Idiopathic pulmonary fibrosis: a Clinical Pearl. SCFC 2015

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  1. 1. Montserrat Carreres Prieto. H.U.Bellvitge
  2. 2. Fibrosi pulmonar idioptica: cas clnic - Home de 71 a. Ex-fumador (50 paq/any) - Ant. Laborals: Indstria metallrgica (amiant) durant 34 a. - Tractament: Foster 100mcg/6mcg (2 inh /12h) ; zaldiar c/8h; ranitidina 150mg/12h; simvastatina 20mg/24h - Diagnosticat de FPI: Nintedanib 150mg/12h - D6 Servei de Dispensaci Ambulatria - Basques, sobretot amb la simvastatina. D3: vmit. - D4-5 refereix no haver-se-la pres. - Pregunten si poden deixar lhipolipemiant.
  3. 3. Fisiopatognia de la FPI -Etiologia: desconeguda -Prevalena: 13 dones i 20 homes /100.000 Hab >65 anys. Supervivncia mitja: 3-5 a. -Factors predisposants: Ambientals: Fum tabac, plvores de metalls o fustes... Fisiopatolgics: Refluxe gastroE Gentics: mutacions en els gens que codifiquen per les telomerasses (TERT, TERC)... -Diagnstic: Exclusi: TACAR. Histopatologia -Clnica: Dispnea d'esfor progressiva, tos seca.Raneres crepitants.Acropaquies Comorbiditats: Refluxe GastroE, HiperTA pulmonar Enfisema Pulmonar, Transtorns associats al son, Obesitat Pneumopatia intersticial: Progressiva, Crnica, Irreversible, Localitzada Patr fibrtic vs inflamatori: honeycombing lung
  4. 4. Tractament PIRFENIDONA (EMA 2011, FDA 2014). Esbriet NINTEDANIB (FDA 2014) Ofev MA Desconegut. ImmunosupressorTGF- i PDGF iTK: VEGFR, PDGFR, FGFR DOSI setmanals: 267 mg/8h mx 801mg/8h. ALIMENTS 150mg/12h. ALIMENTS MTB CYP1A2(70-80%) ; CYP2C9, 2C19, 2D6 y 2E1. Esterasses BIBF 1202; UGT1A1, A7, A8 i A10 Glicoprotena P INT Inhibidors (ex): CYP1A2 (ciprofloxacino), CYP2C9 (fluvoxamina) CYP2C19 (cloranfenicol), CYP2D6 (fluoxetina) CYP2E1(disulfiram) Inductors CYP1A2: tabac Inhibidors Glicoprotena P (ex): Amiodarona, diltiazem, simvastatina, claritromicina... Inductors Glicoprotena P (ex): Carbamazepina, Fenitona, Dexametasona, Rifampicina EA Gastrointestinals: diarrees, basques, vmits Dermatolgiques: fotosensibilitat Alteracions en ALT/ASP/bilirubina Gastrointestinals: diarrees, basques, vmits Vasculars: Hemorrgies Dermatolgiques: erupcions cutnies i mucositis Alteracions en ALT/ASP/bilirubina CI Alcohol, fluvoxamina, aranja, suc de aranja Hipersensibilitat a cacahuets/soja; Abans de 2011: glucocorticoids immunomoduladors N-acetilcisteina 2011. Guies ATS/ERS/JRS/ALAT : referncia. Ja parla de Pirfenidona: antifibrtic 2013. Guies NICE: Pirfenidona; SEPAR(2013) i ALAT(2014): Pirfenidona. Nintedanib
  5. 5. Farmacocintica estatines
  6. 6. Missatges claus. Pneumopatia de predomini fibrtic: prdua funci pulmonar. Estndard tractament ha passat dantiinflamatoris i immunomoduladors a ANTIFIBRTICS: Pirfenidona o nintedanib Redueixen la progressi de la malaltia... per no la mortalitat RAMS: Pirfenidona: (GI+dermatolgics); Nintedanib: (GI). Cal que sadministrin AMB ALIMENTS Fan falta ms AC per avaluar la seguretat, un HEAD TO HEAD...
  7. 7. Bibliografia 1. Raghu G, et al.; ATS/ERS/JRS/ALATCommittee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788824. 2. The diagnosis and management of suspected idiopathic pulmonary fibrosis. NICE clinical guideline 163. 2013 3. Pirfenidone for treating idiopathic pulmonary fibrosis. NICE technology appraisal guidance 282. 2013 4. Richeldi et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014 May 29;370(22):2071-82 5. Raghu G, et al. Nintedanib and pirfenidone. New antifibrotic treatments indicated for idiopathic pulmonary fibrosis offer hopes and raises questions. Am J Respir Crit Care Med. 2015 Feb 1;191(3):252-4 6. King et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92. 7. Recomendaciones para el diagnstico y tratamiento de la FPI. ALAT. 2014 8. Nalysnyk et al Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev 2012; 21: 126, 355361 9. Xaubet et al. Normativa sobre el diagnstico y tratamiento de la fibrosis pulmonar idioptica. Arch Bronconeumol. 2013;49(8):343353 10. Garca-Sabina et al. Farm Hosp. Specific considerations on the prescription and therapeutic interchange of statins. 2012 Mar-Apr;36(2):97-108 11. Tzouvelekis et al. Update on therapeutic management of idiopathic pulmonary fibrosis. Therapeutics and Clinical Risk Management 2015:11 359370 Moltes Grcies!