Respiratory/Mucolytics, CF & fibrosis drugslow yield

Mucolytics, CF & pulmonary fibrosis drugs

also: carbocisteine · dornase alfa · CFTR modulator · pirfenidone · nintedanib

Overview

Drugs for chronic sputum/mucus problems — COPD/bronchiectasis mucolytics, cystic fibrosis therapies, and the antifibrotics for idiopathic pulmonary fibrosis.

Mechanism

Mucolytics (carbocisteine) reduce sputum viscosity. In CF, dornase alfa cleaves DNA in purulent sputum, nebulised hypertonic saline hydrates it, and CFTR modulators (ivacaftor/lumacaftor) correct the defective chloride channel. Antifibrotics (pirfenidone, nintedanib) slow the decline in IPF.

Indications

COPD/bronchiectasis with chronic productive cough (mucolytics)
Cystic fibrosis
Idiopathic pulmonary fibrosis

The agents

Carbocisteinemucolytic

oral

COPD/bronchiectasis chronic productive cough.

Dornase alfa / nebulised hypertonic salineCF airway clearance

nebulised

CF.

CFTR modulators (ivacaftor/lumacaftor)CF disease-modifying

oral

Mutation-specific.

Pirfenidone / nintedanibantifibrotic

oral

Idiopathic pulmonary fibrosis — slow FVC decline; LFT monitoring.

Adverse effects

Antifibrotics: hepatotoxicity, GI upset, photosensitivity (pirfenidone)serious

Mucolytics: GI upsetcommon

Cautions & contraindications

Antifibrotics in significant hepatic impairmentall

Interactions

Nintedanib + anticoagulants (bleeding); CFTR modulators CYP3A4

Monitoring & kinetics

LFTs (antifibrotics); lung function

Oral / nebulised. Specialist-initiated for CF/IPF.

Source: NICE — IPF / CF · BNF — Mucolytics / respiratory