Future research directions in idiopathic pulmonary fibrosis: summary of a National Heart, Lung, and Blood Institute working group.
Idiopathic pulmonary fibrosis (IPF) is an insidious inflammatory fibroproliferative disease whose cause and course before diagnosis are unknown, and for which existing treatments are of limited benefit. The National Heart, Lung, and Blood Institute convened a working group to develop specific recommendations for future IPF research. Inflammatory and immune processes are involved in IPF pathogenesis, and current therapeutic strategies are aimed at suppressing the inflammation. Recent data suggest that the molecular processes underlying the fibrogenesis may provide new opportunities for therapeutic intervention. Specific areas of future research recommended by the working group include studies to elucidate the etiology of IPF, to develop novel diagnostic techniques and molecular diagnostics, to establish a program for identification of molecular targets for IPF treatment and identification and generation of agonists or antagonists that inhibit fibrogenesis, to foster investigations that couple the use of new technologies (e.g., laser capture microdissection, microarrays, and mass spectroscopic analysis of proteins) with data from the human genome project, to establish a national consortium of Clinical Centers of Excellence to conduct coordinated clinical and laboratory studies of well-characterized patients and patient-derived materials, and to stimulate research to develop animal models of persistent and progressive pulmonary fibrosis for evaluation of new intervention approaches.
Division of Pulmonary and Critical Care Medicine, Weill Medical College of Cornell University, New York, New York, USA.
Ronald G
RG
Crystal
Peter B
PB
Bitterman
Brooke
B
Mossman
Marvin I
MI
Schwarz
Dean
D
Sheppard
Laura
L
Almasy
Harold A
HA
Chapman
Scott L
SL
Friedman
Talmadge E
TE
King
Leslie A
LA
Leinwand
Lance
L
Liotta
George R
GR
Martin
David A
DA
Schwartz
Gregory S
GS
Schultz
Carston R
CR
Wagner
Robert A
RA
Musson
D006801:Humans; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D012106:Research