1 / 24

ENFERMEDADES PULMONARES INTERSTICIALES

ENFERMEDADES PULMONARES INTERSTICIALES. DR. ALFREDO DE LA CRUZ MUÑOZ Neumologo Internista Guatemala C.A. INFILTRACION INTERSTICIAL DIFUSA PULMONAR (IIDP). Las enfermedades pulmonares intersticiales ( interstitial lung diseases, ILD)

cisco
Download Presentation

ENFERMEDADES PULMONARES INTERSTICIALES

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. ENFERMEDADES PULMONARES INTERSTICIALES DR. ALFREDO DE LA CRUZ MUÑOZ Neumologo Internista Guatemala C.A.

  2. INFILTRACION INTERSTICIAL DIFUSA PULMONAR (IIDP) • Las enfermedades pulmonares intersticiales (interstitial lung diseases, ILD) • incluyen un gran número de enfermedades que afectan al parénquima del pulmón (los alvéolos, el epitelio alveolar, el endotelio capilar y los espacios entre estas estructuras, así como a los tejidos perivasculares y linfáticos).

  3. Un criterio de utilidad para la clasificación consiste en separar a las ILD en dos grupos con base en la histopatología mayor subyacente: • 1) las que se acompañan de inflamación y fibrosis predominantes, y • 2) las que tienen como aspectos que predominan reacciones granulomatosas en las zonas intersticiales o vasculares

  4. 1. ALVEOLITIS, INFLAMACIÓN INTERSTICIAL Y FIBROSISa. causa conocida • Amianto/Asbesto • Humos, gases • Fármacos (antibióticos, amiodarona, oro) y agentes quimioterápicos • Radiación • Neumonía por aspiración • Secuela del síndrome apneico del adulto

  5. b. CAUSA DESCONOCIDA • Neumonías intersticiales idiopáticas • Fibrosis pulmonar idiopática (neumonía intersticial ordinaria) • Neumonía intersticial descamativa • Enfermedad pulmonar intersticial asociada a bronquiolitis respiratoria • Neumonía intersticial aguda (lesión alveolar difusa) • Neumonía organizativa criptógena (bronquiolitis obliterante con neumonía organizativa) • Neumonía intersticial inespecífica

  6. Enfermedades del tejido conjuntivo • Lupuseritematoso diseminado, artritisreumatoide, espondilitisanquilosante, esclerosissistémica, síndrome de Sjögren, polimiositis-dermatomiositis Síndromes de hemorragia pulmonar • Síndrome de Goodpasture, hemosiderosis pulmonar idiopática, capilaritispulmonar aislada Proteinosis pulmonar alveolar • Trastornos infiltrativos linfocitarios (neumonitis intersticial linfocítica asociada a una enfermedad del tejido conjuntivo) • Neumonías eosinófilas • Linfangioleiomiomatosis • Amiloidosis

  7. Enfermedades heredadas • Esclerosis tuberosa, neurofibromatosis, enfermedad de Niemann-Pick, enfermedad de Gaucher, síndrome de Hermansky-Pudlak • Enfermedades digestivas o hepáticas (enfermedad de Crohn, cirrosis biliar primaria, hepatitis crónica activa, colitis ulcerosa) • Enfermedad del injerto contra hospedador (trasplante de médula ósea; trasplante de órganos sólidos)

  8. 2. RESPUESTA PULMONAR: GRANULOMATOSA • A. CAUSA CONOCIDA • Neumonitis por hipersensibilidad (polvos orgánicos) • Polvos inorgánicos: silicato de berilio

  9. B. CAUSA DESCONOCIDA • Sarcoidosis • Granulomatosis de células de Langerhans (granulomaeosinófilo del pulmón) • Vasculitis granulomatosa • Granulomatosis de Wegener, granulomatosis alérgica de Churg-Strauss • Granulomatosisbroncocéntrica • Granulomatosislinfomatoide

  10. IPF (IDIOPATHIC PULMONARY FIBROSIS) AND CFA (CRYPTOGENIC FIBROSING ALVEOLITIS ) ARE SYNONYMOUS[2] AND ARE ASSOCIATED WITH THE HISTOPATHOLOGICAL PATTERN UIP.[

  11. IPF or CFA classify patients according to histological entities Idiopathic Interstial Pneumonias (IIPS) • Usual interstitial pneumonia (UIP). 47 A 71% • desquamativeinterstitialpneumonia (DIP). • Nonspecific interstitial pneumonia (NSIP). • Respiratory bronchiolitis interstitial lung disease (RBILD) • Acute interstitial pneumonia (AIP) • Lynphoid interstitial pneumonia (LIP).

  12. Cardinal features of IPF/UIP includedrycough, exertionaldyspnea, end-inspiratoryvelcrorales, diffuseparenchymalinfiltratesonchestradiographs, honeycombingon HRCT, a restrictivedefectonpulmonaryfunctiontests (PFTs), and impairedoxygenation.[2,3,42,43]Exertionaldyspneaprogressesinexorablyovermonthstoyears.[

  13. HISTORIA NATURAL • The onset is indolent, but IPF/UIP progresses inexorably over months to years, with progressive fibrosis and destruction of lung parenchyma.[58] Spontaneous remissions do not occur,[5,56,63,64] but some patients stabilize following an initial decline.[53,56,58] Most patients die of respiratory failure within 3 to 8 years of onset of symptoms; mean survival is 2.8 to 3.6 years.[

  14. EPIDEMIOLOGIA • Idiopathic pulmonary fibrosis is rare, but precise data regarding incidence and prevalence are lacking. In 1988, 4,851 deaths in the United States were attributed to pulmonary fibrosis (ICD-9, 515, n = 4,694) and idiopathic pulmonary fibrosis (ICD-9, 516.3, n = 157).[46

  15. FACTORES DE RIEZGO • IPF/UIP is more common in males[43,56,75,78,83] and in current or former smokers.[53,65,75,76,83-85] Other risk factors include exposure to dusts or metals,[75] organic solvents,[86] and residence in agricultural or polluted urban areas

  16. GENETICA • The mode of transmission of familial IPF is not known, but is believed to be autosomal dominant with variable penetrance in approximately 70% of cases; there is no clear mode of transmission in the remaining 30%

  17. FUNCION PULMONAR • characteristically demonstrate reduced lung volumes [e.g., vital capacity (VC), and total lung capacity (TLC)]; normal or increased expiratory flow rates; increased forced expiratory volume in 1 second/forced VC (FEV1/FVC) ratio; reduced diffusing capacity for carbon monoxide (DLCO).[2,27,66,77,103-105] Hypoxemia or increased alveolar-arterial oxygen difference [p(A-a02)], which is accentuated by exercise, is a cardinal feature of IPF

  18. ESPEROMETRÍA

  19. RADIOGRAFIA

  20. HRCT

  21. PANAL DE ABEJA

  22. PATOLOGIA

  23. TRATAMIENTO • In oneclinicalsurvey, 61% of IPF patientsunderage 70 weretreatedwithcorticosteroids, comparedwith 28% of patientsoverage 70.[44]Immunosuppressiveorcytotoxicagents are used in only 2 to 17% of patientswith IPF/CFA.[42,44,47,65]Othertreatmentoptions (albeitunproven) includecolchicine,[47,127,179,245] D-penicillamine,[245]perfenidone,[41] N-acetylcysteine (NAC),[179] and gamma-interferon

  24. Treatment for IPF needs to be individualized • AZA (2-3 mg/kg/day, maximum daily dose 150 mg • oral CP (dose 1-2 mg/kg/day; maximum dose 150 mg/day) plus prednisone • corticosteroids (e.g., 0.5 mg/kg/day) can be tried.

More Related