HRCT in Diffuse Interstitial Lung Disease

1. HRCT in Diffuse Interstitial Lung Disease Cases Seminar

2. HRCT in Diffuse Interstitial Lung Disease High Resolution CT Diagnosis • Pattern of abnormality • Distribution of disease • Associated findings • Clinical history

3. HRCT in Diffuse Interstitial Lung Disease Steps in HRCT DiagnosisImportant Questions for the Radiologist What is/are the predominant CT pattern(s)? What is/are the likely pathological process? What is the diagnosis?

4. HRCT in Diffuse Interstitial Lung Disease Case 1Pulmonary infiltrates, dry cough, and fever for one month duration

5. HRCT in Diffuse Interstitial Lung Disease Case 1Pulmonary infiltrates, dry cough, and fever for one month duration

6. HRCT in Diffuse Interstitial Lung Disease Case 1Pulmonary infiltrates, dry cough, and fever for one month duration

7. HRCT in Diffuse Interstitial Lung Disease Case 1Pulmonary infiltrates and fever for one month duration Pattern of abnormality & distribution: • Multifocal consolidations & GGO • Peribroncho-vascular Differential diagnosis: Infection; Cryptogenic organizing pneumonia; Chronic eosinophilic pneumonia; Kaposi´s sarcoma; Vasculitis; BACC; Lymphoma Cryptogenic organizing pneumonia

8. HRCT in Diffuse Interstitial Lung Disease Cryptogenic organizing pneumonia A flu-like illness may precede the onset of symptoms in 1/3 of cases Imaging findings: • Patchy, nonseg, pph airspace consolidation • Peribronchovascular distribution - Irregular linear opacities - Small nodules; isolated or associated with consolidation

9. HRCT in Diffuse Interstitial Lung Disease Case 2Gradually onset of mild dyspnea and chronic cough in a mild smoker

10. HRCT in Diffuse Interstitial Lung Disease Case 2 Gradually onset of mild dyspnea and chronic cough in a mild smoker

11. HRCT in Diffuse Interstitial Lung Disease Case 2 Gradually onset of mild dyspnea and cough in a mild smoker Pattern of abnormality & distribution: • Ill-defined nodules • Centrilobular, bilateral & upper lobes Differential diagnosis: • Infection • Respiratory bronchiolitis • Hypersensitivity pneumonitis Respiratory Bronchiolitis

12. HRCT in Diffuse Interstitial Lung Disease Respiratory Bronchiolitis • Common incidental finding in cigarette smokers (“smokers’ bronchiolitis”) • Usually not associated with any symptoms • Small % of patients present with chronic cough and progressive SOB

13. HRCT in Diffuse Interstitial Lung Disease Case 3Is this an interstitial lung disease?42 year-old female with several pneumothoraces. Moderate dyspnea

14. HRCT in Diffuse Interstitial Lung Disease Case 3 Is this an interstitial lung disease?

15. HRCT in Diffuse Interstitial Lung Disease Case 3 Is this an interstitial lung disease?

16. HRCT in Diffuse Interstitial Lung Disease Case 3 Is this an interstitial lung disease? Pattern of abnormality & distribution: • Cystic • Diffuse and bilateral Differential diagnosis: • None • Emphysema ? Lymphangioleiomyomatosis

17. HRCT in Diffuse Interstitial Lung Disease Lymphangioleiomyomatosis • Uncommon interstitial lung disease (33 cases at AFIP between 1960 and 2003) • Exclusively affects women during the reproductive years • Abnormal proliferation of LAM cells: lungs, thoracic and retroperitoneal lymphatics • Large lung volumes (50%) • Bilateral uniform small thin-walled cysts • Chylothorax and pneumothoraces

18. HRCT in Diffuse Interstitial Lung Disease Case 456 year-old male with progressive hypoxia

19. HRCT in Diffuse Interstitial Lung Disease Case 4 Progressive hypoxia

20. HRCT in Diffuse Interstitial Lung Disease Case 4 Progressive hypoxia Pattern of abnormality & distribution: • Reticular pattern with honeycombing • Peripheral, subpleural, bilateral & symmetric Differential diagnosis: • Asbestosis • Chronic HP • CVD UIP pattern

21. HRCT in Diffuse Interstitial Lung Disease Usual Interstitial Pneumonia • M/C type of interstitial pneumonia • Idiopathic in majority of patients; corresponds to clinical diagnosis of idiopathic pulmonary fibrosis (IPF) • Patients between 50 and 70 years of age • Poor prognosis, mean survival 4 years • Histologic pattern of UIP: also seen in association with connective tissue disease

22. HRCT in Diffuse Interstitial Lung Disease Case 528 year-old male. Heavy smoker

23. HRCT in Diffuse Interstitial Lung Disease Case 5 Heavy smoker. Cysts in the lungs

24. HRCT in Diffuse Interstitial Lung Disease Case 5 Heavy smoker. Cysts in the lungs Pattern of abnormality & distribution: • Small nodules + cysts + emphysema • Bilateral & symmetric. Bases spared Differential diagnosis: • Langerhans cell histiocytosis • LIP • Subacute HP Langerhan´s cell histiocytosis

25. HRCT in Diffuse Interstitial Lung Disease Langerhans cell histiocytosis Granulomatous process along bronchioles Proliferation of Langerhans cells Initially 1 - 10 mm nodules Destruction of bronchiolar wall Bronchiolar dilatation & cyst formation Abbott GF et al . RadioGraphics 2004; 24: 821

26. HRCT in Diffuse Interstitial Lung Disease Case 638 year-old female. Reddish skin lesions in the legs and dyspnea

27. HRCT in Diffuse Interstitial Lung Disease Case 6 Skin lesions and dyspnea

28. HRCT in Diffuse Interstitial Lung Disease Case 6 Skin lesions and dyspnea Pattern of abnormality & distribution: • Multiple ill-defined & well defined • Bilateral. Perilymphatic. Upper lobes Differential diagnosis: • Sarcoidosis • Metastases • Vasculitis Sarcoidosis

29. HRCT in Diffuse Interstitial Lung Disease Sarcoidosis • Incidence of 2 - 10 per 100.000 • Equal incidence in white men and women • 17 times more common in blacks • Onset at 20 to 40 years; second peak in the sixth decade • Chest is affected in 90% of cases • Skin (erythema nodosum & lupus pernio) is affected in up to 70% of patients

30. HRCT in Diffuse Interstitial Lung Disease Case 767 year-old female with dry eyes and mouth since 5 years. Nonproductive cough and mild dyspnea

31. HRCT in Diffuse Interstitial Lung Disease Case 7 Dry eyes and mouth since 5 years

32. HRCT in Diffuse Interstitial Lung Disease Case 7 Dry eyes and mouth since 5 years Pattern of abnormality & distribution: • GGO & bilateral well defined cysts • Random Differential diagnosis: • LAM • LIP • Subacute HP Lymphocytic interstitial pneumonia

33. HRCT in Diffuse Interstitial Lung Disease Lymphocytic interstitial pneumonia (LIP) • Slow onset with gradually increasing cough and dyspnea • Associated with CVD and AIDS • Common in Sjogren’s syndrome • Lymphadenopathy

34. HRCT in Diffuse Interstitial Lung Disease Case 848 year-old female with articular disease and pulmonary involvement. Cough and dyspnea

35. HRCT in Diffuse Interstitial Lung Disease Case 8Articular disease with pulmonary involvement

36. HRCT in Diffuse Interstitial Lung Disease Case 8Articular disease with pulmonary involvement

37. HRCT in Diffuse Interstitial Lung Disease Case 8Articular disease with pulmonary involvement Pattern of abnormality & distribution: • Reticular pattern with honeycombing • Bilateral. Symmetric Differential diagnosis: • IPF/UIP • UIP-pattern associated CTD • UIP-pattern in chronic HP UIP- pattern in scleroderma

38. HRCT in Diffuse Interstitial Lung Disease Progressive systemic sclerosis. UIP-pattern • Uncommon systemic disorder characterized by inflammation, fibrosis, and vascular obliteration of the skin and visceral organs Thoracic abnormalities: • Lung fibrosis (20 - 65%) • Esophageal dilatation • Mediastinal lymphadenopathy • Pulmonary hypertension Kim EA et al. J Comput Assist Tomogr, 2001

39. HRCT in Diffuse Interstitial Lung Disease Case 956 year-old female with cough, fever, and dyspnea

40. HRCT in Diffuse Interstitial Lung Disease Case 9 Cough, fever, and dyspnea

41. HRCT in Diffuse Interstitial Lung Disease Case 9 Cough, fever, and dyspnea Pattern of abnormality & distribution: • GGO with lobular areas of air-trapping (“Head-cheese” sign) • Bilateral. Patchy Differential diagnosis: • PCP • RB-ILD • Hypersensitivity pneumonitis Hypersensitivity pneumonitis

42. HRCT in Diffuse Interstitial Lung Disease Hypersensitivity pneumonitis • Subacute onset of respiratory symptoms • Exposure to a range of organic antigens • Cigarette smoke appears to protect againts its development • Restrictive, obstructive, or mixed pulmonary function abnormalities with abnormal gas exchange • Chronic form is usually underdiagnosed

43. HRCT in Diffuse Interstitial Lung Disease Case 1066 years-old female undergoing treatment for rheumatological disease. Mild shortness of breath and a dry cough.

44. HRCT in Diffuse Interstitial Lung Disease Case 10 Complicated lung disease

45. HRCT in Diffuse Interstitial Lung Disease Case 10 Complicated lung disease Pattern of abnormality & distribution: • Consolidation • Bilateral. Along bronchovascular bundles Differential diagnosis: • Bronchopneumonia • Organizing pneumonia pattern • Pulmonary lymphoma Organizing pneumonia pattern (Gold salts)

46. HRCT in Diffuse Interstitial Lung Disease Organizing pneumonia pattern (Drug toxicity) • Dyspnea with or without dry cough • Few weeks or months after initiating therapy • Peripheral or peribronchiolar distribution • High likelihood of recovery after drug cessation and/or corticosteroid therapy www.pneumotox.com

47. HRCT in Diffuse Interstitial Lung Disease Case 1148 year-old female with a subacute history of cough and mild dyspnea

48. HRCT in Diffuse Interstitial Lung Disease Case 11 Subacute history of cough and mild dyspnea

49. HRCT in Diffuse Interstitial Lung Disease Case 11 Subacute history of cough and mild dyspnea Pattern of abnormality & distribution: • GGO. Some consolidation & “crazy paving” • Bilateral. Subpleural Differential diagnosis: • Organizing pneumonia pattern • Nonspecific interstitial pneumonia • Desquamative interstitial pneumonia / RB-ILD Non-specific interstitial pneumonia

50. HRCT in Diffuse Interstitial Lung Disease Non-specific Interstitial Pneumonia (NSIP) • Many cases idiopathic • Associated with drug reaction, CVD, or HP • Symptoms similar to IPF; dyspnea and cough with average duration of 8 months • Occur in adults; average age, approximately 50 years