Latest Drug and Treatment Guidelines Discussed at July's ILD Meeting

Piazza in the ancient town of Modena, Italy

I arrived early to the monthly informational and support meeting for people with ILDs at my university hospital. People’s reaction to my weight loss was lovely. Right on time, we met the very famous Italian doctor Dr. Luca Riheldi. Charming. Interesting. Brilliant.

He is from Modena, Italy, which is a small town with great genetics. The brilliant tenor Pavarotti was born and died there and Enzo Ferrari was a native son who helped inspire the racetrack there. Dr. Riheldi was the doctor who held the conferences and headed the writing of the new guidelines for the treatment of Idiopathic Pulmonary Fibrosis (IPF). This disease is by far and away the most populist of all the ILDs.

Dr. Riheldi, using my university hospital’s ILD Clinic as a guide, opened the first clinic for rare lung diseases in Italy and one of just a handful in Europe. They have discovered that 30% of all lung diseases worldwide are Interstitial Lung Diseases. There is just not a proper group of educated people in place to diagnosis and treat the illnesses.

In 2007, his new clinic opened and included a staff of pulmonologist, radiologists and pathologists. He felt that the combine of these three types of specialists are imperative to the proper diagnosis and treatment of ILDs. They meet once a week to discuss every case. These diseases are very difficult to diagnosis properly and, due to the complexity of the diseases, are often misdiagnosed.

To have such a clinic is rare and even in the US, there are only three or four high level Interstitial Lung Disease Clinics, my university hospital being one of those. Thankfully.

Their goal is patient care, education and research. He said all three are needed to maximize knowledge. Publishing in peer review journals is the way research is accepted in the medical world. He has published a lot and told us that my hospital is the #1 publishing hospital in the world for ILDs.

In the EU, he even pushed to include ILDs as part of their yearly acknowledgement of Rare Disease Day. It initially was February 29 (a rare date, indeed!) but they wanted it to be every year, so it is noted on February 28^th each year. It brings the spotlight to ILDs.

Back to the writing of the newly adopted first ever guidelines for treatment of IPF:  He told us that he is currently working with a group of doctors to write a similar evidence based management for all other ILDs including mine, which is Hypersensitivity Pneumonitis. Yahoo!! It should be published; I believe he said, in September of this year.

The other exciting news is that he is heading a Phase 2 study for IPF patients only of oral Triple Kinase Inhibitor BIBF 1120 which has greatly slowed the progression of fibrosis in IPF patients. He told us that these enzymes have also been successful in suppressing ovarian cancer in patients for the past 10 years. He said the genes in cancer are close to IPF.

Anyway, the Phase 2 study included 400 patients in Europe only. They ask the FDA if they wanted to be part of it and they declined. Dumb. Now that it has shown such promise, the FDA has decided to be part of the Phase 3 study. The co-head of my hospital’s ILD Clinic is going to be the head of the study in the US.

If you have IPF, try to get into this study. The charts he showed us were amazing. It nearly stopped the progression of fibrosis. The only serious side effect seemed to be issues with the G.I. Tract.

All of my readers with an ILD, hang in there. A new drug is coming down the pike to slow down the progression of fibrosis, the nemesis of all ILDs. The future looks brighter.