DR. ALFREDO DE LA CRUZ
MUÑOZ
Neumologo Internista
Guatemala C.A.
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).

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

Amianto/Asbesto
 Humos, gases
 Fármacos (antibióticos, amiodarona,
oro) y agentes quimioterápicos
 Radiación
 Neumonía por aspiración
 Secuela del síndrome apneico del
adulto
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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
Lupus eritematoso diseminado, artritis reumatoide,
espondilitis anquilosante, esclerosis sistémica,
síndrome de Sjögren, polimiositis-dermatomiositis
Síndromes de hemorragia pulmonar
 Síndrome de Goodpasture, hemosiderosis pulmonar
idiopática, capilaritis pulmonar aislada
Proteinosis pulmonar alveolar
 Trastornos infiltrativos linfocitarios (neumonitis
intersticial linfocítica asociada a una enfermedad
del tejido conjuntivo)
 Neumonías eosinófilas
 Linfangioleiomiomatosis
 Amiloidosis

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)

A. CAUSA CONOCIDA
 Neumonitis por hipersensibilidad (polvos
orgánicos)
 Polvos inorgánicos: silicato de berilio
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Sarcoidosis
Granulomatosis de células de Langerhans
(granuloma eosinófilo del pulmón)
Vasculitis granulomatosa
Granulomatosis de Wegener,
granulomatosis alérgica de Churg-Strauss
Granulomatosis broncocéntrica
Granulomatosis linfomatoide

IPF (IDIOPATHIC PULMONARY FIBROSIS)
AND CFA (CRYPTOGENIC FIBROSING
ALVEOLITIS ) ARE SYNONYMOUS[2] AND
ARE ASSOCIATED WITH THE
HISTOPATHOLOGICAL PATTERN UIP.[
Idiopathic Interstial Pneumonias (IIPS)
 Usual interstitial pneumonia (UIP). 47 A 71%
 desquamative interstitial pneumonia (DIP).
 Nonspecific interstitial pneumonia (NSIP).
 Respiratory bronchiolitis interstitial lung
disease (RBILD)
 Acute interstitial pneumonia (AIP)
 Lynphoid interstitial pneumonia (LIP).

Cardinal features of IPF/UIP include dry
cough, exertional dyspnea, endinspiratory velcro rales, diffuse
parenchymal infiltrates on chest
radiographs, honeycombing on HRCT, a
restrictive defect on pulmonary function
tests (PFTs), and impaired
oxygenation.[2,3,42,43] Exertional dyspnea
progresses inexorably over months to
years.[

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.[

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

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

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%

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

In one clinical survey, 61% of IPF patients
under age 70 were treated with
corticosteroids, compared with 28% of
patients over age 70.[44] Immunosuppressive
or cytotoxic agents are used in only 2 to
17% of patients with IPF/CFA.[42,44,47,65] Other
treatment options (albeit unproven) include
colchicine,[47,127,179,245] D-penicillamine,[245]
perfenidone,[41] N-acetylcysteine (NAC),[179]
and gamma-interferon
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.
