[Pulmonary fibrosis--a therapeutic dilemma?].
The idiopathic interstitial pneumonias, especially the idiopathic pulmonary fibrosis (IPF), are life-threatening lung disorders, for which no effective treatment option exists. In view of IPF, the American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus statement recommends a combined therapy with corticosteroids and azathioprine or cyclophosphamide, although data from conclusive clinical trials are yet missing and the recurrent clinical experience is that these drugs do not really help in IPF. Up to now, lung transplantation represents the last and only therapeutic option for IPF subjects. Based on new pathophysiological concepts of IPF, there are meanwhile a couple of different agents under preclinical and clinical assessment, and the increasing number of clinical trials ongoing in IPF raise the hope that an effective treatment comes into reach. The agents investigated and their targets are: acetylcysteine (reactive oxygen species [ROS] scavenging), interferon-gamma 1b (modulation of Th1/Th2 balance, direct antifibrotic effects), pirfenidone and GC 1008 (blockade of transforming growth factor-beta), FG 3019 (blockade of connective tissue growth factor), imatinib mesylate (blockade of platelet-derived growth factor), bosentan (blockade of endothelin), zileutin (blockade of leukotrienes), etanercept (blockade of tumor necrosis factor-alpha), heparin (alveolar anticoagulation). Hopefully, these new therapeutic strategies may help to improve prognosis of IPF in the future.
Günther
Andreas
A
University of Giessen Lung Center (UGLC), Klinikstrasse 36, 3539, Giessen. andreas.guenther@uglc.de
Markart
Philipp
P
Eickelberg
Oliver
O
Seeger
Werner
W
D000111:Acetylcysteine / Q000008:administration & dosage / Q000627:therapeutic use; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000627:therapeutic use; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D001549:Benzamides; D000077300:Bosentan; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D000068800:Etanercept; D016166:Free Radical Scavengers / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D000068877:Imatinib Mesylate; D007074:Immunoglobulin G / Q000008:administration & dosage / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007371:Interferon-gamma / Q000008:administration & dosage / Q000627:therapeutic use; D016040:Lung Transplantation; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology / Q000628:therapy; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use; D011743:Pyrimidines / Q000008:administration & dosage / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D018124:Receptors, Tumor Necrosis Factor / Q000008:administration & dosage / Q000627:therapeutic use; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D001549:Benzamides; D016166:Free Radical Scavengers; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011728:Pyridones; D011743:Pyrimidines; D018124:Receptors, Tumor Necrosis Factor; D013449:Sulfonamides; D007371:Interferon-gamma; D000068877:Imatinib Mesylate; D003520:Cyclophosphamide; C093844:pirfenidone; D001379:Azathioprine; D000068800:Etanercept; D000077300:Bosentan; D000111:Acetylcysteine