Drugs are avail­able for the treat­ment of IPF

BioSpectrum (Asia) - - Content - Prof Philip Eng Se­nior Con­sul­tant, Re­s­pi­ra­tory & ICU Medicine at Mount Elizabeth Med­i­cal Cen­tre, Sin­ga­pore

Idio­pathic pul­monary fi­bro­sis (IPF) is a se­ri­ous lung dis­ease that pri­mar­ily af­fects pa­tients over the age of 50 and af­fects more men than women. While IPF is con­sid­ered a rare dis­ease – it af­fects ap­prox­i­mately three mil­lion peo­ple world­wide – an age­ing Asian pop­u­la­tion means that more and more men in Asia will be at risk of hav­ing IPF. Fur­ther­more, with IPF be­ing dif­fi­cult to di­ag­nose, those who have the con­di­tion could sim­ply at­tribute it to signs of ‘grow­ing old’. In con­junc­tion with IPF World Week, Prof Philip Eng, Se­nior Con­sul­tant, Re­s­pi­ra­tory & ICU Medicine at Mount Elizabeth Med­i­cal Cen­tre, Sin­ga­pore re­cently spoke to Priyanka Ba­j­pai of BioSpec­trum Asia about the deadly dis­ease and lat­est ad­vance­ments in its treat­ments. Prof Eng has pub­lished more than 120 peer-re­viewed ar­ti­cles and books. He also sits on sev­eral ed­i­to­rial boards such as the Sin­ga­pore Al­lergy & Asthma News­let­ter, MIMS Re­s­pi­ra­tory Guide and Jour­nal of Bron­chol­ogy.

Can you briefly ex­plain what IPF is, and the symp­toms and signs to look out for? How com­mon is this con­di­tion?

Idio­pathic pul­monary fi­bro­sis (IPF) is a se­ri­ous and life-threat­en­ing con­di­tion in which the lungs be­come thick­ened, stiff, and scarred over a pe­riod of time. The lungs then pro­gres­sively lose their abil­ity to take in and trans­fer oxy­gen into the blood­stream, de­creas­ing the amount of oxy­gen, the lungs can sup­ply to ma­jor or­gans of the body.

IPF af­fects ap­prox­i­mately three mil­lion pa­tients world­wide. There are no ac­cu­rate statis­tics of preva­lence of this dis­ease in Sin­ga­pore or most of Asia. Based on the es­ti­mated preva­lence of 20 pa­tients per 100,000 pop­u­la­tion from data from US and Europe, I would es­ti­mate there would be about 1,000 pa­tients in Sin­ga­pore. Symp­toms of IPF in­clude breath­less­ness, dry and per­sis­tent cough­ing which goes on for months. Clin­i­cal ex­am­i­na­tion shows club­bing (widen­ing and round­ing) of the tips of fin­gers and toes. Some­times weight loss oc­curs if the dis­ease is se­vere. More than 80 per cent of IPF pa­tients have a dis­tinct crack­ling sound that a HCP may be able to hear by ex­am­i­na­tion with a stetho­scope.

What causes IPF?

IPF is de­fined as an idio­pathic dis­ease (i.e. of un­known cause). Be­fore one makes the di­ag­no­sis of IPF, it is im­por­tant to ex­clude cer­tain other causes, for ex­am­ple en­vi­ron­men­tal tox­ins, cer­tain drugs and other con­nec­tive tis­sue dis­eases like SLE.

Who is more likely to be af­fected by IPF, and are there pre­ven­tive mea­sures that one can take?

IPF gen­er­ally af­fects males over the age of 65. More than 70 per cent of pa­tients with IPF are males and smok­ers or ex-smok­ers. A per­son suf­fer­ing from gas­troe­sophageal re­flux dis­ease (GERD) is at a higher risk of hav­ing IPF. Un­for­tu­nately, there is noth­ing one can do to pre­vent the dis­ease. The im­por­tant mes­sage is for one who has per­sis­tent symp­toms like short­ness of breath or cough to go for med­i­cal eval­u­a­tion, rather than at­tribute it to the nor­mal age­ing process.

What is the treat­ment for IPF? Are there any re­cent new de­vel­op­ments?

Gen­er­ally, pa­tients get pro­gres­sively more short of breath with time and will need long-term oxy­gen in the long run. Once they are end stage, lung trans­plan­ta­tion should be con­sid­ered if the pa­tient is fit. Two new drugs have been re­cently ap­proved for the treat­ment of IPF – Pir­fenidone and Nin­te­nanib. Stud­ies have shown them to be ef­fec­tive in slow­ing down the de­cline of lung func­tion.

What is the gen­eral prog­no­sis for IPF? Has it im­proved be­cause of the ad­vance­ments above?

Although sur­vival rates vary by pa­tient, me­dian sur­vival af­ter IPF di­ag­no­sis is two to three years in many stud­ies all over the world. It is too early to say if the new drugs will make a ma­jor im­pact in over­all sur­vival of these pa­tients, although it is very promis­ing.

Prof Philip Eng Se­nior Con­sul­tant, Re­s­pi­ra­tory & ICU Medicine at Mount Elizabeth Med­i­cal Cen­tre, Sin­ga­pore

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