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2 out of 7

Which of the following is a feature of idiopathic pulmonary fibrosis? (AIIMS May 2009)

A Dry cough
B Clubbing

C Fine end-inspiratory crepitations
D All of the above

Ans. D

All of the above

Idiopathic pulmonary fibrosis

1). The commonest cause of interstitial lung disease,

2). Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause (also known as cryptogenic fibrosing alveolitis).

3). Symptoms Dry cough Q; exertional dyspnoea; malaise; weight; arthralgia.

4). Signs Cyanosis; finger clubbing Q; fine end-inspiratory crepitationsQ.

5). Complications Type 1 respiratory failure; increased risk of lung cancer Q.

6). Tests Blood:

a. ABG (decrease PaO2, Decrease PaCO2) b. CRP; c. immunoglobulins; d. ANA (30% +ve,

b. Rheumatoid factor (10% +ve).

c. CXR: Lung volume , bilateral lower zone reticulo-nodular shadows; honeycomb lungQ (advanced disease).

d. CT scan show similar changes to the CXR but is more sensitive and is an essential tool for diagnosis.

7). Spirometry: RestrictiveQ pattern is seen.

8). BAL (broncho alveolar lavage) may indicate activity of alveolitis:

9). 99TCm-DTPA scan: may reflect disease activity.

10). Rarely Lung biopsy may be needed for diagnosis.

11). Management A large proportion of patients have chronic irreversible disease cell unresponsive to treatment

12). Drugs used are :

a. Prednisolone

b. Cyclophosphamide

c. Lung transplantation

Guidelines for Referral and Transplantation Idiopathic Pulmonary Fibrosis

(Ref. Hari-18th Pg- 2190, table 266.1)

1). Referral

Pathologic or radiographic evidence of UIP regardless of vital capacity

2). Transplantation

a. Pathologic or radiographic evidence of UIP and any of the following criteria

b. DLCO <39%

c. Decrement in FVC 10% during 6 months of follow-up

d. Decrease in SpO2 below 88% during a 6-min walk test

e. Honeycombing on HRCT (fibrosis score >2)