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Usual Interstitial Pneumonia

Title

Ñ    Usual Interstitial Pneumonia (Cryptogenic Fibrosing Alveolitis, Idiopathic Pulmonary Fibrosis)

Definition

Ñ    Type of interstitial lung disease

Ñ    Widespread diffuse inflammatory reaction involving:

·          Small airways

·          Alveoli

Ñ    Unknown aetiology

Pathogenesis

Cause

Ñ    Unknown

A similar condition occurs in the following:

LMS

Ñ    Rheumatoid arthritis

Ñ    Systemic lupus erythematosus

Ñ    Dermatomyositis

Ñ    Systemic sclerosis

Ñ    Sjögren’s syndrome

GIT

Ñ    Coeliac disease

Ñ    Ulcerative colitis

KUS

Ñ    Renal tubular acidosis

Pathological Process

Ñ    Alveolar space:

Increased cells, mainly type II pneumocytes and macrophages

Ñ    Alveolar walls:

Infiltration with T lymphocytes, plasma cells, thickening of alveolar walls and fibrosis

Ñ    Activation of macrophages and alveolar epithelial cells results in production of growth factors that stimulate deposition of type I and type III collagen

Clinical Features

History

RS

Ñ    Progressive breathlessness

Ñ    Dry cough

Examination

IS

Ñ    Cyanosis

Ñ    Finger clubbing:

60% of patients

RS

Ñ    Tachypnoea

Ñ    Reduced chest expansion

Ñ    Fine late inspiratory crepitations bilaterally

Acute form of illness

Ñ    Hamman-Rich syndrome

Complications

CVS

Ñ    Pulmonary hypertension

Ñ    Cor pulmonale

Investigations

Fluids

Blood

Biochemistry

Blood gases

Ñ    Arterial hypoxaemia

Ñ    Low P_aCO₂

Immunology

Ñ    ANA and Rheumatoid factor:

Positive in 30%

Imaging                

Chest X-ray

Ñ    Reticulonodular shadowing

High Resolution CT scan (HRCT)

Ñ    Peripheral reticular and ground glass opacification

Physiological assessment

Respiratory Function Tests

Ñ    Restrictive ventilatory defect

Ñ    Carbon monoxide gas transfer:

Reduced

Endoscopy

Bronchoalveolar lavage

Ñ    Increased number of cells:

Neutrophils and macrophages

Tissue Diagnosis

Transbronchial, open or thoracoscopic lung biopsy

Ñ    Histological confirmation

Ñ    Exclude sarcoidosis and lymphangitis carcinomatosis

Management

Control

Drugs

Ñ    Steroids

Ñ    Immunosuppression:                 

Azathioprine

Cyclophosphamide

Support

Drugs

Ñ    Domiciliary oxygen:

Cor pulmonale

Prognosis

Ñ    3.5 year median survival

Highest mortality:

·          Males

·          Patients > 55 years of age

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