Annual Rheumatology & Therapeutics Review for Organizations & Societies

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1 Annual Rheumatology & Therapeutics Review for Organizations & Societies

2 A Rheumatologist s Approach to Interstitial Lung Disease

3 Outline ILD classification and patterns in CTD The clinical landscape and evaluation Management of CTD-ILD

4 Why Focus on ILD? Occurs commonly across the entire spectrum of CTD A multidisciplinary approach has been demonstrated to be useful Potentially the most devastating of pulmonary manifestations A driver of significant morbidity / mortality in CTD Often poses the most significant challenges to the practicing clinician

5 CTD-ILD is Not a Single Disease CTD ILD RA SLE SjS PM/DM SSc MCTD UCTD CTD ILD UIP NSIP OP LIP AIP

6 Usual Interstitial Pneumonia (UIP) Idiopathic UIP = IPF Honeycombing, fibrosis Likely the most common pattern in RA 2nd most common pattern in SSc Can see with HP, sarcoid, drug toxicity Least responsive to immunomodulatory therapy Poor prognosis Am. J. Respir. Crit. Care Med. 2002;165:

7 Non-Specific Interstitial Pneumonia (NSIP) Cellular vs. fibrotic vs. mixed Ground glass Minimal honeycombing Strong suspicions for CTD Also seen with HP, infections, drug toxicity Survival in idiopathic interstitial pneumonia: C-NSIP > F-NSIP > UIP Am. J. Respir. Crit. Care Med. 2002;165:

8 Lymphocytic Interstitial Pneumonia (LIP) Cystic lung disease Seen with Sjögren's RA SLE Differential Dx: Primarily LAM (Lymphangioleiomyomatosis) Other rare diseases

9 Other Patterns Acute interstitial pneumonia / diffuse alveolar damage infections CTDs Respiratory bronchiolitis-ild Smoking related lung disease Not CTD-associated Desquamative interstitial pneumonia Smoker s mostly Rarely with CTD

10 Prevalence Rates? Published estimates vary: SSc: 25-90% PM/DM: 5-70% RA: 2-63% SjS: 8-57% SLE: 3-38% Subclinical ILD in ~ 50%? Frankel & Brown Clinical Pulmonary Medicine 2006 Doyle et al AJRCCM 2012

11 Outline ILD classification and patterns in CTD The clinical landscape and evaluation Management of CTD-ILD

12 The Clinical Landscape Established CTD Determine whether ILD is CTD-associated Idiopathic ILD: Identifying occult CTD Idiopathic ILD: Suggestive forms of CTD-ILD

13 55-year-old man with RA, former smoker, develops exertional dyspnea and cough 10-year history of RF / CCP positive, erosive RA arthritis well controlled adalimumab, methotrexate, NSAID resting pox = 91% crackles at B/L bases chronic RA deformities without synovitis FVC 74%, FEV-1 73%, DLCO 64% Normal CBC, CMP, ESR 15

14 RA patients can have birds too A thorough evaluation is needed Exclude INFECTION Exclude drug toxicity (MTX) Consider alternative etiologies Concluding ILD is CTDassociated is a process of elimination

15 Role of Bronchoscopy? Infiltrates in immunocompromised host = infection until proven otherwise BAL is often needed to assess for: infection alveolar hemorrhage Recent data argue against use of BAL to predict outcome in SSc-ILD BAL neutrophilia, eosinophilia is associated with more severe disease Kowal-Bielecka et al Semin Arthritis Rheum 2010

16 Role of Surgical Lung Biopsy? Clinical realities: The biopsy finding may not impact on treatment decisions CTD-ILD patients tend to be treated with immunosuppressive therapies for ILD and the extra-thoracic disease irrespective of ILD pattern

17 Percentage survival Histopathology Matters for Idiopathic Interstitial Pneumonia 100 DIP/RBILD/Cellular NSIP Cellularity Fibrotic NSIP UIP Fibrosis Time (months) Nicholson et al. Am J Respir Crit Care Med 2000;162:

18 Percent Survival Histopathology in CTD-ILD Did NOT Impact Survival UIP CVD-UIP I-NSIP CVD-NSIP SSc: 37 RA: 28 SjS: 11 PM/DM: 8 MCTD: 5 Other: 4 0 Park et al, AJRCCM Follow up period (months)

19 Prognostic Factors for the Survival of Patients with Interstitial Pneumonia Associated with Collagen Vascular Disease Using a Univariate Cox Model n Hazard Ratio 95% Cl p Value Age, yr Male Sex NS Smokers vs. nonsmokers NS Dyspnea score CVD-UIP vs. CVD-NSIP NS RA vs. other CVDs NS Scleroderma vs. other CVDs NS FVC, %, predicted Dl co, %, predicted NS TLC, %, predicted NS PA 02 /Fi 02 ratio, mm Hg NS Park et al, AJRCCM 2007

20 Percentage survival In SSc-ILD, Physiology Drives Mortality subjects with biopsied SSc-ILD: 78% NSIP, 15% UIP DLco + DLco -/ DLco - DLco -/+ Bouros et al. AJCCRM Time (months)

21 Survival (%) SSc-ILD: Disease Extent, Severity Impact Prognosis (ie, NOT pathologic pattern) HRCT extent % Indeterminate > 30% 75 Limited FVC 70% FVC < 70% Limited Disease Extensive Disease Extensive HR=3.46; p< Duration of follow-up (months) Goh et al. AJRCCM 2008; 177:

22 Indications for Surgical Lung Biopsy? pre-existing CTD and concerns for an alternative etiology atypical HRCT idiopathic ILD and thinking it may be CTD poorly defined CTD ultimately, the decision is individualized

23 The Clinical Landscape Established CTD Determine whether ILD is CTD-associated Idiopathic ILD: Identifying occult CTD Idiopathic ILD: Suggestive forms of CTD-ILD

24 49 year old woman with acute onset of exertional dyspnea and cough No medications Never smoker No occupational or environmental exposures Rheumatologic ROS: Raynaud s phenomenon Exam: Puffy hands / wrists, bi-basilar crackles

25 CBC normal ESR 27 CPK 189 Negative: ANA SS-B, Smith, RNP, dsdna RF, CCP ANCA panel SS-A moderately positive TLC 62% FVC 45% DLco 25% Walk-Ox: 6 L O 2 VATS: NSIP Overlapping OP Increased perivascular collagen

26 environmental occupational medications infection??? familial??? smoking?? CTD-ILD idiopathic

27 Percent Survival Why Assess for CTD? A diagnosis of CTD-ILD may impact: 100 Treatment Prognosis Extra-thoracic disease clinical context surveillance for other features p<0.001 IIP CVD-IP Follow up period (months) Park et al. AJRCCM 2007;175:

28 Other common and potentially important reasons to assess for CTD Patient perspective: Physician perspective: emotional sense of belonging frustrations with being labeled as idiopathic if it s CTD, I can do something about it the last thing I want to tell my patients is that it s idiopathic

29 ILD as the Presenting Manifestation of CTD multi-disciplinary collaboration helps ILD may be the first - or ONLY - manifestation of underlying CTD extrathoracic manifestations of underlying CTD may be subtle

30 Identifying New CTD in Those Presenting with ILD is Common 114 consecutive patients evaluated in an ILD referral center 34 (30%) with well-defined CTD 17 (15%) with well established CTD prior to ILD 17 (15%) diagnosed with new CTD Mittoo S et al. Resp Med :1152

31 What s Helpful? demographics 40 year old women don t get IPF extrathoracic manifestations serologies HRCT findings which ones? histopathology

32 Quantifiable, Specific Extra-thoracic Features Suggesting CTD sclerodactyly Raynaud s phenomenon capillary microscopy keratoconjuctivitis sicca Mechanic hands Digital edema Gottron s papules inflammatory arthritis of bilateral wrists or MCPs esophageal dilation / hypomotility tortuosity dilatation dropout

33 Useful Autoantibodies for CTD-ILD Most common CTD association High-titer ANA (>1:320) RF (>60 IU/mL) Anti-centromere Nucleolar-ANA Anti-CCP Anti-Scl-70 Anti-Ro Anti-La Anti-dsDNA Anti-RNP Anti-Smith Anti-tRNA synthetase (Jo-1, PL-7, PL-12, others) Anti-PM-Scl Many Many / RA SSc SSc RA SSc Many Sjögren's s, SLE SLE MCTD, SLE / SSc SLE PM / DM SSc / PM overlap

34 HRCT Clues for CTD-ILD multi-compartment involvement dilated esophagus pericardial thickening or effusion bilateral, bibasilar, and peripheral-predominant NSIP Hwang et al J Comput Assist Tomogr 33, 410-5

35 Histopathology Features of CTD-ILD Secondary histopathologic features: dense perivascular collagen extensive pleuritis lymphoid aggregates with germinal center formation prominent plasmacytic infiltration Multi-compartment involvement parenchyma, airways, vascular, pleura NSIP, UIP, OP, LIP, AIP/DAD Leslie et al Semin Respir Crit Care Med 2007;28(4):369

36 This is What CTD-ILD Looks Like

37 Screening for CTD-ILD with an ANA, ANA Profile, RF, CCP, and Scl-70, Misses the anti-synthetase Syndrome And many of these patients do not have myositis.

38 The Clinical Landscape Established CTD Determine whether ILD is CTD-associated Idiopathic ILD: Identifying occult CTD Idiopathic ILD: Suggestive forms of CTD-ILD

39 CTD-ILD? 40 year-old woman with nothing extrathoracic ANA positive 1:320 speckled biopsy-proven NSIP overlapping features: organizing pneumonia lymphoid follicles with germinal centers

40 55 year old man with UIP: RF and CCP Both High-positive; No Arthritis RA-ILD? IPF?

41 Limitations of Rheumatologic Classification Criteria Without extra-thoracic manifestations, rheumatologists are reluctant to label CTD even ILD with highly-specific autoantibodies ILD is not included in the classification criteria for any of the CTDs, except as a minor criterion for SSc

42 Interstitial Pneumonia with Autoimmune Features (IPAF)

43 Outline ILD classification and patterns in CTD The clinical landscape and evaluation Management of CTD-ILD

44 Management Aspects: CTD-ILD is not a Single Disease CTD ILD RA SLE SjS PM/DM SSc MCTD UCTD CTD ILD UIP NSIP OP LIP AIP

45 Consider Underlying Histologic Pattern? RA-OP Are these all treated the same? RA-LIP RA-C-NSIP RA-F-NSIP RA-UIP

46 Whom to Treat? Depends on: impairment (subjective and objective) pace of disease other factors (age, co-morbid conditions) Treat: clinically-significant, progressive disease

47 Determining impairment Subjective How to assess dyspnea? Standardized questionnaires? Objective PFTs Walk-oximetry 6MWT Disease extent by HRCT

48 RA-ILD: What s Driving Therapy? disease activity disease activity time disease activity disease activity time RA = ORANGE ILD = BLUE time time

49 Survival, % Cyclophosphamide in SSc-ILD Appears to Impact Physiology and Survival No CYC: FVC -7%, DLCO -9% CYC: FVC +4.3%, DLC0 +1.0% CYC: better survival experience Time, mo White et al Ann Int Med 2000;132:

50 Change from Baseline in FVC Scleroderma Lung Study Cyclophosphamide 49.3% 49% had improvement improved Placebo 26.4% 26% had improvement improved % had worsening 73.6% had worsening 51% worsened 74% worsened Frequency (%) Tashkin et al 2006 NEJM 354;

51 Fibrosing alveolitis in SSc Trial low-dose prednisone, IV CYC x 6 months followed by AZA vs. placebo BASELINE 1-YR FOLLOW-UP Rx (n=22) Placebo (n=23) Rx (n=19) Placebo (n=18) FVC DLCO TLC P Hoyles et al. Arthritis Rheum 2006

52 MMF Improves Lung Function in CTD-ILD median daily prednisone dose: at MMF initiation = 20 mg Pred dose through time among subjects with SSc, PM/DM, RA or LD-CTD after 9-12 months on MMF = 5 mg (p<0.0001) Predicted Mean Mean prednisone dose (mg) weeks weeks before and after MMF initiation CTDdx Red line=ra, Black line=ssc, Blue line=pm/dm, Green line=lung dominant-ctd Fischer et al. J Rheumatol 2013

53 FVC% Change in FVC over time -156 to 0 weeks -104 to 0 weeks -52 to 0 weeks 0 to 52 weeks 0 to 104 weeks 0 to 156 weeks FVC% -2.3 ± 5.0 p= ± 3.3 p= ± 1.7 p=0.6 Figure 2A. Plot of mixed-effects model estimates for FVC% over time 4.9 ± 1.9 p= ± 1.8 p= ± weeks before and after MMF initiation Fischer et al. J Rheumatol 2013 MMF start Weeks before and after MMF initiation

54 MMF in CTD-ILD In a diverse and large cohort of CTD-ILD, we observed that MMF was well tolerated had a low rate of discontinuation was associated with effective corticosteroid tapering was associated with stabilization or improvement in FVC and/or DLco MMF warrants prospective study MMF may replace CYC as 1 st line therapy for CTD-ILD Fischer et al. J Rheumatol 2013

55 Other Options? Azathioprine well tolerated familiar FAST trial in SSc case series suggest role for variety of CTD-ILD Cyclosporine, Tacrolimus may be particularly effective in patients with myositis ILD Rituximab Refractory myositis-ild

56 Gauging Response Subjective symptoms tolerance FVC DLCO 6MWT HRCT best of 5 wins

57 Non-drug Therapy pulmonary rehab use O2 correctly PH assessments GERD N-acetylcysteine (NAC)? Pneumocystis prophylaxis vaccines mental health

58 Summary All of the IIP patterns are seen in CTD (except RB-ILD, DIP) NSIP is the most common pattern in CTD UIP is the most common pattern in RA ILD in pre-existing CTD Exclude alternative etiologies Biopsy the atypical HRCT / atypical scenario CTD-ILD = diagnosis of exclusion Predictors of mortality in SSc-ILD: physiology and extent of ILD ILD as the first manifestation of CTD Multidisciplinary evaluations are useful Consider demographics, serologies, clinical features, radiology, pathology Controversies surrounding suggestive forms of CTD-ILD

59 Summary Not every patient with CTD-ILD needs treatment Consider what s driving need for treatment Extra-thoracic vs. intra-thoracic disease activity Determine degree of impairment, pace of the disease Treat only those with clinically-significant, progressive ILD Management is not evidence-based Consider underlying CTD and ILD pattern MMF use is popular in Colorado warrants prospective study Desperate need for better therapies

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