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Antifibrotic drug newest addition to IPF management regime

18 Jan 2017
 A rare yet fatal disease, idiopathic pulmonary fibrosis (IPF) often takes patients by surprise, says a pulmonologist.

It is an incurable and progressive disease which significantly shortens the lifespan of the afflicted. Still, the disease progression can be delayed by treatment with various medications such as an antifibrotic drug. “Only about 20 to 30% of patients are still alive 5 years after diagnosis. The definitive treatment is lung transplant but this treatment option is still limited to a few patients in Malaysia at this stage due to shortage of donors. Nintedanib—an antifibrotic drug—appears effective as a bridge therapy to slow the rate of disease progression while waiting for a suitable donor,” said consultant pulmonologist Dr. Jamalul Azizi Abdul Rahaman, who is also the National Head of Pulmonology Services MOH.

Besides delaying disease progression by reducing the annual decline in lung function, the oral antifibrotic drug has also demonstrated reduction in risk of acute exacerbations. [Respir Med 2016;113:74–79]

Agreeing with Jamalul Azizi, Dr. Mogana Sivalingam, Head of Medical in Boehringer Ingelheim Malaysia, said the launch of nintedanib in the country fills in the gap in patient care and hails a new standard of therapy for IPF locally. Research has shown the antifibrotic drug can reduce the yearly decline of lung function by around 50%; this is good news for IPF patients and their caregivers.

Among the medications that can be used to manage IPF are corticosteroid monotherapy, colchicines, cyclosporine A, combined corticosteroid and immune-modulator therapy, interferon γ 1b, bosentan, etanercept, acetylcysteine, anticoagulant therapy and pirfenidone. [Am J Respir Crit Care Med 2011;183(6):788–824] Long-term oxygen therapy is also recommended, especially in patients with severe hypoxemia.

Idiopathic pulmonary fibrosis is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. The disease occurs mainly in older adults and is limited to the lungs. It is associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia.

Excessive deposition of extracellular matrix and recurrent injury to the alveolar epithelium are the two known characteristics of IPF. Nevertheless, the pathophysiology at the molecular level is still unclear. More studies are required to better understand the underlying cause and formulate a definitive therapy for the disease.

Jamalul Azizi and Mogana were speaking at the launch of nintedanib, held in Kuala Lumpur recently. 

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Most Read Articles
Pearl Toh, 3 days ago
First-line therapy with the BTK* inhibitor ibrutinib plus the anti-CD20 immunotherapy rituximab confers significant survival advantage over the current gold-standard regimen of fludarabine, cyclophosphamide, and rituximab (FCR) for young, fit patients with treatment-naïve chronic lymphocytic leukaemia (CLL), according to the E1912 trial, a large cooperative group study supported by the US National Cancer Institute.
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