Idiopathic pulmonary fibrosis overview

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Idiopathic pulmonary fibrosis Microchapters

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Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Idiopathic pulmonary fibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Historical Perspective

Pulmonary fibrosis was first dexcribed by Dr. Von Buhl in 1872. Dr. Rindfleish reported a case of a 40 year old patient who presented with worsening cough and dyspnea and he named this case as "Cirrhosis cystica pulmonum" in 1898.

Classification

Idiopathic pulmonary fibrosis is a disease among a large group of the interstitial lung diseases and particularly a subtype of the idiopathic interstitial pneumonia. The major group Acute Interstitial Pneumonia (AIP), Cryptogenic Organizing Pneumonia (COP), Respiratory Bronchiolitis–Interstitial Lung Disease (RB-ILD), and Desquamative Interstitial Pneumonia (DIP)

Pathophysiology

Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma. Although the exact pathogenesis is not fully understood, there are many initiating factors which cause the pulmonary tissue injury. The main features of the lung injury includes inflammation, fibrosis, and granulomas development.

Causes

Common causes of pulmonary fibrosis include autoimmunity, rheumatoid arthritis, scleroderma, tuberculosis, SLE, sarocoidosis, and polymyoisitis. Other causes of pulmonary fibrosis include environmental factors as cigarette smoking and dust exposure.

Differentiating Idiopathic Pulmonary Fibrosis from Other Diseases

Idiopathic pulmonary fibrosis must be differentiated from other causes of interstitial lung diseases which may cause fibrosis as well. Other diseases may include acute interstitial pneumonia, hypersensitivity pneumonia, occupational lung diseases, and pulmonary hemorrhage diseases.

Epidemiology and Demographics

Prevalence of idiopathic pulmonary fibrosis ranges from a low of 0.5 per 100.000 persons to a high of 27.9 per 100.000. Incidence of idiopathic pulmonary fibrosis ranges from a low of 3 per 100,000 persons to a high of 9 per 100,000 persons. The prevalence of idiopathic pulmonary fibrosis increases with age. Idiopathic pulmonary fibrosis is more prevalent in men more than women.

Risk Factors

Common risk factors of pulmonary fibrosis include cigarette smoking and genetic mutations which include hTERT, MUC5B, TERT, and RTEL1. Other risk factors include GERD and wood dust. 

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

There are no diagnostic laboratory findings associated with pulmonary fibrosis. However, useful laboratory findings consistent with the diagnosis of pulmonary fibrosis include abnormal arterial blood gasessputum analysis, and blood picture.

X-ray

X ray imaging does not show specific features for idiopathic pulmonary fibrosis. However, patients with idiopathic pulmonary fibrosis have imaging abnormalities from the beginning of the disease course.

CT scan

MRI

Other Imaging Findings

There are no other imaging findings associated with idiopathic pulmonary fibrosis.

Other Diagnostic Studies

There are no other diagnostic studies associated with idiopathic pulmonary fibrosis.

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References


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