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The importance of radiological evaluation in the discrimination between UIP and NSIP. Dr. Figen Başaran Demirkazık Hacettepe Ü ni versity Department of R ad iology. HRCT Indications. Chronic disease:

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the importance of radiological evaluation in the discrimination between uip and nsip

The importance of radiological evaluation in the discrimination between UIP and NSIP

Dr. Figen Başaran Demirkazık

Hacettepe Üniversity Department of Radiology

hrct indications
HRCT Indications

Chronic disease:

  • To detect lung diseases in patients who have normal or questionable radiographic abnormalities, who have symptoms or pulmonary function findings suggestive of diffuse lung disease.
  • To analyse and limit the differential diagnosis of parenchymal lung diseases (sarcoidosis, lymphangitic carcinomatosis, histiocytosis X, interstitial fibrozis)
hrct indications1
HRCT Indications

Chronic disease:

  • To assess disease activity and response to treatment
  • As a guide for the need or optimal site and type of lung biopsy
hrct indications2
HRCT Indications

Acute disese:

  • To detect lung diseases in patients who have symptoms of acute lung disease and normal or nondiagnostic chest radiographs, particularly in immunocompromised patients.

acute- chronic dispnea, chest pain, fever of unknown origin, abnormal pulmonary functions, fever after BMT or organ transplantation, infection in AIDS patients

slide8

HRCT

1-1.5 mm

8.5-9 mm

slide9

Slice thickness 5 mm

Slice thickness 1 mm

slide10

Kesit kalınlığı 1 mm

Kesit kalınlığı 1 mm

Soft tissue filter

Bone filter

slide12

ATS, ERS International MultidisciplinaryConsensus Classification of the Idiopathic Interstitial

Pneumonias. Am J RespirCrit Care Med 2002;165:277–304.

usual interstitial pneumonia ipf clinical features
USUAL INTERSTITIAL PNEUMONIA/ IPF-clinical features
  • Patients > 50 years old, a median survival time ranging from 2 to 4 years,
  • Progressive worsening dyspnea and nonproductive cough subtle onset of symptoms
  • Slightly more cases in men than women
  • A history of cigarette smoking seems to be a risk factor
  • Do not respond to highdose corticosteroid therapy;

A combination therapy of cytotoxic drugs and corticosteroids seems to be efficacious for acute exacerbations of IPF.

usual interstitial pneumonia histologic features
USUAL INTERSTITIAL PNEUMONIA-histologic features
  • The histologic hallmark of UIP is the presence of scattered fibroblastic foci
  • Typically, involvement is heterogeneous and areas of normal lung alternate with interstitial inflammation and honeycombing
  • Owing to the patchy lung involvement, histologic evaluation of multiple biopsy specimens from one patient may reveal discordanthistologic patterns.
  • Biopsy samples from more than one lobe should be

obtained in any patient with suspected IIP

  • High resolution CT should serve as a guiding tool

for determining the appropriate anatomic location

usual interstitial pneumonia ipf imaging features
USUAL INTERSTITIALPNEUMONIA/ IPF-imaging features

Chest X-ray:

  • May be normal in early disease
  • In advanced disease, it shows decreased lung volumes and subpleural reticular opacities that increase from the apex to the bases of the lungs
usual interstitial pneumonia ipf imaging features1
USUAL INTERSTITIALPNEUMONIA/ IPF-imaging features

HRCT:

A Trio of signs:

  • Apicobasal gradient
  • Subpleural reticular opacities
  • Macrocystic honeycombing combined with traction bronchiectasis
  • Ground- glass opacities are limited
  • Histologic confirmation should be obtained in patients with atypical imaging findings, such as extensive ground-glass opacities, nodules, consolidation, or a predominantly peribronchovascular distribution
slide21

64 y, M

12 months later

acute exacerbations of ipf
AcuteExacerbations of IPF

Criteria of acute exacerbations

1) Acute worsening of dyspnea within 1 month of presentation

2) New pulmonary infiltrates seen on CXRs or CT scans

3) Deterioration in pulmonary function measurements or gas exchange

4) Absence of an identifiable cause, including infections or cardiovascular disease.

Noth I, CHEST 2007; 132:637–650

acute exacerbations of ipf1
Acute Exacerbations of IPF

HRCT:new diffuse, multifocal, or peripheral ground-glass opacities superimposed on subpleural reticular and honeycombing densities.

Pathology: acute alveolar injury with or without hyaline membrane formation.

Noth I, CHEST 2007; 132:637–650

clinical conditions associated with uip pattern
Clinical conditions associated with UIP pattern
  • Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis
  • Collagen vascular disease
  • Drug toxicity
  • Chronic hypersensitivity pneumonitis
  • Asbestosis
nonspecific interstitial pneumonia clinical features
NONSPECIFIC INTERSTITIAL PNEUMONIA:clinical features
  • 40- 50 years old, F=M
  • Symptoms are similar to those of IPF but usually milder.
  • Gradually worsening dyspnea over several months, with fatigue and weight loss.
  • Cigarette smoking is not a risk factor
  • Majority of patients stabilize or improve with corticosteroids in combination with cytotoxic drugs (cyclophosphamide, cyclosporin)
nonspecific interstitial pneumonia histolojic features
NONSPECIFIC INTERSTITIAL PNEUMONIA:histolojic features
  • Temporally and spatially homogeneous lung involvement
  • Cellular subtype: The thickening of alveolar septa is primarily caused by inflammatory cells
  • Fibrosing subtype: Interstitial fibrosis is seen in addition to mild inflammation
  • Cellular NSIP is less common than fibrosing NSIP but shows a better response to corticosteroids and carries a substantially better prognosis
does nsip evolve into uip
Does NSIP evolve into UIP?

The initial injury in UIP could itself cause secondary inflammation and fibrosis that resemble NSIP. No reports have documented the progression of NSIP to UIP.

nonspecific interstitial pneumonia imaging features
NONSPECIFIC INTERSTITIAL PNEUMONIA:imaging features

Chest X-ray:

In early NSIP: the chest radiograph is normal.

In advanced NSIP: bilateral pulmonary infiltrates

The lower lung lobes are more frequently involved, but an obvious apicobasal gradient, as seen in UIP, is usually missing.

nonspecific interstitial pneumonia imaging features1
NONSPECIFIC INTERSTITIAL PNEUMONIA:imagingfeatures

HRCT:

Subpleural and rather symmetric distribution.

Patchy ground-glass opacities are combined with irregular linear or reticular opacities

In advanced disease: traction bronchiectasis and consolidation

Ground-glass opacities remain the most obvious HRCT sign

Subpleural cysts are smaller and limited in extent than those of UIP.

Microcystic honeycombing: NSIP

Macrocystic honeycombing: UIP

Fibrotic NSIP: honeycombing, traction bronchiectasis and intralobular reticular opacities.

clinical conditions associated with nsip pattern
Clinical conditions associated with NSIP pattern
  • Idiopathic NSIP
  • Collagen vascular disease
  • Hypersensitivity pneumonitis
  • Drug-induced pneumonitis
  • Infection
  • Immunodeficiency including HIV infection
slide57

UIP(n = 44) NSIP (n = 50)

Craniocaudal plane

Upper zone predominance 2 (4.5) 1 (2)

Middle zone predominance 1 (2) 1 (2)

Lower zone predominance 37 (85) 45 (90)

Equal in all zones 4 (9) 3 (6)

Axial plane

Peripheral predominance 42 (95) 37 (74)

Central Predominance 1 (2) 0

Diffuse distribution 1 (2) 13 (26) Homogeneity

Patchy distribution 27 (61) 15 (30)

Confluent distribution 17 (39) 35 (70) Ancillary findings

Mediastinal adenopathy 20 (45) 27 (54)

Pulmonary artery enlargement 8 (18) 14 (28).

Eliot TL, J Comput Assist Tomogr 2005;29:339

slide58
The presence of honeycombing as a predominant feature had a specificity of 96%, sensitivity of 41%, positive predictive value of 90%, and negative predictive value of 64% for UIP (P, 0.001).
  • The pattern of predominant ground-glass attenuation + reticular opacity with minimal to no honeycombing had a sensitivity of %96 and a specificity of 41% for the diagnosis of NSIP. The absence of honeycombing as a predominant feature has a high negative predictive value for NSIP (90%).

Eliot TL, J Comput Assist Tomogr 2005;29:339

nsip vs uip
NSIP vs UIP

The key CT features in the diagnosis of NSIP over UIP:

  • Homogeneous lung involvement
  • Without an obvious apicobasal gradient
  • Extensive ground-glass abnormalities,
  • Finer reticular pattern
  • Absence of honeycombing
  • Relative subpleural sparing*

*Silva CI, Radiology. 2008; 246: 288

nsip vs uip1
NSIP vs UIP

Follow-up:

In UIP: progression of ground-glass attenuation to honeycombing is common and indicates irreversible fibrosis

In NSIP: ground-glass opacities usually do not progress to areas of honeycombing, even if there is associated bronchiectasis

  • At follow-up CT, 28% of patients with initial CT findings suggestive of NSIP progressed to findings suggestive of IPF.*

*Silva CI, Radiology 2008; 247:251

prognosis
Prognosis

Median survival time: NSIP:12 years

UIP: 2.8 years

5-year survival rates : Cellular NSIP: 100%

Fibrotic NSIP: 90%

UIP : 43%

10-year survival rates: Cellular NSIP: 100%

Fibrotic NSIP: 35%

UIP : 15%

Cellular NSIP > Fibrotic NSIP > UIP/IPF.

Leslie KO, CHEST 2005; 128:513S

slide63
Sens % Spec %

IPF- clinical diag. 62 97

IPF- radiologic diag. 78.5 90

Non IPF- ILD : clinical diag. 88.8 40

Non IPF- ILD: radiologic diag. 59 40

Raghu G, CHEST 1999; 116:1168–1174

correct diagnoses of hrct according to disease
Correct Diagnoses of HRCT According to Disease

Correct Diagnosis Correct Diagnosis With High Level of Confidence

NSIP (n=36) 79.2 65.3

UIP (n=11) 100 90.9

COP (n=8) 75.0 43.8

AIP (n=10) 75.0 40.0

DIP or RB-ILD 35.7 32.1

LIP (n = 11) 95.5 81.8

Total (n = 90) 76.1 60.6

Tsubamoto M, J Comput Assist Tomogr 2005;29:793

ats ers criteria for diagnosis of ipf in the absence of surgical lung biopsy
ATS-ERS Criteria for Diagnosis of IPF in the Absence of Surgical Lung Biopsy

Major criteria

Exclusion of other known causes of interstitial lung disease (eg, toxic effects of certain drugs, environmental exposures,and connective tissue diseases)

Abnormal results of pulmonary function studies, including evidence of restriction (reduced vital capacity, oftenwith an increased FEV1/FVC ratio) and impaired gas exchange (increased P(A-a)o2 decreased Pao2 withrest or exercise, or decreased Dlco)

Bibasilar reticular abnormalities with minimal ground-glass opacities at high-resolution CT

Transbronchial lung biopsy or bronchoalveolar lavage shows no features to support an alternative diagnosis

Minor criteria

Age > 50 y

Insidious onset of otherwise unexplained dyspnea on exertion

Duration of illness > 3 mo

Bibasilar inspiratory crackles (dry or “Velcro” type)

ATS, ERS International MultidisciplinaryConsensus Classification of the Idiopathic Interstitial

Pneumonias. Am J RespirCrit Care Med 2002;165:277–304.

potential limitations of clinical criteria for the diagnosis of ipf cfa
Potential Limitations of Clinical Criteria for theDiagnosis of IPF/CFA

HRCT % ATS %

Sensitivity 71 71

Specificity 67 75

PPV 71 77

NPV 67 69

Accuracy 69 73

Peckham RM, Respiration 2004;71:165

should you biopsy every patient
Should You Biopsy Every Patient?
  • Given the potential risks and a better definition of

the diagnostic accuracy of a HRCT scan diagnosis,

SLB is not required in all patients with suspected

IPF.

  • It is becoming increasingly accepted that a

highly suggestive clinical presentation, including typical

HRCT scan findings, can be used in the absence

of a lung biopsy specimen to make a likely diagnosis of IPF

Noth I, CHEST 2007; 132:637

slide68
As a general rule, a suspected diagnosis of NSIP will always require a surgical biopsy, whereas a confident diagnosis of IPF without a biopsy is usually correct.

du Bois R,Thorax 2007;62:1008

what is the purpose of performing a surgical biopsy
What is the purpose of performing a surgical biopsy?

1. Knowing at presentation that an individual has NSIP and not UIP

allows the clinician to convey a more optimistic prognosis;

2. A short course of higher dose corticosteroids may have a significantly better efficacy/side effect profile in NSIP than in UIP

3. Even with alternative (immunosuppression) first-line treatment

approaches, the balance of likely good versus adverse effects can be articulated with more precision to an individual patient rather than

quoting average survival and side effect data.

4. Trials of new treatment are being undertaken on individuals with

well defined disease in order to maximise the likely response to the

novel agent.

du Bois R,Thorax 2007;62:1008

slide74
52 years old, M
  • He presented with left back and left flank pain.
  • There was no finding on physical examination.
slide78
Pathology:

Homogeneous fibrozis with intraalveolar macrophage aggregates

NSIP? DIP?

slide79
38 years old, male
  • Cough in cold weather
  • Dispnea for 13 months
slide82

Diagnosis?

3259230mustafakırat

uip nsip
HRCT Features

Ground-glass opacities

Symmetric irregular linear or reticular opacities

Consolidation

Microcystichoneycombing

UIP NSIP

HRCT Features

Reticular opacities- interlobular septal thickening

Macrocystic honeycombing,

Traction bronchiectasis,

Architectural distortion,

Focal ground-glass opacity

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