ATS/ERS/JRS/ALAT合同臨床実践ガイドライン：特発性肺線維症 2011年分のアップデートという形で発表

An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline
Ganesh Raghu, et. al.; on behalf of the ATS, ERS, JRS, and ALAT
Read More: http://www.atsjournals.org/doi/abs/10.1164/rccm.201506-1063ST#.VadLZpe2q3M



http://www.thoracic.org/statements/resources/interstitial-lung-disease/IPF-Exec-Sum.pdf
http://www.thoracic.org/statements/resources/interstitial-lung-disease/IPF-Full-length.pdf

Question 1: Should Patients with IPF Be Treated with Anticoagulation?

Question 2: Should Patients with IPF Be Treated with Imatinib, a Tyrosine Kinase Inhibitor?

Question 3: Should Patients with IPF Be Treated with Combination Prednisone, Azathioprine, andN-Acetylcysteine?

Question 4: Should Patients with IPF Be Treated with Ambrisentan, a Selective ER-A Endothelin Receptor Antagonist?

Question 5: Should Patients with IPF Be Treated with Nintedanib, a Tyrosine Kinase Inhibitor?

Question 6: Should Patients with IPF Be Treated with Pirfenidone?

Question 7: Should Patients with IPF Be Treated with Sildenafil,a Phosphodiesterase-5 Inhibitor?

Question #8: Should Patients with IPF Be Treated with Bosentan or Macitentan, Dual Endothelin Receptor Antagonists (ER-A and ER-B)?

Boehringer Ingelheim Welcomes the Inclusion of OFEV® (nintedanib*) in the Updated International Treatment Guidelines for Idiopathic Pulmonary Fibrosis (IPF)
15 July 2015
http://www.boehringer-ingelheim.com/news/news_releases/press_releases/2015/15_july_2015_ipf.html

ミトコンドリアDNAへの多能幹細胞治療

Metabolic rescue in pluripotent cells from patients with mtDNA disease
Hong Ma, et. al.
http://www.nature.com/articles/nature14546.epdf


ミトコンドリア(mt)DNAをの遺伝子変異は治療オプション乏しい。
遺伝子変異と、heteroplasmy (細胞毎の変異/野生種mtDNA) により臨床症状は異なる。
遺伝子修正多能幹細胞（PSC）を作成し、　ミトコンドリア(mt)DNAによりエンコードされたクリティカルな遺伝子に対する治療

MELASやLeigh症候群への遺伝子修正pluripotent stem cell治療