About the Book

• The first textbook entirely devoted to systemic vasculitis (SV) edited by multinational team of contributors.
• Divided into six sections, the first section deal with epidemiology, genetics and classification of vasculitis syndrome.
• The second section deals with basic sciences and the role of neutrophils, antineutrophil cytoplasmic antibodies along with the details on skin, nerves and brain, lungs and kidney pathology.
• Contains innumerable illustrative microphotographs of various pathological changes in different organs.
• The third section deals with imaging modalities significant in diagnosing and assessing disease activity.
• The fourth section deals with the clinical manifestations based upon organ system.
• The fifth section contains chapters detailing the approach to children and adult with vasculitis, assessing disease activity and damage.
• The last section deals with individual vasculitis syndromes.
• Essential reading for practicing and academic rheumatologists, pathologists, internists and other clinicians involved in the management of patient with systemic vasculitides.

About the Author

Aman Sharma MD MAMS FIACM FICP FRCP (London)

Table of Contents:
SECTION 1 Introduction 1. Epidemiology of Systemic Vasculitis 3 David GI Scott, Paul Bacon Large-Vessel Vasculitis 3 Medium-Vessel Vasculitis 5 Small-Vessel Vasculitis ANCA-associated Vasculitis 6 Small-Vessel Vasculitis-Immune Complex 8 2. Genetics of Vasculitis 10 John Mathew, Ramya J Large-Vessel Vasculitis 10 Medium-Vessel Vasculitis 12 Small-Vessel Vasculitis 12 3. Classification of Vasculitis 16 Aman Sharma, Richard Watts American College of Rheumatology Criteria 17 Chapel Hill Consensus Conference Nomenclature System 17 European Medical Agency Algorithm 18 Classification of Childhood Vasculitis 18 SECTION 2 Basic Sciences 4. Neutrophils in ANCA-associated Vasculitis 23 Véronique Witko-Sarsat, Arnaud Roccabianca, Luc Mouthon The ANCA Antigens PR3 and MPO: Keys in the Pathophysiology of ANCA-Associated Vasculitides 23 Neutrophil Activation in ANCA-Associated Vasculitides: Role of ANCA 26 5. Antineutrophil Cytoplasmic Antibodies 29 Ashish Aggarwal, Aman Sharma, Wolfgang L Gross Antineutrophil Cytoplasmic Antibodies 29 Antineutrophil Cytoplasmic Antibody-Associated Vasculitis 29 Production of ANCA 29 Interaction between ANCAs and their Target Antigens 30 Pathophysiology of AAV 30 Genetic Association of AAV 30 Environmental Factors 30 Pathogenic B-Cell Response and Production of ANCA 30 Aberrant T-Cell Response and Granuloma Formation 31 Monocyte Activation and AAV 31 ANCA-Mediated Interaction between Neutrophils and Endothelial Cells 31 ANCA in the Diagnosis of Vasculitis 31 Should ANCA Titers may be Used for the Management of ANCA-Vasculitis? 32 6. Animal Models of ANCA Vasculitis 35 Mark Little, Alice Coughlan Passive Transfer Model 36 Bone Marrow Transplant Model 36 Mouse Model of Autoimmune Vasculitis 36 Rat Model of Experimental Autoimmune Vasculitis 36 Rodent Models of PR3-ANCA Disease 37 Insights from Animal Models 37 7. Pathogenesis of Vasculitis 40 Durga Prasanna Misra, Anupam Wakhlu, Vikas Agarwal Classification and Nomenclature 40 Pathogenesis of Large-Vessel Vasculitis 40 Pathogenesis of Medium-Vessel Vasculitis 42 Pathogenesis of Immune-Complex-Mediated Small-Vessel Vasculitis 43 ANCA-Associated Small Vessel Vasculitis 44 8. Pathology of Cutaneous Vasculitis 49 BD Radotra Large-Vessel Vasculitis 49 Medium-Vessel Vasculitis 50 Small-Vessel Vasculitis 50 9. Pathology of Pulmonary Vasculitis 54 Amanjit Kaur, Ashim Das Primary Vasculitides 54 Secondary Vasculitides 60 10. Pathology of Renal Vasculitis 64 Ritambhra Nada, Manish Rathi, Aman Sharma, J Charles Jennette Small-Vessel Vasculitis 64 Medium-Vessel Vasculitis 75 Large-Vessel Vasculitis 79 11. Pathology of Central Nervous System and Peripheral Nerves 85 BD Radotra, RK Vasishta Noninfectious Central Nervous System Vasculitides 85 Primary Angiitis of Central Nervous System (PACNS) 87 Central Nervous System Vasculitis due to Infections 90 SECTION 3 Imaging Modalities 12. Imaging in Vasculitis 95 Chirag Ahuja, NK Khandelwal Ultrasonography 95 Computed Tomography 95 Magnetic Resonance Imaging 96 Digital Subtraction Angiography 96 Imaging: What to Look for? 96 Large-Vessel Vasculitis 97 Medium-Vessel Vasculitis 99 Kawasaki Disease 101 Small-Vessel Vasculitis 101 Variable-Vessel Vasculitis 102 Single-Organ Vasculitis 103 Vasculitis Associated with Systemic Disease 103 Infection-Related Vasculitis 103 13. Positron Emission Tomography and Vasculitides 106 Enrico Tombetti, Justin C Mason Principles of PET Imaging 106 18F-FDG PET/CT in Vasculitides 107 Future Promising Technical Improvements 113 SECTION 4 Clinical Manifestations According to Organ Involvement 14. Cutaneous Vasculitis 121 Sunil Dogra, Vinay Keshavamurthy Epidemiology 121 Classification and Nomenclature of Vasculitis 121 Pathogenesis 122 Approach to a Patient with Vasculitis 124 Cutaneous Leukocytoclastic Angiitis (CLA) 126 IgA Vasculitis 126 Urticarial Vasculitis 127 Cryoglobulinemic Vasculitis 127 ANCA-Associated Vasculitis 128 Polyarteritis Nodosa and Cutaneous Arteritis 129 Treatment 129 15. Retinal Vasculitis 132 Reema Bansal, Mohit Dogra, Amod Gupta Clinical Features 133 Diagnostic Work-Up 133 Tuberculosis 135 Herpes Viruses 135 Cytomegalovirus 136 Toxoplasmosis 137 Syphilis 137 Behçet’s Disease 138 Sarcoidosis 138 Systemic Vasculitis Granulomatosis with Polyangiitis (Wegener’s Granulomatosis), Polyarteritis Nodosa, and Takayasu’s Arteritis 138 Frosted Branch Angiitis 140 16. Cardiac Manifestations 142 Rajesh Vijayvergiya, Saujatya Chakraborty Pathogenesis 142 Specific Vasculitis 143 Polyarteritis Nodosa 143 Granulomatosis with Polyangiitis (Wegener’s) 143 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) 143 Microscopic Polyangiitis 144 Cryoglobulinemic Vasculitis 144 Kawasaki’s Disease 144 Takayasu Arteritis 144 Behçet’s Disease 145 17. Pulmonary Manifestations 149 SK Jindal Clinical Manifestations 149 Granulomatosis with Polyangiitis (Wegener’s) 151 Behçet’s Disease 152 Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss Syndrome) 153 Connective Tissue Disorders 153 Miscellaneous Vasculitides with Pulmonary Manifestations 153 Pulmonary Renal Syndrome 154 Management of Pulmonary Vasculitis 154 18. Renal Manifestations 157 Manish Rathi, Aman Sharma, Ritambhra Nada Large-Vessel Vasculitis 157 Medium-Vessel Vasculitis 158 Small-Vessel Vasculitis 158 Other Vasculitis 161 19. Neurological Manifestations 164 Manish Modi, Manoj Goyal Central Nervous System Syndromes 164 Peripheral Nervous System Syndromes 167 20. Vasculitic Neuropathy 173 Manoj Goyal, Manish Modi Classification 173 Epidemiology 174 Pathogenesis and Pathology 175 Clinical Features 176 Types of Vasculitic Neuropathies 176 Diagnosis 180 Differential Diagnosis 181 Management 181 21. Gastrointestinal Manifestations 190 Saroj K Sinha, Raghavendra Prasada, Rakesh Kochhar Gastrointestinal Tract Involvement in Large-Vessel Vasculitis 190 Gastrointestinal Tract Involvement in Medium-Vessel Vasculitis 190 Gastrointestinal Tract Involvement in Small-Vessel Vasculitis 193 IgA Vasculitis (Henoch-Schönlein Purpura) 194 Vasculitis Associated with Systemic Disease 197 Behçet’s Disease Vasculitis 200 Localized Vasculitis of Gastrointestinal Tract 201 22. Head and Neck Manifestations 205 Naresh K Panda, Roshan K Verma Otolaryngologist Perspective 205 Head and Neck Manifestations in GPA 206 Otologic Manifestation 206 Rhinologic Manifestations 206 Otolaryngologist’s Role 207 Laryngeal and Tracheal Manifestation 208 Oral Manifestations 208 Differential Diagnosis 208 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Vasculitis) 208 Kawasaki Disease 209 Polyarteritis Nodosa 209 Giant Cell Arteritis 210 Head and Neck Manifestations 210 SECTION 5 Principles of Management 23. Approach to Childhood Vasculitis 217 Amita Aggarwal, Anuj Shukla Prevalence 217 Clinical Features 217 Case Vignette 218 Henoch-Schönlein Purpura 219 Behçet’s Syndrome 220 Classical Polyarteritis Nodosa 220 Takayasu’s Arteritis 221 24. Approach to Adult with Systemic Vasculitis 225 Pradeep Bambery, Vinay Sagar, Aman Sharma 25. Assessment of Disease Activity and Damage 232 Aman Sharma, Ram Nath Misra Tools for Assessment 232 Radiological Assessment 232 Computerized Tomography 233 Ultrasonography 233 Positron Emission Tomography 233 Serological Markers in Disease Assessment 233 Clinical Indices of Disease Assessment 234 Disease Activity 234 Instruments for Assessing Disease Activity 234 Birmingham Vasculitis Activity Score 235 Indian Takayasu Clinical Activity Score 235 Vasculitis Damage Index 235 Quality of Life Assessment 235 Patient Reported Outcomes 235 26. Newer and Future Treatment of Vasculitis 238 David Jayne Current Outcomes 238 Newer Therapies 239 Other Forms of Vasculitis 240 Future Therapies for Vasculitis 241 Disclosures 243 SECTION 6 Individual Vasculitis Syndromes 27. Giant Cell Arteritis and Polymyalgia Rheumatica 247 Tanaz A Kermani Case Vignette 247 Epidemiology 248 Diagnosis 249 Clinical Features 249 Investigations 250 Diagnostic Approach 252 Management 253 28. Takayasu’s Arteritis 258 Aravind G Hegde, Ruchika Goel, George Joseph, Debashish Danda Case Vignette 258 History 259 Epidemiology 259 Classification Criteria and Diagnosis 259 Pathology 260 Pathogenesis 261 Clinical Features 262 Diagnosis 263 Imaging 263 Differential Diagnosis 264 Treatment 264 Corticosteroids 264 Conventional Immunosuppressives 265 Definition of Refractory Disease in TA 265 Biologic Agents 265 Endovascular and Surgical Interventions 266 Disease Activity Assessment and Monitoring 267 Future Directions 268 29. Kawasaki Disease 272 S Madhusudan, Surjit Singh Case Vignette 272 Epidemiology 272 Etiopathogenesis 273 Clinical Features 273 Clinical Criteria for the Diagnosis of KD 274 Investigations 274 Incomplete and Atypical KD 274 Differential Diagnosis 274 Treatment 275 30. Polyarteritis Nodosa 277 Loïc Guillevin Case Vignette 277 Classification Criteria and Diagnosis 278 Epidemiology 278 Etiology and Precipitating Factors 278 Histology 280 Clinical Features 281 Laboratory Tests 284 Angiography 284 Outcome and Prognosis of PAN 284 Treatment of PAN 285 How should PAN be Treated? 285 31. Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) 289 Aman Sharma, Pradeep Bambery Case Vignette 289 Classification and Nomenclature 289 Epidemiology 290 Pathogenesis 290 Clinical Features 290 Upper Airways and Nose 291 Oral Manifestations 292 Pulmonary Involvement 292 Renal Manifestations 292 Fiberoptic Endoscopy and Bronchoscopy 294 Measurement of Disease Activity 294 Treatment 295 Morbidity 296 32. Microscopic Polyangiitis 300 Chetan Mukhtyar, P Sharma Case Vignette 300 Epidemiology 301 Diagnosis 301 Clinical Features 301 Investigations 301 Diagnostic Approach 302 Management 302 Prognosis 303 33. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) 306 Christian Pagnoux, Corisande Baldwin Case Vignette 306 Epidemiology 308 Pathogenesis 308 Diagnosis 309 Clinical Features 309 Management 314 34. Immunoglobulin A Vasculitis 320 Deepti Suri, Vikas Suri Case Vignette 320 35. Primary Angiitis of Central Nervous System 325 Atul Khasnis, Leonard Calabrese Case Vignette 325 Imaging 325 Biopsy and Pathology 326 Mimics 326 Diagnosing PACNS 327 Treatment 327 36. Behçet’s Disease 329 Fatma Alibaz-Oner, Haner Direskeneli Case Vignette 329 Epidemiology 329 Diagnosis 330 Clinical Features 330 Investigations 333 Diagnostic Approach 333 Management 333 37. Cryoglobulinemic Vasculitis 337 Cloé Comarmond, Patrice Cacoub Case Vignette 337 Epidemiology 337 Diagnosis 338 Clinical Features 339 Diagnostic Approach 340 Management 340 Therapeutic Guidelines for HCV-MC 341 38. Relapsing Polychondritis 344 Aman Sharma, Arjun D Law, Kusum Sharma, Rohini Handa Epidemiology 344 Etiopathogenesis 344 Clinical Features 345 Auricular and Vestibular Involvement 345 Joint Involvement 345 Nasal Involvement 345 Respiratory Tract Involvement 346 Cardiovascular Involvement 346 Ocular Involvement 347 Neurologic Involvement 347 Renal Involvement 347 Dermatological Involvement 347 Other Symptoms 347 Prognosis 348 Associated Conditions 348 Diagnostic Criteria 348 Differential Diagnosis 348 Investigations 348 Assessment of Disease Activity 349 Management 349 Management of Complications and Sequelae 350 39. IgG4 Disease 355 M Vishnu Vardhan Reddy Epidemiology 356 Pathogenesis 356 Diagnosis 357 Histopathological Features of IgG4-Related Disease 358 Quantitative Assessment of the IgG4 Stain 358 Image Characteristics Suggestive of IgG4-Related Disease on 18F-Fdg PET/CT 359 Pancreas 360 Aortitis 363 Peripheral Nerves 363 40. Infection-associated Vasculitis 365 Neha Garg, Ajay Wanchu Viral Infections and Vasculitis 365 HIV-associated Vasculitis 365 Parvovirus B19 Associated Vasculitis 367 Cytomegalovirus 367 Epstein-Barr Virus 367 Varicella-Zoster Virus 368 Bacterial Infections and Vasculitis 368 41. Vasculitis Mimics 372 Subramanian Ramaswamy, Vinod Ravindran Case Vignette 372 When to Suspect Vasculitic Mimics? 373 Infections 373 Disorders of the Connective Tissue Matrix 374 Drug-induced Vasculitis 374 Malignancy-associated Vasculitis 375 Atherothrombotic Vascular Disorders 375 Miscellaneous 375 Diagnostic Approach and Management 376 42. Secondary Vasculitis 378 Varun Dhir Rheumatoid Vasculitis 378 Lupus Vasculitis 381 Paraneoplastic Vasculitis 383 Infection-associated Vasculitis 384 43. Cogan’s Syndrome 388 Tommy CY Chan, Vishal Jhanji Epidemiology 388 Clinical Features 388 Ocular Manifestations 389 Audiovestibular Manifestations 389 Systemic Manifestations 389 Diagnostic Approach and Investigations 389 Treatment 390 44. Malignancy-associated Vasculitis 392 Narendra Kumar, Pankaj Kumar, Rupali Agarwal, Shabab Lalit Angurana Association of Vasculitis and Malignancy 392 Risk Factors and Pathogenesis of Malignancy-associated Vasculitis 393 Clinical Presentation and Prognosis 394 Specific Malignancy-associated Vasculitis 395 ANCA-associated Vasculitis and Malignancy 397 When and How to Rule Out Underlying Malignancy in a Patient of Vasculitis? 398 45. Pregnancy and Vasculitis 401 Shankar Naidu, Susmita Sharma, Aman Sharma, Surjit Singh Fertility Concerns 401 Conception and Preconception Counseling 402 Pregnancy Outcomes 402 Large-Vessel Vasculitis 402 Medium-Vessel Vasculitis 403 Small-Vessel Vasculitis 403 Variable-Vessel Vasculitis 404 46. Prevention and Management of Infections 407 Gaurav Prakash, Subhash Varma, Pankaj Malhotra Defining Immunocompromised Host 407 Common Medications in the Treatment of Vasculitis that Cause Immunosuppression 408 Prevention of Infections in the Patient Treated for Vasculitis 408 Treatment of Vasculitis in Specific Infections 410 Clinical Features and Evaluation of Infections in An Immunocompromised Host 410 Management 411 Index 413