Imaging features of ILD

1. Dr Nivedita Charkrabarty
Dr Aayush
Dr Swetha
Interstitial lung disease

2. Definition
• Heterogeneous group of diffuse lung diseases occurring without known cause
and associated with varying degrees of interstitial lung inflammation and
fibrosis.

3. Classification ILDs
Major
Chronic Fibrosing
1. UIP
2. NSIP
Smoking related
3. DIP
4. RB
Acute/Subacute
5. Organising
pneumonia
6. DAD
Rare
7. Lymphoid IP
8. Pleuropulmonary
fibroelastosis

4. Findings?

6. USUAL INTERSTITIAL PNEUMONIA AND IDIOPATHIC PULMONARY FIBROSIS
• Most common, 40%
• UIP: histologic pattern, IPF: disease
• Heterogeneous pattern: "spatial" and "temporal“ heterogeneity
• >50 yrs
• progressive dyspnea, cough, weight loss, and finger clubbing
• PFT: restrictive pattern

7. Clinical associations
• Collagen-vascular disease,
• Asbestosis,
• Drug toxicity,
• Radiation,
• Chronic hypersensitivity pneumonitis, or familial pulmonary fibrosis

8. • In early stages, the only HRCT abnormality may be fine reticulation
• Honey combing 3mm to 2 cm, 3 in a row
• reticular opacities
• traction bronchiectasis
• subpleural and lower lobe predominance
• GGOs are rare

10. Combined pulmonary fibrosis and emphysema
Diagnostic Criteria ATS and ERS
• 1. A UIP pattern on HRCT or histopathology
• 2. The absence of an alternative etiology (e.g., collagen-vascular disease,
asbestos exposure, drug treatment, hypersensitivity pneumonitis) after careful
clinical evaluation (i.e., history, physical examination, pulmonary function
testing, and laboratory assessment)

11. UIP pattern
• 1. Subpleural and basal predominance
• 2. Reticular abnormality
• 3. Honeycombing with or without traction
bronchiectasis
• 4. Absence of inconsistent features

12. Possible UIP
• 1. Subpleural and basal predominance
• 2. Reticular abnormality
• 3. Absence of inconsistent features

13. Inconsistent with UIP
• 1. Upper or midlung predominance
• 2. Peribronchovascular predominance
• 3. Extensive ground-glass abnormality (extent > reticulation)
• 4. Profuse micronodules (bilateral, predominantly upper lobes)
• 5. Discrete cysts (multiple, bilateral, away from areas of honeycombing)
• 6. Diffuse mosaic attenuation/air trapping (bilateral in three or more lobes)
• 7. Segmental or lobar consolidation

16. Radiography
• CXR: subtle
increase in
opacity at lung
base

17. NONSPECIFIC INTERSTITIAL PNEUMONIA
• Less common
• Specific pathological entity
• Alveolar wall thickening or fibrosis on HPE
• Spatial and temporal homogeneity
• Cellular NSIP
• Fibrotic NSIP

18. Presentation
• Younger age than UIP 40-50 yrs
• 18 to 30 months symptom duration
• Responds to steroids
• Good prognosis

19. Fibrotic NSIP

20. • Ground-glass opacity or reticulation
• “without“ honeycombing
• subpleural and basal lung
• subpleural sparing

22. ORGANIZING PNEUMONIA (CRYPTOGENIC ORGANIZING PNEUMONIA)
• Patchy areas of organizing pneumonia
• mononuclear cells, foamy macrophages, and organizing fibrosis
• peripheral airspaces, including bronchioles, alveolar ducts, and alveoli
• 10 to 20%, common
• BOOP
• Idiopathic OP: COP
• A/w CVD (Myositis syndromes), pulmonary infection, drug reactions, bronchial
obstruction, aspiration, and after toxic fume inhalation

23. Presentation
• Several month h/o non productive cough, low grade fever, malaise, dyspnea
• ~55 years
• Symptom duration shorter, more systemic symptoms
• Steroid responsive
• Good prognosis

24. Radiography

25. Patterns
• Patchy consolidation or GGO (60%), subpleural and/or peribronchial
distribution
• Small, ill-defined nodules, peribronchial or peribronchiolar
• Large nodules or masses, which may be irregular in shape
• Focal or lobar consolidation
• reversed halo sign or atoll sign m/c drug reactions
• Perilobular pattern m/c with myositis syndromes
• Overlapping features with CEP

26. Differentials of Atoll/Reverse halo sign
• Infectious diseases: invasive aspergillosis, histoplasmosis, cryptococcosis,
PCP, TB, Legionella
• Non infectious: COP, Secondary organizing pneumonia
• Vasculitis
• Sarcoidosis
• Lipoid pneumonia
• Lymphomatoid granulomatosis

27. ACUTE INTERSTITIAL PNEUMONIA
• Fulminant disease of unknown cause
• Histologically DAD
• ~50 yrs, prodrome viral like
• mechanical ventilation within 2 weeks
• Hamman Rich syndrome/ Idiopathic ARDS
• Poor prognosis

29. DESQUAMATIVE PNEUMONIA, RESPIRATORY BRONCHIOLITIS,
RESPIRATORY BRONCHIOLITIS-INTERSTITIAL LUNG DISEASE
• Different degrees of lung involvement by the same process
• RB mildest, DIP most severe
• Alveolar macrophagic infiltrate: diffuse in DIP, peribronchiolar in RB
• Strong association with smoking, with cessation prognosis is very good

31. RBILD
Upper lobe predominance

32. LYMPHOID INTERSTITIAL PNEUMONIA
• histology: dense lymphoid interstitial infiltrate
• Extensive alveolar wall involvement
• Small airways with lymphoid follicles: Follicular bronchiolitis
• A/w CVD, immune deficiency, autoimmune disorder, drug toxicity

33. Patterns
• Patchy areas of GGO: immune deficiency
• Centrilobular nodules, Ill defined: AIDS, CVD
• Small, well-defined nodules perilymphatic distribution or
septal thickening: AIDS
• Isolated cystic airspaces: smog rents and other cvds

36. PLEUROPARENCHYMAL FIBROELASTOSIS
• Upper lobe predominant elastotic fibrosis of pleura and adjacent parenchyma
• Temporally homogeneous
• Idiopathic or a/w nonspecific autoantibodies, familial, BMT, lung transplantation with
restrictive allograft syndrome, or infection
• Dry cough, SOB for several yrs
• Progressive
• predominantly upper lung zone pleural thickening
• subpleural reticulation
• upper lobe volume loss
• upward retraction of the hila

38. LYMPHANGIOLEIOMYOMATOSIS
• progressive proliferation of perivascular epithelioid cells (PECs) in relation to
bronchioles, small pulmonary vessels, and lymphatics in the chest and
abdomen
• low-grade destructive metastasizing malignancy
• Associated with Tuberous sclerosis
• almost exclusively in women of childbearing age
• dyspnea, pneumothorax, or cough
• 60% chylous pleural effusions; 80% pneumothoraces; 30% to 40% blood-
streaked sputum or frank hemoptysis

41. Summing Up!

42. Case Profile: 63 year old male, presenting with progressive dyspnea, with
new onset requirement of oxygen aid.

43. Case Profile: An 33 year old female, known case of ALL, post
BMT, presenting with new onset shortness of breath, fever,
chills and cough.

44. Diagnosed with Invasive aspergillosis, and
started on Voriconazole.

45. Known case of pulmonary tuberculosis, with
increasing breathlessness, malaise and one
episode of hemoptysis. Sign?

46. Air crescent sign/ Monod Sign of fungal ball

47. Case Profile: Young female patient of Hodgkin’s
lymphoma on ABVD regimen, presenting with
dyspnea, cough and occasional fever.
Condition and likely causative agent?

48. • Diffuse alveolar Damage
• Bleomycin

49. ThankYou!u