and cytological examinations were negative. Lung biopsy revealed a thickened pleura consisting of large amounts of collagen and elastic fibers, coexisting with subpleural intra-alveolar fibrosis with alveolar septal elastosis, without inflammatory infiltrates. The patient was diagnosed with PPFE secondary to HSCT and eventually succumbed to respiratory failure and infection while waiting for a lung transplant. Physicians should be aware of the typical and atypical characteristics of this rare disease, as its clinical and radiological characteristics may lead to misdiagnosis, particularly as chronic infections. The prognosis remains poor without effective long-term treatment.
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PMID:Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report. 3090 45

Characterized by pleural and subpleural fibrosis with alveolar septal elastosis, pleuroparenchymal fibroelastosis is a rare restrictive lung disease. Symptoms are often subtle, including dyspnea, cough, and weight loss; while acute presentations of spontaneous pneumothorax have been recorded. We report a patient who developed a spontaneous hemothorax, who upon evacuation of the chest was found to have a hemorrhagic lower lobe mass consistent with pleuroparenchymal fibroelastosis. Various conditions are associated with pleuroparenchymal fibroelastosis, suggesting chronic lung injury as a factor in pathogenesis. Hemothorax of this magnitude with relatively no known inciting risk factors, represents an exceptionally rare case presentation.
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PMID:Isolated Left Lower Lobe Pleuroparenchymal Fibroelastosis Presenting as Hemothorax. 3192 59


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