title: Effects of nebulised iloprost on pulmonary function and gas exchange in severe pulmonary hypertension.
abstract: Nebulised iloprost is established therapy of severe pulmonary hypertension; however, the effects on the bronchoalveolar compartment have not been investigated so far. We studied the short- and long-term effects of nebulised iloprost on pulmonary function tests and gas exchange in 63 patients with severe pulmonary hypertension (idiopathic n=17, chronic thromboembolism n=15, connective tissue disease n=12, congenital heart disease n=11, respiratory diseases n=8). Patients received iloprost in increasing dose up to 140 micro g iloprost/24h via an ultrasonic nebuliser. Short-term effects were assessed before and after every nebulisation: peak expiration flow decreased in mean by 1.9% (423+/-98 to 415+/-98) and percutaneous oxygen saturation increased in mean by 0.7% (90+/-6 to 91+/-5) post-nebulisation. There were no significant differences concerning underlying diagnosis or dose of nebulised iloprost. Within 3 months, 9 patients stopped treatment due to non-compliance with frequent nebulisations (n=3), or severe side effects (n=4); 2 patients with additional obstructive lung disease developed bronchoconstriction. Long-term effects were assessed by pulmonary function tests and gas exchange parameters at baseline and after 3 months treatment. There were no significant differences after 3 months therapy neither in FEV(1), FVC, TLC, residual volume nor in diffusions capacity, SO(2) at rest and during 6 min walking test, also in respect of the underlying diseases. However, there was a significant increase in 6 min walking distance (6 MWD) after 3 months (246+/-113 to 294+/-115 m, P<0.05). In conclusion, treatment with nebulised iloprost leads to functional improvement in severe pulmonary hypertension without systematic adverse short- and long-term effects on pulmonary function test or gas exchange. Patients with additional obstructive lung disease might develop bronchoconstriction. Severe side effects leading to discontinuation of treatment occurred in 9% of patients.
authors.affiliation: Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, UK. frank.reichenberger@innere.med.uni-giessen.de
authors.forename: F
authors.initials: F
authors.lastname: Reichenberger
authors.forename: A
authors.initials: A
authors.lastname: Mainwood
authors.forename: N
authors.initials: N
authors.lastname: Doughty
authors.forename: A
authors.initials: A
authors.lastname: Fineberg
authors.forename: N W
authors.initials: NW
authors.lastname: Morrell
authors.forename: J
authors.initials: J
authors.lastname: Pepke-Zaba
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003240:Connective Tissue Diseases / Q000150:complications / Q000503:physiopathology; D005260:Female; D005540:Forced Expiratory Flow Rates / Q000502:physiology; D006331:Heart Diseases / Q000151:congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage* / Q000009:adverse effects; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009330:Nebulizers and Vaporizers; D010366:Peak Expiratory Flow Rate / Q000502:physiology; D011655:Pulmonary Embolism / Q000150:complications / Q000503:physiopathology; D012129:Respiratory Function Tests / Q000379:methods; D013458:Sulfur Dioxide / Q000378:metabolism; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects; D014797:Vital Capacity / Q000502:physiology
chemical_list: D014665:Vasodilator Agents; D013458:Sulfur Dioxide; D016285:Iloprost