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...

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....

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.

Idiopathic pulmonary fibrosis

2020 ◽  
pp. 4177-4185
Author(s):  
P.L. Molyneaux ◽  
A.G. Nicholson ◽  
N. Hirani ◽  
A.U. Wells
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Functional Decline ◽  
Usual Interstitial Pneumonia ◽  
Supportive Therapy ◽  
Productive Cough ◽  
Radiological Imaging ◽  
Exertional Dyspnoea ◽  
Digital Clubbing ◽  
Cryptogenic Fibrosing Alveolitis

The synonymous terms idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis refer to a relentlessly progressive fibrotic lung disorder. Incidence is about 5–15 per 100,000, men are more often affected than women, and it most commonly presents in the seventh and eighth decades. Aetiology remains uncertain. Typical presentation is with progressive exertional dyspnoea without wheeze, a non-productive cough, digital clubbing, and very fine end-inspiratory crackles. Central cyanosis and clinical evidence of pulmonary hypertension are late features. Diagnosis depends on careful exclusion of known causes of interstitial lung disease, followed by demonstration by radiological imaging or biopsy of the pathognomonic lesion of usual interstitial pneumonia. Two antifibrotic compounds, pirfenidone and nintedanib, have been proven to slow functional decline in idiopathic pulmonary fibrosis. Lung transplantation is appropriate in selected cases. Supportive therapy is central to the management of advanced disease. Five-year survival is 10–15%.

Interferon Gamma-1b in the Treatment of Idiopathic Pulmonary Fibrosis

2005 ◽  
Vol 39 (10) ◽  
pp. 1678-1686 ◽  
Author(s):  
Michael A Pacanowski ◽  
Guy W Amsden
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interferon Gamma ◽  
Clinical Studies ◽  
Response To Therapy ◽  
Treatment Modalities ◽  
Clinical Aspects ◽  
Medline Search ◽  
Cryptogenic Fibrosing Alveolitis ◽  
Study Results

Objective: To examine the clinical aspects of idiopathic pulmonary fibrosis (IPF) and the efficacy and safety of interferon gamma-1b (IFNγ−1b) in its treatment. Data Sources: Epidemiologic, preclinical, and clinical studies published in the English language were identified by a MEDLINE search (1966–January 2005) using the search terms idiopathic pulmonary fibrosis, cryptogenic fibrosing alveolitis, and interferon. Additional citations were identified from the reference lists of related publications. Study Selection and Data Extraction: Selected preclinical studies describing the pathophysiologic basis for IFNγ−1b therapy and all clinical studies were included. Additional trials describing other treatment modalities and the determinants of response to therapy in patients with IPF were also reviewed. Data Synthesis: IFNγ−1b targets the fibrotic rather than inflammatory processes of IPF. The efficacy of IFNγ−1b in patients with IPF is inconsistent with regard to changes in pulmonary function and mortality, although a modest survival benefit was observed in the largest clinical trial. Adverse events related to IFNγ−1b are frequent although transient. Several cases of respiratory failure occurring subsequent to the administration of IFNγ−1b are documented. CONCLUSIONS: To date, although trials suggest that earlier-stage IPF may be responsive to IFNγ−1b, study results overall are inconsistent; further investigation is needed.

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