Interstitial Lung Diseases (ILD)
Diffuse Parenchymal Lung Diseases (DPLD)
Edit Csada, MD
29.10.2014.
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Interstitial lung disorders (ILD)
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The interstitial lung disorders are chronic,
nonmalignant, noninfectious diseases of the
lower respiratory tract characterized by
inflammation and derangement of the alveolar
walls.
Secondary fibrosis and pulmonal hypertension
may develop
Prevalence: 7-20/100 000
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Etiology
(ATS/ERS 2002)
1. Known origin
 Drugs (busulfan,
nitrofurantoin, bleom.,
amiodaron)
 Pneumoconiosis
 Connective tissue diseases
 Irradiation
 Malignant diffuse infiltr.
lung diseases
2. Granulomatous
origin
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Sarcoidosis
Hypersensitive
pneumonitis
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Etiology
3. Other DPLD
 Langerhans-cell histiocytosis
 Lymphangioleiomyomatosis
 Alveolar proteinosis
 Wegener granulomatosis
4. IIP
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UIP (IPF)
DIP/RBILD
NSIP
AIP
COP
LIP
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According to the pathogenesis I.

Acute DPLD
allergy (drugs)
 toxin (gas)
 vasculitis/hemorrhage (Goodpasture, idiopathic
hemosiderosis)
 ARDS (trauma, septicaemia)
 unknown (COP, BOOP)

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According to the pathogenesis II.
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Episodic DPLD
eo pneumonia
 vasculitis, hemorrhage
 Churg-Strauss sy
 Hypersensitive pneumonitis
 COP

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According to the pathogenesis III.
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Chronic DPLD (exposition)
inorganic dusts (silicosis, asbestosis)
 organic dusts (bacteria, fungi, animal proteins)
 drugs

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According to the pathogenesis IV.
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Chronic DPLD (systemic diseases)
 sarcoidosis
connective tissue diseases
 malignant diseases (lymphoma, lymphangitis cc)
 vasculitis (Wegener)
 hereditary diseases
 others ( bone marrow transplantation,
inflammatory
intestine diseases, amyloidosis)

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According to the pathogenesis V.
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Chronic DPLD (without systemic disease and
without exposition)
Idiopathic interstitial pneumonitis (IIP)
 alveolar proteinosis
 chronic aspiration
 LAM
 Langerhans-cell histiocytosis
 venoocclusive disease
 idiopathic pulmonal haemosiderosis
 bronchioloalveolar cc.

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Morphologic changes
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Loss of pulmonary capillaries
Alterations of alveolar epithelial cells
Fibrosis of alveolar walls
Pathogenesis
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Total number of inflammatory cells increases
The proportions of inflammatory cells change
The inflammatory cells are activated
 Mediators release – toxic oxygen radical, proteases
 Fibrosis
 Impairment of O2 transport
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Normal cell content of BAL
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90%
7%
1%
alveolar macrophages
lymphocytes
polymorph leucocytes
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•Neutrophyl alveolitis :
cryptogenic fibrotic alveolitis
•Lymfocytic alveolitis :
sarcoidosis, hypersensitive
pneumonitis, beryllosis
•Eosinophil alveolitis
•Mixed-cell alveolitis:
amiodaron fibrosis
(eosinophil+lymphocyte)
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Clinical features in ILD I.
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Symptoms
Dyspnea during exercise
 Fatigue
 Nonproductive cough
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Physical finding
Dry, crackling rales
 Wheezing
 Tubular breath sounds
 Digital clubbing
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Clinical features in ILD II.
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Laboratory changes
ERS can be elevated
 Hypoxaemia
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Chest X-ray, HRCT scan
Reticular
 Nodular
 Reticulonodular
 Ground glass haziness
 Honeycombing
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Clinical features in ILD III.
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Lung function tests
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Scintigraphic findings Tc99, Xe133
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Reduction in VC, TLC
FEV1/FVC is normal, or supranormal
Decrease in diffusing capacity, transfer factor (DLCO)
Oxyergospirometry (exercise test)
Patchy abnormalities
BAL
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Various mixtures of inflammatory cells
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Diagnosis of ILD
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Clinical features
Transbronchial biopsy
VATS
Open lung biopsy
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Therapy of ILD
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Known etiology
 Remove the individual from exposure to the
causative agents
Known and unknown etiology
 Suppress inflammatory process
 Oral corticosteroids (1mg/kg0,25 mg/kg)
 CPA (cyclophosphamide)
 Imuran (azathioprine)
Late stage
 O2 therapy
 Supportive treatment
 Transplantation
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Idioptahic pulmonary fibrosis (IPF)
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Hamman-Rich sy (acute IP)
Cryptogenic fibrosing alveolitis (COP)
Desquamative intersitial pneumonitis (DIP)
Usual interstitial pneumonitis (UIP)
Respiratory bronchiolytis-associted IP (RBILD)
Nonspecific interstitial pneumonitis (NSIP)
Lymphoid interstitial pneumonitis (LIP)
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Idioptahic pulmonary fibrosis (IPF)
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The cause of condition is not clear.
Chr. inflammatory process initiated by immune
complexes
derangement of the lung parenchyma
The clinical features are similar to other ILD
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Radiologic changes of IPF
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Ground glass haziness
Streaky wisps of shadow
Generalized micronodular mottling
Honeycomb lung
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Diagnosis of IPF
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Clinical features
X-ray
CT scan
Diffusing capacity, transfer factor decreased (DLCO)
BAL (neutr.)
Transbronchial biopsy
VATS
Open lung biopsy
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ATS/ERS diagnostic criteria of IPF (biopsy)
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Major criteria
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There is no known cause of ILD (drug, environmental
exposition)
Impaired lung function, restriction, decreased diffusion
capacity
Bilateral basal reticular, streaky shadow, minimal granulomas
There is no other diseases with similar symptoms
Minor criteria
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Age > 50
There is no other cause of dyspnoe
Duration of disease > 3 month
Bilateral basal subcrepitatio
AJRCCM 2002;165:277-304
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Therapy of IPF (ATS)
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No treatment
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Early and combined treatment
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CS+ azathioprine
CS+ cyclophosphamide
CS+ azathioprine+ N-acetylcysteine
Restaging after 3-6-9-12-18 month
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Age > 70 years
Extreme obesity
DM, hearth disease, osteoporosis
Severe impaired lung function
Honeycomb lung
Symptoms, radiology, lung function, side effects
PR, transplantation
Best supportive care, O2 treatment
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Therapy of IPF (ATS)
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New drugs
Interferon
 Bosentan
 Etanercept
 Pirfenidon
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Sarcoidosis (Morbus Boeck)
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It is clinically well defined, systemic,
granulomatous disease of unknown origin.
Histology
 Altered immune
response
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Clinical features of sarcoidosis
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Radiological appearances of sarcoidosis
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Stage/form I
Stage/form II
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Stage/form III
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BHL sy
pulmonary dissemination
with or without BHL
pulmonary fibrosis
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Diagnosis of sarcoidosis
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BHL accompanied by erythema nodosum (Löfgren-sy)
Tuberculin test: negative
Lung function tests
Se ACE (angiotensin converting enzyme) increases
Transbronchial biopsy, perbronchial biopsy (TBNA)
BAL (ly)
Gallium scan
Mediastinoscopy
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Treatment of sarcoidosis
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Stage I: no treatment is necessary
Indication of steroid treatment:
Progressive pulmonary disease
 Severe uveitis
 Hypercalcaemia
 Neurological involvement
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Histiocytosis X (Langerhans)
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This is a disorder of the mononuclear phagocyte
system characterized by the accumulation of
mononuclear phagocytes is various organs.
In pediatric patients
 Letterer-Siwe disease
 Hand-Schüller-Christian disease
In adults
 Histiocytosis X
 Eosinophilic granuloma
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Clinical features of histiocytosisX
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20-40 years of age (smoking)
Nonproductive cough
Dyspnea
Chest pain
Ptx – 10% of all cases
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Characteristics of histiocytosisX
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X-ray changes
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Lung function tests
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Large number of mononuclear phagocytes
Histology
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Mixed restrictive-obstructive pattern
Decreased diffusing capacity
BAL
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Reticulonodular shadow
Small cystic spaces
X bodies (Birbeck) in the cytoplasm
There is no known treatment. Corticosteroids may be
given.
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Langerhans-cell histiocytosis
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Rheumatoid arthritis
Treatment
If the disease is mild, no specific therapy is used.
If the ILD is progressive, corticosteroids are
administered.
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Other immunological diseses
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SLE
Sjögren sy
Scleroderma
Bechterew
Dematomyositis, polymiositis
Periarteritis nodosa
Neurofibromatosis
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Alveolar proteinosis
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Primary-secondary (myeloid leukaemy, dust)
PAS positive lipoprotein
X-ray: butterfly shape infiltration
Therapy: BAL
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Eosinophylic pneumonias
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The eosinophylic pneumonias are characterized
by eosinophilic pulmonary infiltrates and
commonly peripherial blood eosinophylia.
Known and unknown etiology
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Eosinophylic pneumonias
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Known etiology
 Allergic bronchopulmonary aspergillosis (ABPA)
 Parasitic infestations (ascaris, toxocara,etc)
 Drug reactions (nitrofurantoin, sulphonamides)
Idiopathic (unknown etiology)
 Löffler’s sy
 Benign, acute eosinophilic pneumonia (AEP) with
migrating pulmonary infiltrates and minimal clinical
manifestation.
 Chr. eosinophilic pneumonia (CEP)
 Symptoms: cough, sweats, fever, anorexia, weight loss,
chills
 X-ray: Peripherial infiltrates
 Allergic granulomatosis of Churg and Strauss
 Hypereosinophilic sy (HES)
Therapy: corticosteroids
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PNEUMOCONIOSIS
Etiologic agents: inhalation of inorganic dusts
metal dusts
free silica
coal dusts
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HYPERSENSITIVE PNEUMONITIS
(Extrinic allergic alveolitis)
It is an immunologically induced inflammation of lung
parenchyma involving alveolar walls and terminal airways
secondary to repeated inhalation of a variety of organic
dusts and other agents by susceptible host.
Manifestations:
Farmer’s lung (1932) – thermophylic actinomycetes
Bird fancier’s breeder’s or handler’s lung
Miller’s lung
Bagassosis
Byssinosis
Air conditioner’s lung
Coffee worker’s lung
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Thank you for your attention!
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