Disclosures. Traditional Paradigm. Overview 4/17/2010. I have relationships with the following organizations and companies:
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1 Disclosures Pharmacological Therapy for ILD What to Use and How to Use It Harold R Collard MD Interstitial Lung Disease Program University of California San Francisco (UCSF) I have relationships with the following organizations and companies: Research Funding: National Institute of Health University of California Advising: Coalition for Pulmonary Fibrosis Scientific Consulting: Actelion Amira Pharmaceuticals Gilead Science Overview This talk is about pharmacological therapy in interstitial lung disease (ILD) Traditional Paradigm Injury inflammation fibrosis I want you to understand: When to treat What to use How to use it Repetitive injury leads to the development of widespread inflammation and/or fibrosis 1
2 Applies to most ILDs Applies to most ILDs Interstitial Lung Disease Interstitial Lung Disease Exposure-related: - Occupational - Environmental - Avocational - Medication Idiopathic interstitial pneumonia (IIP) Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Idiopathic pulmonary fibrosis (IPF) Connective tissue disease: - Scleroderma - Rheum. arth - Sjogrens Respiratory bronchiolitis interstitial lung dis. (RBILD) Cryptogenic organizing pneumonia (COP) Sarcoidosis Other: - Vasculitis/Diffuse alveolar hemorrhage (DAH) - Langherhans cell histiocytosis (LCH) - Eosinophilic pneumonias - Neurofibromatosis - Inherited disorders - Chronic aspiration - Inflammatory bowel disease Exposure-related: - Occupational - Environmental - Avocational - Medication Idiopathic interstitial pneumonia (IIP) Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Idiopathic pulmonary fibrosis (IPF) Connective tissue disease: - Scleroderma - Rheum. arth - Sjogrens Respiratory bronchiolitis interstitial lung dis. (RBILD) Cryptogenic organizing pneumonia (COP) Sarcoidosis Other: - Vasculitis/Diffuse alveolar hemorrhage (DAH) - Langherhans cell histiocytosis (LCH) - Eosinophilic pneumonias - Neurofibromatosis - Inherited disorders - Chronic aspiration - Inflammatory bowel disease Nonspecific interstitial pneumonia (NSIP) Lymphocytic interstitial pneumonia (LIP) Nonspecific interstitial pneumonia (NSIP) Lymphocytic interstitial pneumonia (LIP) ILD Treatment: Non-IPF ILD that is likely to resolve (sarcoid, COP, cellular HP, DIP, LCH) Prednisone ILD that is unlikely to resolve (CTILD, fibrotic NSIP, fibrotic HP) Prednisone and/or immunomodulatory therapy (azathioprine, cyclophosphamide, mycophenolate) Image courtesy of Kirk Jones, MD 2
3 When to treat When to treat Side effects Adverse reactions Cost BENEFIT RISK When to treat Dosing of therapy Prednisone: 0.5 mg/kg/day (less in sarcoidosis) Azathioprine: 2 mg/kg/day Cyclophosphamide: 2 mg/kg/day (oral) Mycophenolate: 2 grams/day 3
4 Timing of dual agent therapy What about IPF? Labs: Q 2wk Q mo Interstitial Lung Disease Prednisone dose (mg/kg ) None Full Half None Second agent ( ) Exposure-related: - Occupational - Environmental - Avocational - Medication Idiopathic interstitial pneumonia (IIP) Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Idiopathic pulmonary fibrosis (IPF) Connective tissue disease: - Scleroderma - Rheum. arth - Sjogrens Respiratory bronchiolitis interstitial lung dis. (RBILD) Cryptogenic organizing pneumonia (COP) Sarcoidosis Other: - Vasculitis/Diffuse alveolar hemorrhage (DAH) - Langherhans cell histiocytosis (LCH) - Eosinophilic pneumonias - Neurofibromatosis - Inherited disorders - Chronic aspiration - Inflammatory bowel disease Start 1mo 2mo 3mo 4mo 5mo 6mo Nonspecific interstitial pneumonia (NSIP) Lymphocytic interstitial pneumonia (LIP) Treatment is inconsistent Traditional Therapy Percent of respondents Corticosteroid Prednisone (0.5 mg/kg/d, tapered over 2-3 months) + Immunomodulator Azathioprine (2 mg/kg/d) Cyclophosphamide (2 mg/kg/d) Mycophenolate (2 grams/d) Collard Resp Med 2007;101:2011 4
5 Traditional therapy Treatment Relative risk No therapy % CI p value Prednisone to Prednisone plus immunomodulator to Douglas AJRCCM 2000;161:1172 Traditional therapy Some patients still respond P = 0.58 Patients treated with prednisone Response defined as 10 point change in CRP score Response (26%) (82) (82) Stable (37%) No response (37%)? Collard et al. Chest 2004;125:2169 Gay AJRCCM 1998;157:1063 5
6 Data are Lacking For patients with a definite diagnosis of IPF there is no evidence that coticosteroid therapy plays a role in modifying the course of disease. Interferon gamma 1b Acetylcysteine Pirfenidone Novel therapies? No controlled clinical trials in this area Richeldi Cochrane Review 2006 Biological rationale Interferon gamma 1b INFγ limits fibroblast proliferation (Pfeffer et al. Exp Cell Res 1979;121:111) INFγ limits TGFβ-induced collagen production (Eikelberg et al. FASEB J 2001;15:797) IPF lung appears to have decreased INFγ (Majumdar et al. Eur Resp J 1999;14:251) 6
7 Progression-free survival 330 patients INFγ vs. Placebo 1 endpoint: progression free survival (death, drop in FVC, DLCO, or A-a gradient) Raghu NEJM 2004;350:125 Raghu NEJM 2004;350:125 Overall survival INSPIRE 826 pts preserved FVC INFγ vs. placebo 1 endpoint: survival 115 events DISCONTINUED by data safety board for lack of benefit (i.e. reduction in risk of death of 33% or greater) Raghu NEJM 2004;350:125 King Lancet 2009;374:222 7
8 Biological rationale Acetylcysteine Control NAC Treatment with NAC ameliorates bleomycin-induced pulmonary fibrosis in rats. Cortijo et al. Eur Respir L 2001;17:1228 Biological rationale Patients with IPF have decreased levels of glutathione in sputum and BAL fluid 155 subjects Acetylcysteine +/- prednisone/azathioprine 1 endpoint: change in vital capacity at 12 months Beeh et al. Eur Respir J 2002;19:1119 Demedts NEJM 2005;353:2229 8
9 Results Results P = 0.02 P = 0.02 Azuma 2005 Raghu 2004 SHIONOGI Raghu 2008 King 2008 CAPACITY 1 CAPACITY 2 Demedts NEJM 2005;353:2229 Demedts NEJM 2005;353:2229 Biological rationale Pirfenidone Iyer et al. J Pharmacol Exp Ther 1999;291:367 9
10 Shionogi Study Phase 3 double blind randomized controlled 275 subjects with IPF randomized 2:1:2 (high dose, low dose, placebo) Age Desaturation 5% with walk test Lowest SpO2 85% on room air Primary outcome: Change in vital capacity at 52 weeks Variable Shionogi Study Pirfenidone, high dose (n = 108) Pirfenidone, low dose (n = 55) Placebo (n = 104) Age Male gender 79% 86% 78% Smoking history 80% 78% 80% Steroid use 8% 11% 6% Lung biopsy 24% 29% 27% VC % predicted DLCO % predicted Desaturation on walk test 32% 35% 23% Taniguchi ERJ 2010;35:821 Taniguchi ERJ 2010;35:821 Shionogi Study Shionogi Study Taniguchi ERJ 2010;35:821 Taniguchi ERJ 2010;35:821 10
11 Limitations Primary endpoint changed Initially lowest oxygen saturation during walk test Done after data reviewed Limitations Primary endpoint changed Initially lowest oxygen saturation during walk test Done after data reviewed Substantial missing data 30-40% across groups Statistical methodology problematic Collard ERJ 2010;35:728 Collard ERJ 2010;35:728 Limitations Limitations = crude mean change Primary endpoint changed Initially lowest oxygen saturation during walk test Done after data reviewed Substantial missing data 30-40% across groups Statistical methodology problematic No patient centered outcome data Increased photosensitivity (>50%), elevated GGT Morbidity could negate marginal physiological benefit Taniguchi ERJ 2010;35:821 Collard ERJ 2010;35:728 11
12 Capacity Studies Phase 3 double blind randomized controlled PIPF006: 344 subjects randomized 1:1 (high dose, placebo) PIPF004: 435 subjects randomized 2:1:2 (high dose, low dose, placebo) Primary outcome: Change in forced vital capacity at 72 weeks Capacity Studies Inclusion criteria Age Diagnosis of IPF within 48 months FVC > 50%, DLCO > 35% Exclusion criteria Significant COPD FDA Briefing Booklet ( CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM pdf) FDA Briefing Booklet ( CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM pdf) Capacity Studies Capacity Studies Change in FVC % P = = Change in FVC % Azuma, Raghu, Raghu, 2008 King, 2008 P = = = 004 = 004 P < P < FDA Briefing Booklet ( CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM pdf) FDA Briefing Booklet ( CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM pdf) 12
13 Capacity Studies Capacity Studies Relative reduction in FVC decline (%) = 006 = 004 PIPF006 PIPF004 Secondary Endpoints Time to worsening IPF Progression-free survival MWT distance change Dyspnea (UCSD SOBQ) change Survival time CAPACITY ATS Presentation, May 20 th, 2009 CAPACITY ATS Presentation, May 20 th, 2009 Pirfenidone Conclusions Do the data provide substantial evidence that pirfenidone provides a clinically meaningful beneficial effect? Pirfenidone Conclusions Do the data provide substantial evidence that pirfenidone provides a clinically meaningful beneficial effect? Has the safety of pirfenidone been adequately assessed? 13
14 Pirfenidone Conclusions Would you recommend approval of pirfenidone for the treatment of patients with IPF? Treatment of ILD: Algorithm Patient with ILD General Management (What criteria should be used for deciding this?) Manage comorbidities Pulmonary rehabilitation Oxygen therapy Lung Transplantation IPF? NO Prednisone +/- immunomodulator YES Enroll in a clinical trial if eligible No therapy NAC (+/-Pred/Aza) Pirfenidone Thank you! UCSF ILD Program 400 Parnassus Avenue, 5 th Floor Chest Practice (phone) 14
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