# 18.14.8 Pulmonary amyloidosis 4261 S.J. Bourke # 18.14.8 Pulmonary amyloidosis 4261 S.J. Bourke Contents xxvi 18.11 Diffuse parenchymal lung diseases  4166 18.11.1 Diffuse parenchymal lung disease: An introduction  4166 F. Teo and A.U. Wells 18.11.2 Idiopathic pulmonary fibrosis  4177 P.L. Molyneaux, A.G. Nicholson, N. Hirani, and A.U. Wells 18.11.3 Bronchiolitis obliterans and cryptogenic organizing pneumonia  4185 Vasilis Kouranos and A.U. Wells 18.11.4 The lung in autoimmune rheumatic disorders  4191 M.A. Kokosi and A.U. Wells 18.11.5 The lung in vasculitis  4200 G.A. Margaritopoulos and A.U. Wells 18.12 Sarcoidosis  4208 Robert P. Baughman and Elyse E. Lower 18.13 Pneumoconioses  4219 P.T. Reid 18.14 Miscellaneous conditions  4235 18.14.1 Diffuse alveolar haemorrhage  4235 S.J. Bourke and G.P. Spickett 18.14.2 Eosinophilic pneumonia  4238 S.J. Bourke and G.P. Spickett 18.14.3 Lymphocytic infiltrations of the lung  4241 S.J. Bourke 18.14.4 Hypersensitivity pneumonitis  4244 S.J. Bourke and G.P. Spickett 18.14.5 Pulmonary Langerhans’ cell histiocytosis  4256 S.J. Bourke 18.14.6 Lymphangioleiomyomatosis  4257 S.J. Bourke 18.14.7 Pulmonary alveolar proteinosis  4259 S.J. Bourke 18.14.8 Pulmonary amyloidosis  4261 S.J. Bourke 18.14.9 Lipoid (lipid) pneumonia  4263 S.J. Bourke 18.14.10 Pulmonary alveolar microlithiasis  4265 S.J. Bourke 18.14.11 Toxic gases and aerosols  4267 Chris Stenton 18.14.12 Radiation pneumonitis  4271 S.J. Bourke 18.14.13 Drug-​induced lung disease  4272 S.J. Bourke 18.15 Chronic respiratory failure  4282 Michael I. Polkey and P.M.A. Calverley 18.16 Lung transplantation  4292 P. Hopkins and A.J. Fisher 18.17 Pleural diseases  4305 D. de Fonseka, Y.C. Gary Lee, and N.A. Maskell 18.18 Disorders of the thoracic cage and diaphragm  4328 John M. Shneerson and Michael I. Polkey 18.19 Malignant diseases  4338 18.19.1 Lung cancer  4338 S.G. Spiro and N. Navani 18.19.2 Pulmonary metastases  4360 S.G. Spiro 18.19.3 Pleural tumours  4361 Y.C. Gary Lee 18.19.4 Mediastinal tumours and cysts  4368 Y.C. Gary Lee and Helen E. Davies SECTION 19 Rheumatological disorders Section editor: Richard A. Watts 19.1 Joints and connective tissue—​structure and function  4379 Thomas Pap, Adelheid Korb-​Pap, Christine Hartmann, and Jessica Bertrand 19.2 Clinical presentation and diagnosis of rheumatological disorders  4386 Christopher Deighton and Fiona Pearce 19.3 Clinical investigation  4395 Michael Doherty and Peter C. Lanyon 19.4 Back pain and regional disorders  4406 Carlo Ammendolia and Danielle Southerst 19.5 Rheumatoid arthritis  4415 Kenneth F. Baker and John D. Isaacs 19.6 Spondyloarthritis and related conditions  4441 Jürgen Braun and Joachim Sieper 19.7 Infection and arthritis  4457 Graham Raftery and Muddassir Shaikh 19.8 Reactive arthritis  4464 Carmel B. Stober and Hill Gaston 19.9 Osteoarthritis  4470 Andrew J. Barr and Philip G. Conaghan Contents xxvii 19.10 Crystal-​related arthropathies  4482 Edward Roddy and Michael Doherty 19.11 Autoimmune rheumatic disorders and vasculitides  4495 19.11.1 Introduction  4495 David A. Isenberg and Ian Giles 19.11.2 Systemic lupus erythematosus and related disorders  4499 Anisur Rahman and David A. Isenberg 19.11.3 Systemic sclerosis (scleroderma)  4513 Christopher P. Denton and Carol M. Black 19.11.4 Sjögren’s syndrome  4532 Wan-​Fai Ng 19.11.5 Inflammatory myopathies  4537 Ingrid E. Lundberg, Hector Chinoy, and Robert Cooper 19.11.6 Large vessel vasculitis  4546 Raashid Luqmani and Cristina Ponte 19.11.7 ANCA-​associated vasculitis  4556 David Jayne 19.11.8 Polyarteritis nodosa  4569 Loïc Guillevin 19.11.9 Small vessel vasculitis  4573 Richard A. Watts 19.11.10 Behçet’s syndrome  4579 Sebahattin Yurdakul, Izzet Fresko, and Hasan Yazici 19.11.11 Polymyalgia rheumatica  4584 Bhaskar Dasgupta and Eric L. Matteson 19.11.12 Kawasaki disease  4590 Brian W. McCrindle 19.12 Miscellaneous conditions presenting to the rheumatologist  4598 Stuart Carter, Lisa Dunkley, and Ade Adebajo SECTION 20 Disorders of the skeleton Section editor: Cyrus Cooper 20.1 Skeletal disorders—​general approach and clinical conditions  4615 B. Paul Wordsworth and M.K. Javaid 20.2 Inherited defects of connective tissue: Ehlers–​Danlos syndrome, Marfan’s syndrome, and pseudoxanthoma elasticum  4670 N.P. Burrows 20.3 Osteomyelitis  4688 Martin A. McNally and Anthony R. Berendt 20.4 Osteoporosis  4696 Nicholas C. Harvey, Juliet Compston, and Cyrus Cooper 20.5 Osteonecrosis, osteochondrosis, and osteochondritis dissecans  4703 Gavin Clunie 20.6 Bone cancer  4709 Helen Hatcher SECTION 21 Disorders of the kidney and urinary tract Section editor: John D. Firth 21.1 Structure and function of the kidney  4717 Steve Harper and Robert Unwin 21.2 Electrolyte disorders  4729 21.2.1 Disorders of water and sodium homeostasis  4729 Michael L. Moritz and Juan Carlos Ayus 21.2.2 Disorders of potassium homeostasis  4748 John D. Firth 21.3 Clinical presentation of renal disease  4764 Richard E. Fielding and Ken Farrington 21.4 Clinical investigation of renal disease  4781 Andrew Davenport 21.5 Acute kidney injury  4807 John D. Firth 21.6 Chronic kidney disease  4830 Alastair Hutchison 21.7 Renal replacement therapy  4861 21.7.1 Haemodialysis  4861 Robert Mactier 21.7.2 Peritoneal dialysis  4874 Simon Davies 21.7.3 Renal transplantation  4879 Nicholas Torpey and John D. Firth 21.8 Glomerular diseases  4909 21.8.1 Immunoglobulin A nephropathy and IgA vasculitis (HSP)  4909 Jonathan Barratt and John Feehally 21.8.2 Thin membrane nephropathy  4918 Peter Topham and John Feehally Contents xxviii 21.8.3 Minimal-​change nephropathy and focal segmental glomerulosclerosis  4919 Moin Saleem and Lisa Willcocks 21.8.4 Membranous nephropathy  4928 An S. De Vriese and Fernando C. Fervenza 21.8.5 Proliferative glomerulonephritis  4933 Alan D. Salama and Mark A. Little 21.8.6 Membranoproliferative glomerulonephritis  4937 Tabitha Turner-​Stokes and Mark A. Little 21.8.7 Antiglomerular basement membrane disease  4943 Mårten Segelmark and Thomas Hellmark 21.9 Tubulointerstitial diseases  4951 21.9.1 Acute interstitial nephritis  4951 Simon D. Roger 21.9.2 Chronic tubulointerstitial nephritis  4956 Marc E. De Broe, Channa Yamasumana, Patrick C. D’Haese, Monique M. Elseviers, and Benjamin Vervaet 21.10 The kidney in systemic disease  4975 21.10.1 Diabetes mellitus and the kidney  4975 Rudolf Bilous 21.10.2 The kidney in systemic vasculitis  4988 David Jayne 21.10.3 The kidney in rheumatological disorders  5001 Liz Lightstone and Hannah Beckwith 21.10.4 The kidney in sarcoidosis  5012 Ingeborg Hilderson and Jan Donck 21.10.5 Renal involvement in plasma cell dyscrasias, immunoglobulin-​based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias  5016 Pierre Ronco, Frank Bridoux, and Arnaud Jaccard 21.10.6 Haemolytic uraemic syndrome  5027 Edwin K.S. Wong and David Kavanagh 21.10.7 Sickle cell disease and the kidney  5032 Claire C. Sharpe 21.10.8 Infection-​associated nephropathies  5034 A. Neil Turner 21.10.9 Malignancy-​associated renal disease  5041 A. Neil Turner 21.10.10 Atherosclerotic renovascular disease  5044 Philip A. Kalra and Diana Vassallo 21.11 Renal diseases in the tropics  5049 Vivekanand Jha 21.12 Renal involvement in genetic disease  5065 D. Joly and J.P. Grünfeld 21.13 Urinary tract infection  5074 Charles Tomson and Neil Sheerin 21.14 Disorders of renal calcium handling, urinary stones, and nephrocalcinosis  5093 Christopher Pugh, Elaine M. Worcester, Andrew P. Evan, and Fredric L. Coe 21.15 The renal tubular acidoses  5104 John A. Sayer and Fiona E. Karet 21.16 Disorders of tubular electrolyte handling  5112 Nine V.A.M. Knoers and Elena N. Levtchenko 21.17 Urinary tract obstruction  5124 Muhammad M. Yaqoob and Kieran McCafferty 21.18 Malignant diseases of the urinary tract  5136 Tim Eisen, Freddie C. Hamdy, and Robert A. Huddart 21.19 Drugs and the kidney  5150 Aine Burns and Caroline Ashley Index Volume 4 List of abbreviations  xxxv List of contributors  xlv SECTION 22 Haematological disorders Section editors: Chris Hatton and Deborah Hay 22.1 Introduction to haematology  5169 Chris Hatton 22.2 Haematopoiesis  5172 22.2.1 Cellular and molecular basis of haematopoiesis  5172 Paresh Vyas and N. Asger Jakobsen 22.2.2 Diagnostic techniques in the assessment of haematological malignancies  5181 Wendy N. Erber Contents xxix 22.3 Myeloid disease  5189 22.3.1 Granulocytes in health and disease  5189 Joseph Sinning and Nancy Berliner 22.3.2 Myelodysplastic syndromes  5197 Charlotte K. Brierley and David P. Steensma 22.3.3 Acute myeloid leukaemia  5205 Nigel Russell and Alan Burnett 22.3.4 Chronic myeloid leukaemia  5213 Mhairi Copland and Tessa L. Holyoake† 22.3.5 The polycythaemias  5227 Daniel Aruch and Ronald Hoffman 22.3.6 Thrombocytosis and essential thrombocythaemia  5239 Daniel Aruch and Ronald Hoffman 22.3.7 Primary myelofibrosis  5247 Evan M. Braunstein and Jerry L. Spivak 22.3.8 Eosinophilia  5254 Peter F. Weller 22.3.9 Histiocytosis  5259 Chris Hatton 22.4 Lymphoid disease  5263 22.4.1 Introduction to lymphopoiesis  5263 Caron A. Jacobson and Nancy Berliner 22.4.2 Acute lymphoblastic leukaemia  5269 H. Josef Vormoor, Tobias F. Menne, and Anthony V. Moorman 22.4.3 Hodgkin lymphoma  5280 Vijaya Raj Bhatt and James O. Armitage 22.4.4 Non-​Hodgkin lymphoma  5288 Vijaya Raj Bhatt and James O. Armitage 22.4.5 Chronic lymphocytic leukaemia  5302 Clive S. Zent and Aaron Polliack 22.4.6 Plasma cell myeloma and related monoclonal gammopathies  5310 S. Vincent Rajkumar and Robert A. Kyle 22.5 Bone marrow failure  5325 22.5.1 Inherited bone marrow failure syndromes  5325 Irene Roberts and Inderjeet S. Dokal 22.5.2 Acquired aplastic anaemia and pure red cell aplasia  5336 Judith C.W. Marsh, Shreyans Gandhi, and Ghulam J. Mufti 22.5.3 Paroxysmal nocturnal haemoglobinuria  5348 Lucio Luzzatto 22.6 Erythroid disorders  5354 22.6.1 Erythropoiesis  5354 Vijay G. Sankaran 22.6.2 Anaemia: pathophysiology, classification, and clinical features  5359 David J. Weatherall† and Chris Hatton 22.6.3 Anaemia as a challenge to world health  5366 David J. Roberts and David J. Weatherall† 22.6.4 Iron metabolism and its disorders  5371 Timothy M. Cox and John B. Porter 22.6.5 Anaemia of inflammation  5402 Sant-​Rayn Pasricha and Hal Drakesmith 22.6.6 Megaloblastic anaemia and miscellaneous deficiency anaemias  5407 A.V. Hoffbrand 22.6.7 Disorders of the synthesis or function of haemoglobin  5426 Deborah Hay and David J. Weatherall† 22.6.8 Anaemias resulting from defective maturation of red cells  5450 Stephen J. Fuller and James S. Wiley 22.6.9 Disorders of the red cell membrane  5456 Patrick G. Gallagher 22.6.10 Erythrocyte enzymopathies  5463 Alberto Zanella and Paola Bianchi 22.6.11 Glucose-​6-​phosphate dehydrogenase deficiency  5472 Lucio Luzzatto 22.6.12 Acquired haemolytic anaemia  5479 Amy Powers and Leslie Silberstein 22.7 Haemostasis  5490 22.7.1 The biology of haemostasis and thrombosis  5490 Gilbert C. White, II, Harold R. Roberts, and Nigel S. Key 22.7.2 Evaluation of the patient with a bleeding tendency  5509 Trevor Baglin 22.7.3 Thrombocytopenia and disorders of platelet function  5520 Nicola Curry and Susie Shapiro 22.7.4 Genetic disorders of coagulation  5532 Eleanor S. Pollak and Katherine A. High 22.7.5 Acquired coagulation disorders  5546 T.E. Warkentin † It is with great regret that we report that Tessa L. Holyoake died on 30 August, 2017. † It is with great regret that we report that David J. Weatherall died on 8 December, 2018. Contents xxx 22.8 Transfusion and transplantation  5563 22.8.1 Blood transfusion  5563 D.S. Giovanniello and E.L. Snyder 22.8.2 Haemopoietic stem cell transplantation  5579 E.C. Gordon-​Smith and Emma C. Morris SECTION 23 Disorders of the skin Section editor: Roderick J. Hay 23.1 Structure and function of skin  5591 John A. McGrath 23.2 Clinical approach to the diagnosis of skin disease  5596 Vanessa Venning 23.3 Inherited skin disease  5602 Thiviyani Maruthappu and David P. Kelsell 23.4 Autoimmune bullous diseases  5612 Kathy Taghipour and Fenella Wojnarowska 23.5 Papulosquamous disease  5621 Christopher E.M. Griffiths 23.6 Dermatitis/​eczema  5630 Peter S. Friedmann, Michael J. Arden-​Jones, and Roderick J. Hay 23.7 Cutaneous vasculitis, connective tissue diseases, and urticaria  5639 Volha Shpadaruk and Karen E. Harman 23.8 Disorders of pigmentation  5677 Eugene Healy 23.9 Photosensitivity  5688 Hiva Fassihi and Jane McGregor 23.10 Infections of the skin  5695 Roderick J. Hay 23.11 Sebaceous and sweat gland disorders  5699 Alison M. Layton 23.12 Blood and lymphatic vessel disorders  5709 Peter S. Mortimer and Roderick J. Hay 23.13 Hair and nail disorders  5724 David de Berker 23.14 Tumours of the skin  5732 Edel O’Toole 23.15 Skin and systemic diseases  5743 Clive B. Archer and Charles M.G. Archer 23.16 Cutaneous reactions to drugs  5752 Sarah Walsh, Daniel Creamer, and Haur Yueh Lee 23.17 Management of skin disease  5761 Rod Sinclair SECTION 24 Neurological disorders Section editor: Christopher Kennard 24.1 Introduction and approach to the patient with neurological disease  5775 Alastair Compston and Christopher Kennard 24.2 Mind and brain: Building bridges between neurology, psychiatry, and psychology  5778 Adam Zeman 24.3 Clinical investigation of neurological disease  5781 24.3.1 Lumbar puncture  5781 R. Rhys Davies and Andrew J. Larner 24.3.2 Electrophysiology of the central and peripheral nervous systems  5785 Christian Krarup 24.3.3 Imaging in neurological diseases  5802 Andrew J. Molyneux, Shelley Renowden, and Marcus Bradley 24.3.4 Investigation of central motor pathways: Magnetic brain stimulation  5817 K.R. Mills 24.4 Higher cerebral function  5821 24.4.1 Disturbances of higher cerebral function  5821 Peter J. Nestor 24.4.2 Alzheimer’s disease and other dementias  5830 Jonathan M. Schott 24.5 Epilepsy and disorders of consciousness  5860 24.5.1 Epilepsy in later childhood and adulthood  5860 Arjune Sen and M.R. Johnson 24.5.2 Narcolepsy  5882 Matthew C. Walker 24.5.3 Sleep disorders  5886 Paul J. Reading 24.5.4 Syncope  5896 Andrew J. Larner 24.5.5 The unconscious patient  5901 David Bates Contents xxxi 24.5.6 Brainstem death and prolonged disorders of consciousness  5908 Ari Ercole, Peter J. Hutchinson, and John D. Pickard 24.6 Disorders of the special senses  5913 24.6.1 Visual pathways  5913 Sara Ajina and Christopher Kennard 24.6.2 Eye movements and balance  5922 Michael Strupp and Thomas Brandt 24.6.3 Hearing loss  5931 Linda Luxon 24.7 Disorders of movement  5937 24.7.1 Subcortical structures: The cerebellum, basal ganglia, and thalamus  5937 Mark J. Edwards and Penelope Talelli 24.7.2 Parkinsonism and other extrapyramidal diseases  5946 Elisaveta Sokolov, Vinod K. Metta, and K. Ray Chaudhuri 24.7.3 Movement disorders other than Parkinson’s disease  5956 Bettina Balint and Kailash Bhatia 24.7.4 Ataxic disorders  5976 Nicholas Wood 24.8 Headache  5987 Peter J. Goadsby 24.9 Brainstem syndromes  6006 David Bates 24.10 Specific conditions affecting the central nervous system  6010 24.10.1 Stroke: Cerebrovascular disease  6010 J. van Gijn (revised by Peter M. Rothwell) 24.10.2 Demyelinating disorders of the central nervous system  6026 Alasdair Coles and Siddharthan Chandran 24.10.3 Traumatic brain injury  6042 Tim Lawrence and Laurence Watkins 24.10.4 Intracranial tumours  6048 Jeremy Rees 24.10.5 Idiopathic intracranial hypertension  6054 Alexandra Sinclair 24.11 Infections of the central nervous system  6060 24.11.1 Bacterial infections  6060 Diederik van de Beek and Guy E. Thwaites 24.11.2 Viral infections  6082 Fiona McGill, Jeremy Farrar, Bridget Wills, Menno De Jong, David A. Warrell, and Tom Solomon 24.11.3 Intracranial abscesses  6097 Tim Lawrence and Richard S.C. Kerr 24.11.4 Neurosyphilis and neuro-​AIDS  6100 Hadi Manji 24.11.5 Human prion diseases  6109 Simon Mead and R.G. Will 24.12 Disorders of cranial nerves  6120 Robert D.M. Hadden 24.13 Disorders of the spinal cord  6127 24.13.1 Diseases of the spinal cord  6127 Anu Jacob and Andrew J. Larner 24.13.2 Spinal cord injury and its management  6135 Wagih El Masri(y) and Michael Barnes 24.14 Diseases of the autonomic nervous system  6150 Christopher J. Mathias and David A. Low 24.15 The motor neuron diseases  6166 Tom Jenkins, Alice Brockington, and Pamela J. Shaw 24.16 Diseases of the peripheral nerves  6176 Robert D.M. Hadden 24.17 Inherited neurodegenerative diseases  6197 Swati Sathe 24.18 Disorders of the neuromuscular junction  6295 David Hilton-​Jones and Jacqueline Palace 24.19 Disorders of muscle  6304 24.19.1 Structure and function of muscle  6304 Michael G. Hanna and Enrico Bugiardini 24.19.2 Muscular dystrophy  6310 Kate Bushby and Chiara Marini-​Bettolo 24.19.3 Myotonia  6328 David Hilton-​Jones 24.19.4 Metabolic and endocrine disorders  6334 David Hilton-​Jones and Richard Edwards 24.19.5 Mitochondrial disease  6343 Patrick F. Chinnery and D.M. Turnbull 24.20 Developmental abnormalities of the central nervous system  6350 Chris M. Verity, Jane A. Hurst, and Helen V. Firth 24.21 Acquired metabolic disorders and the nervous system  6368 Neil Scolding 24.22 Neurological complications of systemic disease  6376 Neil Scolding Contents xxxii 24.23 Paraneoplastic neurological syndromes  6384 Jeremy Rees 24.24 Autoimmune encephalitis and Morvan’s syndrome  6393 Camilla Buckley and Angela Vincent SECTION 25 Disorders of the eye Section editor: Christopher P. Conlon 25.1 The eye in general medicine  6399 Tasanee Braithwaite, Richard W.J. Lee, and Peng T. Khaw SECTION 26 Psychiatric and drug-​related disorders Section editor: Michael Sharpe 26.1 General introduction  6445 Michael Sharpe 26.2 The psychiatric assessment of the medical patient  6447 Jane Walker, Roger Smyth, and Michael Sharpe 26.3 Common psychiatric presentations in medical patients  6454 26.3.1 Confusion  6454 Bart Sheehan and Thomas Jackson 26.3.2 Self-​harm  6457 Kate E.A. Saunders and Keith Hawton 26.3.3 Medically unexplained symptoms  6460 Michael Sharpe 26.3.4 Low mood  6462 Jane Walker 26.4 Psychiatric treatments in the medically ill  6465 26.4.1 Psychopharmacology in medical practice  6465 Philip J. Cowen 26.4.2 Psychological treatments  6470 Michael Sharpe and Simon Wessely 26.5 Specific psychiatric disorders  6475 26.5.1 Delirium  6475 Bart Sheehan 26.5.2 Dementia  6478 Bart Sheehan 26.5.3 Organic psychoses  6482 Curtis McKnight and Jason Caplan 26.5.4 Alcohol misuse  6486 Jonathan Wood 26.5.5 Substance misuse  6490 Stephen Potts 26.5.6 Depressive disorder  6493 Joseph Cerimele and Lydia Chwastiak 26.5.7 Bipolar disorder  6498 Kate E.A. Saunders and John Geddes 26.5.8 Anxiety disorders  6501 Ted Liao and Steve Epstein 26.5.9 Acute stress disorder, adjustment disorders, and post-​traumatic stress disorder  6506 Jonathan I. Bisson 26.5.10 Eating disorders  6509 Christopher G. Fairburn 26.5.11 Schizophrenia  6513 Stephen M. Lawrie 26.5.12 Somatic symptom and related disorders  6517 Michael Sharpe 26.5.13 Personality disorders  6520 Iain Jordan 26.6 Changing unhealthy behaviours  6524 26.6.1 Brief interventions for excessive alcohol consumption  6524 Amy O’Donnell, Eileen Kaner, and Nick Heather 26.6.2 Obesity and weight management  6529 Susan Jebb and Paul Aveyard 26.6.3 Smoking cessation  6533 Paul Aveyard 26.7 Psychiatry, liaison psychiatry, and psychological medicine  6536 Michael Sharpe SECTION 27 Forensic medicine Section editor: John D. Firth 27.1 Forensic and legal medicine  6541 Jason Payne-​James, Paul Marks, Ralph Bouhaidar, and Steven B. Karch Contents xxxiii SECTION 28 Sport and exercise medicine Section editor: John D. Firth 28.1 Sport and exercise medicine  6565 Cathy Speed SECTION 29 Biochemistry in medicine Section editor: Christopher P. Conlon 29.1 The use of biochemical analysis for diagnosis and management  6577 Brian Shine and Nishan Guha SECTION 30 Acute medicine Section editor: John D. Firth 30.1 Acute medical presentations  6591 Sian Coggle, Elaine Jolly, and John D. Firth 30.2 Practical procedures  6644 Elaine Jolly, Sian Coggle, and John D. Firth Index