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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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

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

There is insufficient evidence to recommend routine screening for idiopathic pulmonary fibrosis.

Natural History, Complications, and Prognosis

If left untreated, idiopathic pulmonary fibrosis will lead to complete respiratory failure and death. Common complications of idiopathic pulmonary fibrosis include pulmonary hypertension, lung cancer, and cardiovascular comorbidities. The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate from 2 to 5 years.

Diagnosis

Diagnostic Study of Choice

The diagnostic criteria for IPF includes major criteria and minor criteria. The major criteria must include exclusion of other causes of interstitial lung diseaes, pulmonary function tests that is evident of lung fibrosis, bibasilar reticular abnormalities in CT lung, and lung biopsy shows fibrosis. The minor criteria should include 3 of 4 criterion as age > 50, duration of illness more than 3 months, bibasilar inspiratory crackles, and insidious onset of exertional dyspnea.

History and Symptoms

Common symptoms of idiopathic pulmonary fibrosis include DyspneacoughClubbingCrackles, and arhtralgia. Less common symptoms include Hamman-Rich Syndrome.

Physical Examination

Patients with idiopathic pulmonary fibrosis usually appear fatigued and short of breath. Physical examination of patients with occupational lung disease is usually remarkable for bronchial breathing, increased vocal resonance, and fine crepitations.

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

On chest CT scan, Idiopathic pulmonary fibrosis is characterized by honeycombing appearance of the lungsbronchiectasis, ground glass opacities, and distortion of the lung opacities.

MRI

There are no MRI findings associated with idiopathic pulmonary fibrosis.

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

Lung transplantation is essential in treatment of patients with idiopathic pulmonary fibrosis. Pulmonary fibrosis is the most common interstitial lung disease requiring lung transplant and it should be performed early in order to increase the survival rate.

Primary Prevention

The primary prevention of pulmonary fibrosis or usual interstitial pneumonia includes smoking cessation and vaccination against influenza.

Secondary Prevention

The primary and secondary prevention strategies for idiopathic pulmonary fibrosis are the same.

References


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