title: [Successful treatment of a severe case of idiopathic pulmonary fibrosis with rapamycin].
abstract: 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.
authors.affiliation: Kliniken der Stadt Köln gGmbH, Krankenhaus Merheim, Köln. BuschhausL2@kliniken-koeln.de
authors.forename: Lutz
authors.initials: L
authors.lastname: Buschhausen
authors.forename: Matthias
authors.initials: M
authors.lastname: Kamm
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Arns
authors.forename: Eckhard
authors.initials: E
authors.lastname: Schulze-Lohoff
authors.forename: Manfred
authors.initials: M
authors.lastname: Weber
mesh_terms: 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
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus