1. Interstitial lung disease
Approach to ILD
In collagen vascular disease
Division of pulmonology
Jeung Eun Park

2. Contents Definition of ILD Classification of ILD
Approach of patients with ILD
ILD in collagen vascular disease

3. Interstitial lung disease (ILD)
Diffuse Interstitial Lung Disease (DILD, ILD)
Diffuse Parenchymal Lung Disease (DPLD)
A heterogeneous group of lung parenchymal disease by varying patterns of inflammation and fibrosis
Aloveoli, alveolar epithelium, capillary endothelium
The spaces between these structures
Perivascular and lymphatic tissues

4. Clinical features Exertional dyspnea
CXR : Bilateral diffuse interstitial infiltrates
PFT : Restrictive pattern
Increased alveolar-arterial oxygen difference
Absence of pulmonary infection or neoplasm
Pathology : inflammation and/or fibrosis or granuloma

5. 2002 ATS/ERS Classification
Interstitial lung disease
ILD
of known causes
(drug, environ, CVD)
Idiopathic
Interstitial
Pneumonia (IIP)
Granulomatous
ILD
(sarcoidosis)
Other forms of
ILD
(LAM, HX)
Idiopathic Pulmonary
Fibrosis (IPF)
= UIP
IIP other than IPF
Respiratory bronchiolitis–
associated interstitial lung disease
Desquamative
interstitial pneumonia
Acute interstitial pneumonia
Cryptogenic organizing
pneumonia
Nonspecific interstitial
pneumonia
Lymphocytic interstitial
pneumonia
2002 ATS/ERS Classification

6. 2002 ATS/ERS

7. IPF vs non-IPF Idiopathic pulmonary fibrosis (IPF) UIP
Most Common type
Most Poor prognosis

8. 2002 ATS/ERS

9. Radiographic features of the idiopathic interstitial pneumonias
Clinical Diagnosis
Usual Radiographic Features
Typical Findings on HRCT
IPF
Basal-predominant reticulation abnormality with volume loss
Peripheral, basal, subpleural reticulation with honeycombing, traction bronchiectasis, and focal ground-glass attenuation
NSIP
Ground-glass and reticular opacification
Peripheral, basal, subpleural, symmetrical ground-glass attenuation with irregular lines and consolidation
COP
Patchy bilateral consolidation
Subpleural or peribronchial patchy consolidation and/or nodules
AIP
Diffuse ground-glass density/consolidation
Diffuse consolidation and ground-glass opacification, often with lobular sparing and late traction bronchiectasis
DIP
Ground-glass opacity
Peripheral, lower lung zone ground-glass attenuation with reticulation and/or small cysts
RB-ILD
Bronchial wall thickening, ground-glass opacification
Diffuse bronchial wall thickening with poorly defined centrilobular nodules and patchy ground-glass opacification
LIP
Reticular opacities and nodules
Diffuse centrilobular nodules, ground-glass attenuation, septal and bronchovascular wall thickening, and thin-walled cysts

10. UIP NSIP NSIP BOOP DAD DAD
UIP in a 63-year-old man with pSS. This image demonstrates coarse reticular opacities in both lungs with
associated traction bronchiectasis and subpleural honeycombing.
NSIP shows extensive ground glass abnormality, with associated reticular
abnormality and traction bronchiectasis.
BOOP: obtained at the level of the inferior pulmonary vein shows patchy areas of ground-glass attenuation and consolidation
with a subpleural or peribronchovascular distribution in both lungs.
DAD :show patchy and geographic distribution of ground-glass attenuation with consolidation bilaterally in the upper and
middle lung zones
DAD

11. UIP
NSIP
UIP: mixed pattern (정상조직과 병변 조직이 혼재) irregular interstitial fibrosis (solid arrow)
with mononuclear cell infiltration and foci of fibroblastic proliferation (arrowheads). Intervening normal lung tissue is also seen (open arrows
NSIP :shows uniform interstitial fibrous thickening with infiltration of a few mononuclear cells
DAD shows diffuse interstitial thickening with fibroblastic proliferation and mononuclear cell infiltration.
BOOP : shows intraalveolar fibroblastic plugging (arrows) in alveolar spaces and alveolar duct
AIP
DAD
BOOP

12. Histologic Patterns of NSIP
Cellular NSIP
Predominantly chronic
interstitial inflammation
with lymphocytes & plasma cells
Fibrotic NSIP
Predominantly variable extent of
uniform interstitial fibrosis
± cellular component

13. Clinico–radiologic–pathologic diagnosis (CRP diagnosis)
병리학적 소견 또는 방사선학적 소견이 진단명이 아니다 !!
형태학적 소견(병리학적 소견, 방사선학적 소견)과 임상진단명을 혼돈해서는 안 된다.
Clinician must R/O
Connective tissue disease
Drug
Occupational exposure
Environmental exposure
Toxic fume
Infection

14. Clinical Dx, if idiopathic
Histologic and clinical classification of idiotathic interstitial pneumonias
Pathologic Dx
Clinical Dx, if idiopathic
UIP (pattern)
IPF
NSIP (pattern)
NSIP
BOOP (pattern)
COP
DAD (pattern)
AIP
LIP (pattern)
LIP
DIP (pattern)
DIP
RB-ILD (pattern)
RB-ILD

15. UIP pattern IPF (idiopathic) Drugs Infections Chronic HP
Collagen vascular disease
Asbestosis

16. NSIP pattern Idiopathic NSIP Collagen vascular disease HP
Drug (methotrexate, amiodarone, etc.)
Infection
Immunodeficiency including HIV infection

17. BOOP Pattern Idiopathic process (COP)
Organizing diffuse alveolar damage
Organizing infections
Organization distal to obstruction
Organizing aspiration pneumonia
Organizing drug reactions, fume, and toxic exposures
Collagen vascular disease
Extrinsic allergic alveolitis/hypersensitivity pneumonitis
Eosinophilic lung disease
Inflammatory bowel disease
As a secondary reaction in chronic bronchiolitis
As a reparative reaction around other processes (including abscesses, Wegener’s granulomatosis, neoplasms, and others)

18. Common drug-induced ILD
Antimicrobial agents
Cephalosporins
Isoniazid
Nitrofurantoin
Penicillins
Sulfonamides
Anti-inflammatory agents
Aspirin
Gold
Methotrexate
NSIDs
Penicillamine
Cardiovascular drugs
Amiodarone
ACEi
β-Blockers
Hydralazine
Hydrochlorothiazide

19. Antineoplastic agents
CNS drugs
Carbamazepine
Chlorpromazine
Imipramine
Phenytoin
OHA
Chlorpropamide
Tolazamide
Tolbutamide
Antineoplastic agents
Bleomycin
Busulfan
Chlorambucil
Cyclophosphamide
Melphalan
Mercaptopurine
Mitomycin
Mitomycin C
Illicit drugs
Cocaine
Heroin
Methadone
Propoxyphene

20. Frequency of pulmonary involvement in various collagen vascular diseases
RadioGraphics, 2002

21. Dermatomyositis-related ILD (UIP pattern)
결체 조직 질환 (CVD)과 연관된 ILD의 예(I) : CVD-related ILD
Dermatomyositis-related ILD (UIP pattern)
M/41, Proximal muscle weakness
Lung Bx
Lung biopsy : irregular intertstitial fibrosis and mononuclear cell infiltration with subpleural predominance
HRCT :
Muscle Bx
UIP pattern

22. Systemic Sclerosis-related ILD (NSIP)
결체 조직 질환 (CVD)과 연관된 ILD의 예(II) : CVD-related ILD
Systemic Sclerosis-related ILD (NSIP)
F/50, progressive dyspnea
Anti-Scl-70 (+)
NSIP pattern

23. Sjogren syndrome-related ILD (LIP)
결체 조직 질환 (CVD)과 연관된 ILD의 예(III) : CVD-related ILD
Sjogren syndrome-related ILD (LIP)
HRCT shows centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts
(curved arrows) were seen in both lungs. (b)
Patholoty : shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows).
LIP pattern

24. Comparison of survival curves

25. Am J Respir Crit Care Med Vol, 2005

27. CHEST ,2006