Non-neoplastic Lung Disease II

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1 Pathobasic Non-neoplastic Lung Disease II Spasenija Savic Prince Pathology

2 Program Systematic approach to surgical lung biopsies with ILD Examples (chronic ILD): Idiopathic interstitial pneumonias: UIP, NSIP

3 Interstitial Lung Disease Known cause/ association Idiopathic Enviroement (HP, ) Drugs Infections Sarcoidosis Collagen vasc. dis. IPF, NSIP RB-ILD, DIP COP, AIP LIP, PPFE Spec. pathology: Eos. Pneumonia LCH, LAM, PAP ATS/ERS, Am J Respir Crit Care Med., 2013/2002

4 Onset Clinical history acute, subacute, chronic Systemic disease, drugs Occupation, exposure Immune status

5 Radiology CT= Gross pathology of ILD Distribution Localized/unilateral vs bilateral patchy or diffuse Subpleural/paraseptal, bronchiolocentric, lymphatic, random Radiologic pattern Ground glass consolidation, reticulation, nodules, mosaic patterns Extend of fibrosis (end stage honeycombing)

6 Multidisciplinary Discussion Clinical context + CT pattern BAL FACS non diagnostic TBB Cryo Bronchoskopy: BAL, biopsy UHB: 20% non diagnostic 1mm Surgical lung biopsy Diagnosis, Treatment adapted from Raj R, Chest, 2016

7 Histology: Systematic Approach I Low magnification (pattern recognition)! 1. Distribution patchy or diffuse Subpleural/paraseptal Bronchiolocentric Lymphatic random 2. Main site of involvement Alveolar septal, airspace, mixed

8 Histology: Systematic Approach I 3. Inflammation Intensity: mild, moderate, intense Macrophages: Distribution Presence of Granulomas 4. Age of injury Acute (hyaline membranes) Subacute (intraalveolar/ interstitial fibromyxoid tissue) Chronic (dense scars) Temporally homogenous vs. heterogenous

9 Normal NSIP Pattern Bronchiolocentr. Pattern UIP Pattern Coll. vasc. dis. Idiop. NSIP Chron. HP Drugs HP RB-ILD Coll. vasc. dis. Drugs IPF Chron. HP Coll. vasc. dis. Drugs Smith ML, Arch Pathol Lab Med, 2016; ATS/ERS, Am J Respir Crit Care Med., 2013/2002

10 Normal NSIP Pattern Bronchiolocentr. Pattern UIP Pattern Coll. vasc. Dis. Idiop. NSIP Chron. HP Drugs HP RB-ILD Coll. vasc. Dis. Drugs IPF Chron. HP Coll. vasc. Dis. Drugs Same histological pattern in different diseases Smith ML, Arch Pathol Lab Med, 2016; ATS/ERS, Am J Respir Crit Care Med., 2013/2002

11 Additional Clues Organizing pneumonia pattern Granulomas Inflammation Dust

12 Algorithm for Idiopathic Interstitial Pneumonias SA Yousem, USCAP 2005

13 ATS/ERS, Am J Respir Crit Care Med., 2013/2002

14 Idiopathic Pulmonary Fibrosis (IPF) Occuring primarily in older smokers (M>F, 60-70) Worst prognosis of all idiopathic IP 50% DOD in 3 years Contraindication for stereoids Treatment with antifibrotic drugs Pirfenidone or Nintedanib

15 IPF definition: Chronic, progressive fibrosing IP of unknown cause Limited to the lungs Assoc. with histopathologic and/or radiologic pattern of UIP

16 CT: Honeycombing

17

18

19

20 UIP Pattern 1. Marked fibrosis with architectural distortion Subpleural/paraseptal +/- honeycombing 2. Patchy fibrosis 3. Fibroblastic foci 4. Absence of marked inflammation, granulomas

21 UIP Pattern 1. Marked fibrosis with architectural distortion Subpleural, +/- honeycombing 2. Patchy fibrosis 3. Fibroblastic foci 4. Absence of marked inflammation, granulomas Fibroblastic focus: Edematous fibroblastic tissue Convex in shape Adjacent to the region of more chronic fibrosis

22 UIP Pattern 1. Marked fibrosis with architectural distortion Subpleural, +/- honeycombing 2. Patchy fibrosis 3. Fibroblastic foci 4. Absence of marked inflammation, granulomas Fibroblastic focus: Edematous fibroblastic tissue Convex in shape Adjacent to the region of more chronic fibrosis Alcian blue PAS Fibrosis fibroblastic foci preserved lung tissue = Temporal heterogeneity

23

24

25 Idiopathic Non-Specific Interstitial Pneumonia F>M, 50, non-smokers Acute to subacute onset CT Bilateral reticular or ground glass infiltrates Rare honeycomb fibrosis Respond to steroids (cellular phase) Prognosis very good

26 Histology: NSIP Pattern Diffuse; alveolar septal Temporally homogenous Cellular: interstitial inflammation prominent, minimal fibrosis Fibrosing: Fibrosis (collagen) prominent, mild inflammation Architecture preserved Fibroblastic foci rare No granulomas

27 NSIP: cellular NSIP: fibrosing

28 NSIP Pattern: DD Collagen vascular disease Hypersensitivity pneumonitis Drug reactions Infection (viral) Areas with NSIP-pattern common in IPF compare to CT results

29 Granulomatous ILD Granulomas not a component of idiopathic interstitial pneumonias (except LIP) Consider: Hypersensitivity pneumonitis Sarcoidosis Infection

30 COMING NEXT THE ILD PATHOLOGY QUIZ

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