[Successful treatment of a severe case of idiopathic pulmonary fibrosis with rapamycin].
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a progressive chronic disorder of the lower respiratory tract. The main clinical feature is a progressive shortness of breath, particularly on exercise. An overproduction and deposition of collagen and a proliferation of mesenchymal cells are the histopathologic characteristics. Rapamycin is an immunosuppressive agent with antiproliferative effects on mesenchymal cells including fibroblasts. It was this rationale that prompted the authors to administer rapamycin in a case of rapidly progressive IPF.


METHODS
In a 73-year-old female with a 2-month history of IPF, treatment with steroids and interferon gamma-1b did not improve the detrimental clinical course. Treatment with rapamycin was started; subsequently, clinical condition and objective findings improved markedly within weeks. She is now under treatment for 18 months.


CONCLUSIONS
The authors presume that partial remission is related to rapamycin which may be effective in blocking the progressive fibrosis and increased collagen synthesis thought to be pathophysiologically relevant in this disease. Further studies have to show whether rapamycin may be a treatment option in idiopathic pulmonary fibrosis.
Buschhausen
Lutz
L
Kliniken der Stadt Köln gGmbH, Krankenhaus Merheim, Köln. BuschhausL2@kliniken-koeln.de
Kamm
Matthias
M
Arns
Wolfgang
W
Schulze-Lohoff
Eckhard
E
Weber
Manfred
M
D000368:Aged; D004417:Dyspnea / Q000188:drug therapy / Q000209:etiology; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008134:Long-Term Care; D011658:Pulmonary Fibrosis / Q000188:drug therapy*; D019233:Retreatment; D020123:Sirolimus / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure
D007166:Immunosuppressive Agents; D020123:Sirolimus