Table of Contents Table of Contents 2 List of Tables 7 List of Figures 8 Introduction 9 Global Markets Direct Report Coverage 9 Systemic Sclerosis (Scleroderma) Overview 10 Therapeutics Development 11 Pipeline Products for Systemic Sclerosis (Scleroderma) - Overview 11 Pipeline Products for Systemic Sclerosis (Scleroderma) - Comparative Analysis 12 Systemic Sclerosis (Scleroderma) - Therapeutics under Development by Companies 13 Systemic Sclerosis (Scleroderma) - Therapeutics under Investigation by Universities/Institutes 15 Systemic Sclerosis (Scleroderma) - Pipeline Products Glance 16 Late Stage Products 16 Clinical Stage Products 17 Early Stage Products 18 Systemic Sclerosis (Scleroderma) - Products under Development by Companies 19 Systemic Sclerosis (Scleroderma) - Products under Investigation by Universities/Institutes 21 Systemic Sclerosis (Scleroderma) - Companies Involved in Therapeutics Development 22 Allergan Plc 22 Angion Biomedica Corp. 23 arGentis Pharmaceuticals, LLC 24 Bayer AG 25 BioLineRx, Ltd. 26 BiOrion Technologies B.V. 27 Boehringer Ingelheim GmbH 28 Bristol-Myers Squibb Company 29 Corbus pharmaceuticals, Inc. 30 CSL Limited 31 Daval International Limited 32 Digna Biotech, S.L. 33 F. Hoffmann-La Roche Ltd. 34 Fibrocell Science, Inc. 35 GenKyoTex S.A. 36 GlaxoSmithKline Plc 37 iBio, Inc. 38 Inventiva SAS 39 Kadmon Corporation, LLC 40 miRagen Therapeutics, Inc. 41 Peptinov SAS 42 Ribomic Inc. 43 Stratatech Corporation 44 Vida Therapeutics Inc. 45 VivaCell Biotechnology Espana S.L. 46 Systemic Sclerosis (Scleroderma) - Therapeutics Assessment 47 Assessment by Monotherapy Products 47 Assessment by Target 48 Assessment by Mechanism of Action 51 Assessment by Route of Administration 54 Assessment by Molecule Type 56 Drug Profiles 58 abatacept - Drug Profile 58 Product Description 58 Mechanism of Action 58 R&D Progress 58 acALY-18 - Drug Profile 61 Product Description 61 Mechanism of Action 61 R&D Progress 61 acetylcysteine - Drug Profile 62 Product Description 62 Mechanism of Action 62 R&D Progress 62 Aimspro - Drug Profile 63 Product Description 63 Mechanism of Action 63 R&D Progress 63 ANG-3070 - Drug Profile 65 Product Description 65 Mechanism of Action 65 R&D Progress 65 ARG-201 - Drug Profile 66 Product Description 66 Mechanism of Action 66 R&D Progress 66 belimumab - Drug Profile 68 Product Description 68 Mechanism of Action 68 R&D Progress 68 BL-1110 - Drug Profile 72 Product Description 72 Mechanism of Action 72 R&D Progress 72 BOT-191 - Drug Profile 74 Product Description 74 Mechanism of Action 74 R&D Progress 74 C-82 - Drug Profile 75 Product Description 75 Mechanism of Action 75 R&D Progress 75 Cell Therapy for Dermatology and Immunology - Drug Profile 76 Product Description 76 Mechanism of Action 76 R&D Progress 76 disitertide - Drug Profile 79 Product Description 79 Mechanism of Action 79 R&D Progress 79 factor XIII (human) - Drug Profile 82 Product Description 82 Mechanism of Action 82 R&D Progress 82 FCX-013 - Drug Profile 83 Product Description 83 Mechanism of Action 83 R&D Progress 83 GKT-831 - Drug Profile 84 Product Description 84 Mechanism of Action 84 R&D Progress 84 GSK-2330811 - Drug Profile 85 Product Description 85 Mechanism of Action 85 R&D Progress 85 IBIOCFB-03 - Drug Profile 86 Product Description 86 Mechanism of Action 86 R&D Progress 86 IVA-337 - Drug Profile 88 Product Description 88 Mechanism of Action 88 R&D Progress 88 JBT-101 - Drug Profile 89 Product Description 89 Mechanism of Action 89 R&D Progress 89 KD-025 - Drug Profile 91 Product Description 91 Mechanism of Action 91 R&D Progress 91 MGN-4220 - Drug Profile 93 Product Description 93 Mechanism of Action 93 R&D Progress 93 nintedanib - Drug Profile 94 Product Description 94 Mechanism of Action 94 R&D Progress 94 Oligonucleotide for Scleroderma - Drug Profile 100 Product Description 100 Mechanism of Action 100 R&D Progress 100 onabotulinumtoxin A - Drug Profile 101 Product Description 101 Mechanism of Action 101 R&D Progress 101 PAT-048 - Drug Profile 104 Product Description 104 Mechanism of Action 104 R&D Progress 104 PDX-002 - Drug Profile 105 Product Description 105 Mechanism of Action 105 R&D Progress 105 PPV-06 - Drug Profile 106 Product Description 106 Mechanism of Action 106 R&D Progress 106 RBM-006 - Drug Profile 107 Product Description 107 Mechanism of Action 107 R&D Progress 107 riociguat - Drug Profile 108 Product Description 108 Mechanism of Action 108 R&D Progress 108 Small Molecule to Inhibit NFkB for Immunology and Oncology - Drug Profile 110 Product Description 110 Mechanism of Action 110 R&D Progress 110 Stem Cell Therapy for Crohns Disease, Systemic Sclerosis and Multiple Sclerosis - Drug Profile 111 Product Description 111 Mechanism of Action 111 R&D Progress 111 Synthetic Peptides to Inhibit TCR for Autoimmune Disorders and Dermatitis - Drug Profile 112 Product Description 112 Mechanism of Action 112 R&D Progress 112 tocilizumab - Drug Profile 113 Product Description 113 Mechanism of Action 113 R&D Progress 113 VCE-0048 - Drug Profile 117 Product Description 117 Mechanism of Action 117 R&D Progress 117 VEDA-1209 - Drug Profile 118 Product Description 118 Mechanism of Action 118 R&D Progress 118 VTI-1000 Series - Drug Profile 119 Product Description 119 Mechanism of Action 119 R&D Progress 119 Systemic Sclerosis (Scleroderma) - Recent Pipeline Updates 120 Systemic Sclerosis (Scleroderma) - Dormant Projects 156 Systemic Sclerosis (Scleroderma) - Discontinued Products 158 Systemic Sclerosis (Scleroderma) - Product Development Milestones 159 Featured News & Press Releases 159 Dec 08, 2015: New Phase III study of nintedanib in people with systemic sclerosis and lung fibrosis 159 Nov 12, 2015: miRagen Therapeutics Initiates First Clinical Trial for Anti-Fibrosis Product Candidate MRG-201 159 Nov 11, 2015: Genkyotex's GKT137831 Granted Orphan Drug Designation in the US and EU for the Treatment of Systemic Sclerosis 160 Oct 13, 2015: Corbus Pharmaceuticals to Present at the 14th Annual BIO Investor Forum 161 Oct 07, 2015: Corbus Pharmaceuticals Announces First Patient Dosed in Phase 2 Study of Resunab for Systemic Sclerosis 161 Aug 31, 2015: Corbus Pharmaceuticals Commences Patient Enrollment in Phase 2 Clinical Study of Resunab for the Treatment of Systemic Sclerosis 161 Aug 19, 2015: Corbus Pharmaceuticals' Investigational Drug Resunab Granted Fast Track Status by the U.S. FDA for the Treatment of Systemic Sclerosis 163 Aug 06, 2015: Corbus Pharmaceuticals to Present at the Canaccord Genuity Growth Conference on August 13, 2015 163 Aug 06, 2015: Data On PDX-002 Published In Biomaterials Journal 164 Jun 12, 2015: Corbus Pharmaceuticals Announces FDA Orphan Drug Designation for Resunab for the Treatment of Systemic Sclerosis (Scleroderma) 164 Appendix 165 Methodology 165 Coverage 165 Secondary Research 165 Primary Research 165 Expert Panel Validation 165 Contact Us 165 Disclaimer 166

List of Tables Number of Products under Development for Systemic Sclerosis (Scleroderma), H1 2016 11 Number of Products under Development for Systemic Sclerosis (Scleroderma) - Comparative Analysis, H1 2016 12 Number of Products under Development by Companies, H1 2016 13 Number of Products under Development by Companies, H1 2016 (Contd..1) 14 Number of Products under Investigation by Universities/Institutes, H1 2016 15 Comparative Analysis by Late Stage Development, H1 2016 16 Comparative Analysis by Clinical Stage Development, H1 2016 17 Comparative Analysis by Early Stage Development, H1 2016 18 Products under Development by Companies, H1 2016 19 Products under Development by Companies, H1 2016 (Contd..1) 20 Products under Investigation by Universities/Institutes, H1 2016 21 Systemic Sclerosis (Scleroderma) - Pipeline by Allergan Plc, H1 2016 22 Systemic Sclerosis (Scleroderma) - Pipeline by Angion Biomedica Corp., H1 2016 23 Systemic Sclerosis (Scleroderma) - Pipeline by arGentis Pharmaceuticals, LLC, H1 2016 24 Systemic Sclerosis (Scleroderma) - Pipeline by Bayer AG, H1 2016 25 Systemic Sclerosis (Scleroderma) - Pipeline by BioLineRx, Ltd., H1 2016 26 Systemic Sclerosis (Scleroderma) - Pipeline by BiOrion Technologies B.V., H1 2016 27 Systemic Sclerosis (Scleroderma) - Pipeline by Boehringer Ingelheim GmbH, H1 2016 28 Systemic Sclerosis (Scleroderma) - Pipeline by Bristol-Myers Squibb Company, H1 2016 29 Systemic Sclerosis (Scleroderma) - Pipeline by Corbus pharmaceuticals, Inc., H1 2016 30 Systemic Sclerosis (Scleroderma) - Pipeline by CSL Limited, H1 2016 31 Systemic Sclerosis (Scleroderma) - Pipeline by Daval International Limited, H1 2016 32 Systemic Sclerosis (Scleroderma) - Pipeline by Digna Biotech, S.L., H1 2016 33 Systemic Sclerosis (Scleroderma) - Pipeline by F. Hoffmann-La Roche Ltd., H1 2016 34 Systemic Sclerosis (Scleroderma) - Pipeline by Fibrocell Science, Inc., H1 2016 35 Systemic Sclerosis (Scleroderma) - Pipeline by GenKyoTex S.A., H1 2016 36 Systemic Sclerosis (Scleroderma) - Pipeline by GlaxoSmithKline Plc, H1 2016 37 Systemic Sclerosis (Scleroderma) - Pipeline by iBio, Inc., H1 2016 38 Systemic Sclerosis (Scleroderma) - Pipeline by Inventiva SAS, H1 2016 39 Systemic Sclerosis (Scleroderma) - Pipeline by Kadmon Corporation, LLC, H1 2016 40 Systemic Sclerosis (Scleroderma) - Pipeline by miRagen Therapeutics, Inc., H1 2016 41 Systemic Sclerosis (Scleroderma) - Pipeline by Peptinov SAS, H1 2016 42 Systemic Sclerosis (Scleroderma) - Pipeline by Ribomic Inc., H1 2016 43 Systemic Sclerosis (Scleroderma) - Pipeline by Stratatech Corporation, H1 2016 44 Systemic Sclerosis (Scleroderma) - Pipeline by Vida Therapeutics Inc., H1 2016 45 Systemic Sclerosis (Scleroderma) - Pipeline by VivaCell Biotechnology Espana S.L., H1 2016 46 Assessment by Monotherapy Products, H1 2016 47 Number of Products by Stage and Target, H1 2016 49 Number of Products by Stage and Mechanism of Action, H1 2016 52 Number of Products by Stage and Route of Administration, H1 2016 55 Number of Products by Stage and Molecule Type, H1 2016 57 Systemic Sclerosis (Scleroderma) Therapeutics - Recent Pipeline Updates, H1 2016 120 Systemic Sclerosis (Scleroderma) - Dormant Projects, H1 2016 156 Systemic Sclerosis (Scleroderma) - Dormant Projects (Contd..1), H1 2016 157 Systemic Sclerosis (Scleroderma) - Discontinued Products, H1 2016 158 List of Figures Number of Products under Development for Systemic Sclerosis (Scleroderma), H1 2016 11 Number of Products under Development for Systemic Sclerosis (Scleroderma) - Comparative Analysis, H1 2016 12 Number of Products under Development by Companies, H1 2016 13 Number of Products under Investigation by Universities/Institutes, H1 2016 15 Comparative Analysis by Late Stage Development, H1 2016 16 Comparative Analysis by Clinical Stage Development, H1 2016 17 Comparative Analysis by Early Stage Products, H1 2016 18 Assessment by Monotherapy Products, H1 2016 47 Number of Products by Top 10 Targets, H1 2016 48 Number of Products by Stage and Top 10 Targets, H1 2016 48 Number of Products by Top 10 Mechanism of Actions, H1 2016 51 Number of Products by Stage and Top 10 Mechanism of Actions, H1 2016 51 Number of Products by Top 10 Routes of Administration, H1 2016 54 Number of Products by Stage and Top 10 Routes of Administration, H1 2016 54 Number of Products by Top 10 Molecule Types, H1 2016 56 Number of Products by Stage and Top 10 Molecule Types, H1 2016 56

Allergan Plc Angion Biomedica Corp. arGentis Pharmaceuticals, LLC Bayer AG BioLineRx, Ltd. BiOrion Technologies B.V. Boehringer Ingelheim GmbH Bristol-Myers Squibb Company Corbus pharmaceuticals, Inc. CSL Limited Daval International Limited Digna Biotech, S.L. F. Hoffmann-La Roche Ltd. Fibrocell Science, Inc. GenKyoTex S.A. GlaxoSmithKline Plc iBio, Inc. Inventiva SAS Kadmon Corporation, LLC miRagen Therapeutics, Inc. Peptinov SAS Ribomic Inc. Stratatech Corporation Vida Therapeutics Inc. VivaCell Biotechnology Espana S.L.