Idiopathic pulmonary fibrosis

2010 ◽  
pp. 3375-3382
Author(s):  
A.U. Wells ◽  
A.G. Nicholson ◽  
N. Hirani
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Features ◽  
Clinical Syndrome ◽  
Lung Disorder ◽  
Fibrosing Alveolitis ◽  
Cryptogenic Fibrosing Alveolitis

The synonymous terms idiopathic pulmonary fibrosis (IPF) and cryptogenic fibrosing alveolitis (CFA) refer to a relentlessly progressive fibrotic lung disorder that is the underlying diagnosis in over one-half of patients presenting with typical clinical features of the ‘CFA clinical syndrome’ (see Chapter 18.11.1). Incidence is about 10 to 15 per 100 000, men are more often affected than women, and it most commonly presents in the seventh and eighth decades. Aetiology remains uncertain....

Autoimmunity associated with cardiac, respiratory, and renal disease

2013 ◽  
pp. 201-212
Author(s):  
Gavin P Spickett
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Renal Disease ◽  
Rheumatic Fever ◽  
Respiratory Disease ◽  
Cardiac Disease ◽  
Interstitial Pneumonitis ◽  
Fibrosing Alveolitis ◽  
Cryptogenic Fibrosing Alveolitis ◽  
Alveolar Proteinosis

Cardiac disease 1: myocarditis and cardiomyopathy Cardiac disease 2: eosinophilic syndromes Cardiac disease 3: recurrent pericarditis and Dressler’s syndrome Cardiac disease 4: rheumatic fever Respiratory disease 1: idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) Respiratory disease 2: pulmonary alveolar proteinosis (PAP) Respiratory disease 3: lymphoid interstitial pneumonitis and sarcoidosis...

A Comparison of the Clinical Features of ANCA-Positive and ANCA-Negative Idiopathic Pulmonary Fibrosis Patients

Respiration ◽  
2009 ◽  
Vol 77 (4) ◽  
pp. 407-415 ◽  
Author(s):  
Tomoko Nozu ◽  
Mitsuko Kondo ◽  
Kazuo Suzuki ◽  
Jun Tamaoki ◽  
Atsushi Nagai
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Features

Idiopathic Pulmonary Fibrosis

2017 ◽  
Author(s):  
Lawrence A Ho ◽  
Bridget F Collins ◽  
Ganesh Raghu
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Pulmonary Physiology ◽  
Cryptogenic Fibrosing Alveolitis ◽  
Progressive Dyspnea ◽  
Chest X Ray ◽  
Sixth Decade

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a chronic and progressive fibrosing interstitial pneumonia of unknown cause that typically manifests after the fifth to sixth decade of life. The fibrosis is limited to the lung, and clinical features include progressive dyspnea and a restrictive pulmonary physiology. IPF is characterized by a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and histopathology. This review discusses the definition, epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis of IPF. Figures depict the pathogenesis of IPF, a chest x-ray in a patient with IPF, HRCT scans, and histopathologic features of UIP. Tables list HRCT criteria for UIP patterns, the elements required for IPF diagnosis, and the GAP model for IPF prognosis. This review contains 8 highly rendered figures, 3 tables, and 108 references.

Sign in / Sign up

Export Citation Format

Share Document