I Parenchymal lung disease

a

Acute ILD

69
b

Subacute ILD

175
c

Eosinophilic pneumonia (PIE)

144
d

Organizing pneumonia (OP)

87
e

Acute eosinophilic pneumonia (AEP)

17
f

Acute fibrinous organizing pneumonia (AFOP)

6
g

Pulmonary fibrosis

64
h

Subclinical ILD

8
i

Diffuse pulmonary calcification

5
j

Exogenous lipoid pneumonia

8
k

Lung nodule or nodules

18
l

Diffuse alveolar damage (DAD)

50
m

ILD with a granulomatous component

23
n

Pulmonary alveolar proteinosis (PAP)

7
o

Localized pulmonary fibrosis

5
p

Interstitial foreign body reaction/granulomas

7
q

Pulmonary nodulosis

4
r

Endogenous lipoid pneumonia (phospholipidosis)

2
s

A mass or masses

4
t

Pneumoconiosis-talcosis

6
u

'Recall' radiation pneumonitis

5
v

Subclinical changes in PFTs

13
w

Rapidly progressive pulmonary fibrosis

14
x

Biapical fibrosis

4
y

Exacerbation of preexisting ILD or fibrosis

8
z

Areas of consolidation

7
aa

Deterioration of pulmonary LAMM

1
ab

Radiation-induced lung injury (RILI)

1
ac

Relapsing pneumonitis

1

II Pulmonary edema - ALI - ARDS

a

Noncardiogenic pulmonary edema - NCPE

151
b

ALI - ARDS

148
c

ARDS and the hemolytic-uremic syndrome

3
d

Cardiogenic pulmonary edema

13
e

Transfusion-related ALI (TRALI)

4
f

Isolated hypoxemia/arterial desaturation

10
g

Negative pressure pulmonary edema

6
h

Overload pulmonary edema

3
i

Flash pulmonary edema

4
j

Transient pulmonary opacities

6
k

The ATRA syndrome

1

III Pulmonary hemorrhage

a

Diffuse alveolar hemorrhage - DAH

79
b

Pneumorenal syndrome

9
c

Hemoptysis

16
d

ANCA-associated DAH

7
e

Pulmonary hematoma

1

IV Airway involvement

a

Bronchospasm - wheezing - asthma

130
b

Bronchospasm and angioedema

10
c

Obliterative bronchiolitis

15
d

Lone cough

31
e

Acute violent coughing

6
f

Severe or catastrophic bronchospasm

24
g

Respiratory bronchiolitis ILD (RB-ILD)

1
h

Tracheobronchial necrosis

3
i

Foreign body bronchiolitis - Diffuse aspiration bronchiolitis

3
j

Calcification of cartilaginous rings

2
k

Bronchiectasis

4
l

Bronchial stump dehiscence

1
m

Bronchial stricture/stenosis

2
n

Obstructive airway dysfunction

5
o

Airway hyperresponsiveness

4
p

Thermal, chemical or caustic airway injury

0
q

Reactive airway dysfunction syndrome (RADS)

2

V Pleural and/or pericardial involvement

a

Pleural effusion, lone

60
b

Eosinophilic pleuritis/effusion

28
c

Pleural thickening/fibrosis

22
d

Pleural and/or pericardial effusion and the lupus syndrome

28
e

Serosanguineous effusion - Hemothorax

9
f

Pneumothorax

20
g

The ovarian hyperstimulation syndrome (OHSS)

4
h

Chylothorax

5
i

Acute pleuropulmonary reaction

10
j

Subpleural fat deposits

1
k

Pleural calcifications

1
l

Malignant mesothelioma

2
m

Pleuro-pericardial effusion

24
n

Bloody pericardial effusion - Hemopericardium

1
o

Acute pleuric chest pain

1
p

Pleuroparenchymal fibroelastosis

2
q

Empyema

1
r

Pleural mass or masses

1
s

Polyserositis

1

VI Pulmonary vasculopathy

a

Pulmonary thromboembolism

38
b

Pulmonary arterial hypertension

36
c

Pulmonary veno-occlusive disease

9
d

Pulmonary vasculitis/capillaritis

18
e

Lipid/fat embolism

10
f

The hemolytic-uremic syndrome

8
g

Foreign body pulmonary vasculopathy

10
h

Acrylic cement embolism

3
i

Acute pulmonary hypertension

6
j

Mercury embolism

2
k

Eosinophilic pulmonary vasculitis

3
l

Acute pulmonary vasoconstriction and consequent PHTn

4
m

Silicone pulmonary embolism

1
n

Crystal pulmonary embolism

3
o

Air embolism (right-sided)

1
p

Angiomatoid pulmonary vasculopathy

1
q

Rebound pulmonary hypertension

2
r

Septic pulmonary emboli

1
t

Pulmonary artery aneurysm

1
u

Pulmonary microthrombi

1
v

Foreign body embolism

1

VII Mediastinal involvement

a

Lymphadenopathy

15
b

Lymphadenopathy with reactive changes

4
c

Mediastinal lipomatosis

1
d

Fibrosing mediastinitis

5
e

Granulomatous lymphadenopathy

3
f

Enlarged thymus

1
g

Mediastinal bleeding/hemorrhage

5
h

Pneumomediastinum

3
i

Mediastinitis

1
j

Compression of mediastinal organs

1
k

Mediastinal infiltration

1

VIII Central/large airway involvement/closure

a

Angioedema

58
b

Airway hematoma causing UAO

9
c

UAO other than angioedema or hematoma

12
d

Thermal, chemical or caustic airway injury

9
e

Laryngospasm

6
f

Central airway instability/malacia

1
g

Tracheal narrowing - Sabre sheath trachea

1
h

Vocal cord dysfunction, adduction or closure

3
i

Midline destructive lesions

1
j

Dysphonia

1
k

Airway fire/explosion

2
k

Submucosal tracheal bleeding

1
m

Drug-associated airway foreign body

1
o

Perforation of the nasal septum

1
q

Tongue involvement

3

IX Neuromuscular involvement

a

Respiratory muscle weakness or paralysis

16
b

Respiratory dyskinesia

3
c

Chest wall/muscle rigidity

3
d

Ventilatory depression - Apnea

32
e

Disordered breathing during sleep

6
f

Hiccup

14
g

Myopathy of respiratory muscles

2
h

Isolated dyspnea

2

X Systemic reactions or conditions

a

DRESS

54
b

Antiphospholipid antibody syndrome (APLS)

10
c

Vascular/capillary leak syndrome

11
d

Drug-induced lupus

47
e

Autoimmunity

10
f

Anaphylaxis

115
g

Hypersensitivity reaction

36
h

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

14
i

Immune reconstitution syndrome - IRS/IRCS

2
j

Myopathy - Myositis

8
k

Sarcoid-like disease

9
l

Subclinical ANA

4
m

Systemic vasculitis

17
n

Tumor lysis syndrome

5
o

Sweet syndrome

6
p

Serum sickness-like reaction

2
q

Systemic inflammatory response

3
r

Fluid retention

7
s

ANCA-related disease or vasculitis

16
t

Jarisch-Herxheimer reaction

3
u

MOF/MODS

10
v

Systemic eosinophilic syndrome

1

XI Miscellaneous

a

Cyanosis, low SpO2 and a normal measured PaO2

14
b

Acute non-pleuritic chest pain/tightness/pressure

32
c

Acute pleuritic chest pain

8
d

Metabolic acidosis

5
e

Opportunistic pulmonary infection

31
f

Opportunistic airway infection

2
g

Aspiration pneumonia

10
h

Addictive risk

3
i

Isolated hypercapnea

1
j

Vertebral compression fracture

2
k

Rib hypertrophy, periostitis

1
l

Pharmacobezoar

0
m

Pulmonary emphysema - Lung bullae

4
n

Neutropenia - Agranulocytosis

10
o

Cavitary pulmonary opacities

4
p

Pneumocystis jiroveci pneumonia

7
q

Pneumocystis jiroveci colonization

3
r

Death from inhalation of compound

2
s

Tumoral cavitation

2
t

Lung abscess or abscesses

2
u

Pseudocyanotic skin discoloration

1
v

Mycobacterial (M tuberculosis-NTM-BCG) intrathoracic disease

12
w

Chest deformity - Platythorax

1
x

Lung compression from ascites

1
y

Death from overdose

16
z

Cutaneous vasculopathy (occlusive or vasculitic)

2
aa

Fire-eater's lung

1
ab

Yellow nail syndrome

3
ac

Chronic chest or musculoskeletal pain

3
ad

Rib fracture

3
ae

Esophageal tear/rupture: Boerhaave syndrome

1
af

Esophageal ulceration/stenosis/stricture

2
ag

Localized chest pain

1
ah

Superior vena cava syndrome

1

XII Heart/circulatory involvement

a

Left ventricular failure

19
b

Valvular heart disease

17
c

Pericardial effusion or tamponade

23
d

Myocarditis

11
e

Tako-tsubo cardiomyopathy

6
f

Cardiomyopathy

27
g

Acute coronary syndrome - Myocardial infarction

20
h

Eosinophilic myocarditis

4
i

Endocarditis

1
j

Myocardial stunning

3
k

Pericardial thickening/constriction

5
l

Arrhythmias - Dysrhythmias

8
m

Cardiopulmonary arrest

6
n

Hypotension, cardiovascular collapse, shock

7
o

Coronary artery disease

3
p

Long QT syndrome

2
r

Coarctation of the aorta (acquired)

1
r

Congestive heart failure

0
s

Heart block

3
t

'Torsades de pointe'

1
u

Left atrial/ventricular thrombosis

1
v

Arterial hypotension

1
w

Hypertrophic cardiomyopathy

1

XIII Neoplasias - Neoplasms

a

Solid tumors including lung cancer

4
b

Lymphoproliferative disease

5
c

Leukemia

1
d

Malignant mesothelioma

2

XIV Hemoglobinopathies, acquired

a

Methemoglobinemia

33
b

Sulfhemoglobinemia

2
c

Carboxyhemoglobinemia

3
d

Interference with COHb measurement

1
e

Interference with SpO2 measurement

1

XV Pathology

a

Path: Cellular ILD - NSIP-pattern

13
b

Path: Eosinophilic pneumonia (subacute or acute)

3
c

Path: Organizing pneumonia pattern

14
d

Path: Acute fibrinous organizing pneumonia, AFOP-pattern

7
e

Path: ILD with a granulomatous component

17
f

Path: Diffuse alveolar damage (DAD) - Hyaline membranes

17
g

Path: Diffuse alveolar hemorrhage (DAH)

2
h

Path: Pulmonary fibrosis (NSIP/fibrotic-pattern)

10
i

Path: A reactive alveolar or bronchiolar epithelium

7
j

Path: UIP-pattern

1
k

Path: DIP-pattern

11
l

Path: GIP-pattern

1
m

Path: Lymphoid hyperplasia, LIP-pattern

4
n

Path: PAP-pattern - secondary PAP

1
o

Path: Endogenous lipoid pneumonia - phospholipidosis

2
p

Path: Exogenous lipoid pneumonia

2
q

Path: Interstitial foreign body deposits/reaction

2
r

Path: Smudged geographic necrosis

1
s

Path: Pneumoconiosis, talcosis, talcoma

2
t

Path: Pulmonary amyloid deposits

1
u

Path: Crystal storage disease

1
u

Path: Diffuse pulmonary calcification

0
u

Path: Pulmonary edema

3
v

Path: Subacute/acute cellular bronchiolitis

1
w

Path: RB-ILD

1
x

Path: Obliterative broncholitis

4
y

Path: Foreign body bronchiolitis

0
z

Path: Pulmonary capillaritis

1
aa

Path: Pulmonary vasculitis other than capillaritis

5
ab

Path: Eosinophilic vasculitis

0
ac

Path: Fat embolism

2
ad

Path: Silicone embolism/deposits

1
ae

Path: Foreign body microangiopathy/vasculopathy

2
af

Path: Elemental mercury embolism

0
ag

Path: Cement embolism

0
ah

Path: Crystal pulmonary embolism

0
ai

Path: Venoocclusive disease (PVOD)

1
aj

Path: Pulmonary hypertension incl. angiomatoid vasculopathy

0
ak

Path: Pleuritis n.o.s.

0
al

Path: Eosinophilic pleuritis

0
am

Path: Pleural fibrosis

0
an

Path: Pleuroparenchymal fibroelastosis

1
ao

Path: Kayexalate lung

1
ap

Path: Alveolar carbonaceous deposits

1
aq

Path: Fire eater's lung

1
ar

Path: Pulmonary microvascular granulocyte sequestration

1
as

Path: Deterioration of pulmonary LAMM

1
at

Path: Textiloma - Gossypiboma

0
au

Path: Occlusive skin vasculopathy

2
av

Path: Siliconoma

1
aw

Path: Smoking-related interstitial fibrosis (SRIF)

1
ax

Path: Lymphadenopathy with reactive changes

0
ay

Path: Granulomatous lymphadenopathy

1
az

Path: Eosinophilic bronchiolitis

1
ba

Path: Opportunistic pulmonary infection

1
bb

Path: Bronchiolar vasculitis and necrosis

1
bc

Path: Interstitial pulmonary edema

1
bd

Path: Pulmonary necrotizing granulomas

0
be

Path: Tracheal or bronchial wall ulceration

0
bf

Path: Foreign body reaction in large airway wall

1

XVI Imaging

a

Imaging: Diffuse haze

1
b

Imaging: Ground-glass opacities (GGO)

4
c

Imaging: Mosaic pattern of attenuation

0
d

Imaging: Batwing or butterfly pattern

0
e

Imaging: Subpleural pulmonary opacities

1
f

Imaging: Bibasilar opacities

1
g

Imaging: Diffuse whiteout

0
h

Imaging: Biapical or mid-lung opacities

0
i

Imaging: Areas of involvement with a recognizable anatomic distribution

1
j

Imaging: Wandering pulmonary opacities

0
k

Imaging: Areas of consolidation

3
l

Imaging: Peribronchovascular consolidation

0
m

Imaging: Interlobular septal thickening

0
n

Imaging: Intralobular septal thickening - Crazy paving

1
o

Imaging: Localized ground-glass

0
p

Imaging: Reticulation

0
q

Imaging: An IPF-like imaging pattern

0
r

Imaging: Areas of involvement with low attenuation numbers

2
s

Imaging: Areas of involvement with high attenuation numbers

1
u

Imaging: Diffuse random micronodules

0
v

Imaging: Diffuse centrilobular micronodules

1
w

Imaging: Multiple lung nodules

1
x

Imaging: Halo sign

0
y

Imaging: Reversed halo (Atoll) sign

0
z

Imaging: A large nodule, nodules or masses

0
aa

Imaging: Nodules, mass or masses with a central area of low attenuation

0
ab

Imaging: Cavitary lung nodules

0
ac

Imaging: XXX

0
ad

Imaging: A calcified mass or masses

0
ae

Imaging: Progressive massive fibrosis (PMF)

0
af

Imaging: Lung bullae or cysts

0
ag

Imaging: Pulmonary emphysema

0
ah

Imaging: Pneumatoceles

1
ai

Imaging: A tree-in-bud pattern

0
aj

Imaging: Bronchiectasis

0
ak

Imaging: Calcification of cartilaginous rings

0
al

Imaging: Bronchial wall thickening

0
am

Imaging: XXX

0
an

Imaging: Pleural effusion

0
ao

Imaging: Massive pleural effusion

0
ap

Imaging: Pleural thickening

0
aq

Imaging: Pneumothorax

0
ar

Imaging: Hydropneumothorax

0
as

Imaging: Pleural mass or masses

0
at

Imaging:

0
au

Imaging:

0
av

Imaging:

0
aw

Imaging:

0
ax

Imaging: Vasc

0
ay

Imaging: Vasc

1
az

Imaging: Vasc

0
ba

Imaging:

0
bb

Imaging: Pneumomediastinum

0
bc

Imaging: Lymph node enlargement

0
bd

Imaging:

0
be

Imaging: Enlarged thoracic venous collaterals

0

XVII Infection

a

Pneumonia n.o.s.

3
b

Bacterial pneumonia

0
c

Pulmonary aspergillosis

4
d

Pneumocystis jiroveci colonization

1
e

Pneumocystis jiroveci pneumonia

10
f

Viral pneumonia

0
g

Tuberculosis

3
h

Pulmonary NTM infection

2
i

BCG infection

2
j

Pulmonary mucormycosis

1
k

Bacterial colonization of the airways

1
l

Fungal colonization of the airways

0
m

Pseudomembranous Aspergillus tracheobronchitis

1

XVIII Distinctive signs and/or symptoms: eye catchers

a

x

0
b

x

0
c

x

0
d

x

0
e

x

0
f

x

0
g

x

0
h

x

0
j

x

0
k

Eye-catcher: Multiple radiolucent balls

2
l

Eye-catcher: Tongue edema

1
m

Eye-catcher: Acrylic cement embolism

2
n

Eye-catcher: Lipiodol pulmonary embolism

1

XIX Cytology (BAL, pleural fluid, FNA) - BAL fluid

a

Cytology: Lymphocytic BAL

1
b

Cytology: Neutrophilic BAL

1
c

Cytology: Eosinophilic BAL

0
d

Cytology: Hemorrhagic BAL

0
e

Cytology: Stainable iron-laden macrophages in BALF

0
f

Cytology: Foamy (phospholipidotic) macrophages (BALF, pleural fluid, FNA)

1
g

Cytology: Atypical alveolar cells in BALF

0
h

-

0
i

Cytology: Multinucleated giant cells in BALF

0
j

Free mineral oil in BALF

0
k

Cytology: Talc crystals in macrophages or lying freely in BALF

1

XX Diagnostic criteria for DIRD