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

1. The importance of radiological evaluation in the discrimination between UIP and NSIP Dr. Figen Başaran Demirkazık Hacettepe Üniversity Department of Radiology

4. 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)

5. 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

6. 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

8. HRCT 1-1.5 mm 8.5-9 mm

9. Slice thickness 5 mm Slice thickness 1 mm

10. Kesit kalınlığı 1 mm Kesit kalınlığı 1 mm Soft tissue filter Bone filter

12. ATS, ERS International MultidisciplinaryConsensus Classification of the Idiopathic Interstitial Pneumonias. Am J RespirCrit Care Med 2002;165:277–304.

13. 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.

14. 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

16. 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

19. 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

20. UIP

21. 64 y, M 12 months later

22. 67 y, M

31. IPF

32. 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

33. 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

35. Clinical conditions associated with UIP pattern • Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis • Collagen vascular disease • Drug toxicity • Chronic hypersensitivity pneumonitis • Asbestosis

36. RA

37. RA

38. 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)

39. 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

41. 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.

42. 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.

43. 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.

44. Fibrosing NSIP

45. Cellular NSIP

47. 60 y , F

48. 53 y, M

50. Pathology:NSIP