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Objectives To examine associations between function, quality of life and structural outcomes in patients achieving remission vs low disease activity in early RA. Methods Demographic, clinical and radiographic variables were collected at baseline and then annually from the Early Rheumatoid Arthritis Study (ERAS) and Early Rheumatoid Arthritis Network (ERAN) inception cohorts in routine care from 1986 to 2012. Disease activity was categorized: mean DAS28 score between years 1 and 5: remission [mean remission DAS (mRDAS) Results Of 2701 patients, 562 (21%) were categorized mRDAS, 330 (12%) mLDAS, 279 (10%) sRDAS, 203 (7.5%) sLDAS and 93 (3%) sBR. Patients categorized as mRDAS had increasingly divergent improved HAQ, SF-36 PCS, MCS and total SvdH scores compared with mLDAS (P-values 0.001 to Conclusion These findings from routine care support ACR/EULAR guidelines that remission is a preferable goal over low disease activity in early RA.

Objectives To describe the prevalence of haematological abnormalities in individuals with RA at the point of diagnosis in primary care and the associations between haematological abnormalities, vaccinations and subsequent risk of common infections. Methods We studied 6591 individuals with newly diagnosed RA between 2004 and 2016 inclusive using the UK Royal College of General Practitioners Research and Surveillance Centre primary care database. The prevalence of haematological abnormalities at diagnosis (anaemia, neutropenia and lymphopenia) was established. Cox proportional hazards models were used to evaluate the association between each haematological abnormality and time to common infections and the influence of vaccination status (influenza and pneumococcal vaccine) on time to common infections in individuals with RA compared with a matched cohort of individuals without RA. Results Anaemia was common at RA diagnosis (16.1% of individuals), with neutropenia (0.6%) and lymphopenia (1.4%) less so. Lymphopenia and anaemia were associated with increased infection risk [hazard ratio (HR) 1.18 (95% CI 1.08, 1.29) and HR 1.37 (95% CI 1.08, 1.73), respectively]. There was no evidence of an association between neutropenia and infection risk [HR 0.94 (95% CI 0.60, 1.47)]. Pneumonia was much more common in individuals with early RA compared with controls. Influenza vaccination was associated with reduced risk of influenza-like illness only for individuals with RA [HR 0.58 (95% CI 0.37, 0.90)]. Conclusion At diagnosis, anaemia and lymphopenia, but not neutropenia, increase the risk of common infections in individuals with RA. Our data support the effectiveness of the influenza vaccination in individuals with RA.

The therapeutic armamentarium available for treatment of rheumatoid arthritis (RA) has changed significantly over the past 30 years, transforming the therapeutic landscape and prognosis for a substantial proportion of patients with RA. Combination therapies represent an important therapeutic paradigm for management of rheumatoid arthritis. The rationale for combination therapies is clear and demonstrated to bring treatment benefit to patients achieving lower disease activity scores and reduced radiologic progression according to ‘treat-to-target’ principles. A rigorous evidence-based debate is required involving not only parameters related to disease activity scores and radiologic progression, but related to the cost-effectiveness analysis of using many of these newer agents compared to older csDMARDs. This chapter addresses the evidence related to the utilization of combination strategies for the management of RA as compared to monotherapy.

This chapter outlines the basic science and theory of rheumatology required for MRCP Part 1, before addressing the clinical aspects of rheumatology for MRCP Part 2. To ensure effective revision, material is presented in short sections with bullet lists, tables, and boxes to highlight key facts.

Objectives We assessed comorbidity burden in people with RA at diagnosis and early disease (3 years) and its association with early mortality and joint destruction. The association between lung disease and mortality in RA is not well studied; we also explored this relationship. Methods From a contemporary UK-based population (n = 1, 475 762) we identified a cohort with incident RA (n = 6591). The prevalence of comorbidities at diagnosis of RA and at 3 years was compared with age- and gender-matched controls (n = 6591). In individuals with RA we assessed the prognostic value of the Charlson Comorbidity Index and Rheumatic Disease Comorbidity Index calculated at diagnosis for all-cause mortality and joint destruction (with joint surgery as a surrogate marker). We separately evaluated the association between individual lung diseases [chronic obstructive pulmonary disease (COPD), asthma and interstitial lung disease] and mortality. Results Respiratory disease, cardiovascular disease, stroke, diabetes, previous fracture and depression were more common (P < 0.05) in patients with RA at diagnosis than controls. Comorbidity (assessed using RDCI) was associated with all-cause mortality in RA [adjusted hazard ratio (HR) 1.26, 95% CI 1.00–1.60]. There was no association with joint destruction. COPD, but not asthma, was associated with mortality (COPD HR 2.84, 95% CI 1.13–7.12). Conclusion There is an excess burden of comorbidity at diagnosis of RA including COPD, asthma and interstitial lung disease. COPD is a major predictor of early mortality in early RA. Early assessment of comorbidity including lung disease should form part of the routine management of RA patients.

The Oxford Handbook of Rheumatology, 4th edition, includes a chapter on the hereditary diseases of connective tissue. Of importance, up-to-date genetic information and classification of subtypes of osteogenesis imperfecta is reviewed. There is a summary of a broad range of diseases of connective tissue including Marfan syndrome, Stickler disease, and arthrogryposis—conditions encountered occasionally by the rheumatologist. The chapter details the new 2017 criteria for Ehlers–Danlos syndrome and outlines the new classification for the hypermobility spectrum disorders and the distinction between generalized, localized, and regional hypermobility and hypermobility Ehlers–Danlos syndrome, both in adults and in children.

The Oxford Handbook of Rheumatology, 4th edition, takes the reader through a pragmatic and realistic way of assessing musculoskeletal and rheumatological disease presentation in its first four chapters. The text reflects real life, with differential diagnoses suggested as information becomes available through the details from history, then examination, then investigations. The chapters focus on patterns of, and focal regional, symptoms in adults and children. In this chapter, the book considers musculoskeletal and rheumatic conditions which occur in, or are closely associated with, other conditions of the skin, eye, heart, lung, kidney, and in hormonal, neurological, and gastrointestinal systems. Such an approach should help general practitioners and rheumatology trainees caring for their patients across their medical comorbidities and in general medicine. There is also a section on musculoskeletal–rheumatological features in patients with neoplasia. The chapter focuses primarily on adult disease.

The Oxford Handbook of Rheumatology 4th edition, has been extensively updated to thoroughly review aspects of musculoskeletal pain. Pain pathophysiology is reviewed. Chronic pain and fibromyalgia in adults and in children and adolescents is dealt with in detail. The reader is advised to cross reference from this chapter to Chapters 1–3 in the Handbook, where regional musculoskeletal pain conditions are listed and reviewed. In localized pain syndromes, the chapter has an overview of complex regional pain syndrome (CRPS), which is not infrequently encountered in rheumatology and musculoskeletal clinics. Included in detail for this edition, is the assessment and management of pain in children, which is a highly specialized clinical area of medicine and will be of use to the adult rheumatologist and general practitioner as well as paediatric specialists. Readers should cross reference to Chapter 23 on medications, for ‘pain medications’ in the Handbook

This chapter describes the clinical and laboratory classification criteria for antiphospholipid syndrome. It highlights the complexity and diagnostic challenges of antiphospholipid syndrome and discusses potential mimics of the disease that need to be excluded. Current therapeutic targets as well as novel therapeutic avenues are explored. The chapter finishes with a section on catastrophic antiphospholipid syndrome, which constitutes a rheumatological emergency. The clinical presentation, laboratory features, treatment, and outcomes of this life-threatening presentation of antiphospholipid syndrome are also discussed.

This chapter describes the epidemiology, aetiopathogenesis, presenting features, clinical manifestations, and management of osteoarthritis. Commonly used investigations for osteoarthritis, their indication, and interpretation are described. A summary of the commonly used drugs for the treatment of osteoarthritis, their mechanism of action, and key points for starting are included. Non-pharmacological holistic management is also discussed. Exciting emerging therapies that could change the future landscape of osteoarthritis management are introduced.

This extensive chapter facilitates a focused regional musculoskeletal assessment with additional detail specific to children and adolescents. Taking each region or joint in turn, the reader is supported with (1) tables of differential diagnoses; (2) detailed descriptions of anatomy including reference ranges; (3) lines of enquiry that identify work- or sport-related injury; (4) examination that recognizes radiation, neuropathy, impingement, myopathy, and stress; (5) functional assessment; and (6) specific tests. In children, there is an additional focus on developmental or normal variants and dysplasia. The chapter also draws attention to common errors in assessment and mimics, the relative merits of investigation, and, when not covered elsewhere, treatments of joint-specific problems. The chapter is thorough, although not exhaustive, and supplements general enquiry.

This chapter focuses on Sjögren’s syndrome and covers epidemiological, pathophysiological, and clinical aspects of disease. It discusses classification criteria and disease-specific features, along with potential differential diagnoses. The chapter also includes a section on the extra-glandular (systemic) manifestations of disease, illustrating its potential for multiorgan involvement. The chapter provides a guide to the reader regarding how to best approach a patient presenting with related symptoms and signs. Furthermore, it discusses relevant investigations for the key manifestations of disease, the sicca symptoms, and describes common biopsy findings. The chapter closes with the treatment of Sjögren’s syndrome and a note on prognosis.

This chapter explores the epidemiology and pathophysiology of idiopathic inflammatory myopathies. The chapter takes a focus on polymyositis and dermatomyositis and describes relevant diagnostic criteria, clinical presentations, investigations, and therapeutic options. Other inflammatory myopathies discussed include inclusion-body myositis and other (adult) inflammatory myopathies such as those induced by drugs. The chapter closes with a description of juvenile idiopathic inflammatory myopathies, a heterogeneous group of inflammatory muscle conditions affecting young people and consisting of juvenile dermatomyositis and amyopathic juvenile dermatomyositis among others.

The multisystem, autoimmune nature of systemic lupus erythematosus (SLE) and its broad spectrum of presenting clinical manifestations are explored in detail in this chapter. Important comorbidities and associations between SLE and the antiphospholipid syndrome and pregnancy are also discussed. The chapter details the various autoantibodies associated with the disease, along with their prevalence and associated clinical features. In addition, it provides a detailed review of the management of SLE, starting from conservative measures through to disease-modifying, immunosuppressive therapy to plasma exchange and stem cell transplantation. Novel therapeutic agents are also summarized, along with a note on prognosis. The chapter includes a section on childhood and neonatal SLE, covering presentation, relevant investigations, and treatment.

General rheumatological assessment is for musculoskeletal pain, stiffness or swelling, weakness, fever, rashes, and fatigue. This chapter describes both the nature of those symptoms helpful in making a diagnosis and also the subjective elements influenced by exertion, loss of function, anxiety, cultural norms, and other symptoms. In children and adolescents, symptoms are also influenced by developmental stage and may present simply as a limp or loss of limb function. Other referrals are for symptom complexes (such as Raynaud’s disease) or isolated abnormal investigations (such as elevated erythrocyte sedimentation rate or antinuclear antibody). Both paediatric and adult assessment requires the effective use of the (p)GALS screening examination tool described here. The nature of symptoms requiring rheumatological assessment varies according to diagnosis and has a subjective element influenced by exertion, impact, anxiety, cultural norms, and associated symptoms. They include musculoskeletal pain and stiffness, weakness or loss of limb function, fever, and also fatigue. Other referrals require assessment of symptom complexes or isolated abnormal investigations.

This chapter describes large, medium, and small vessel primary vasculitides. The 2012 Chapel Hill Consensus Conference nomenclature of vasculitis is introduced, and acts as a framework for more detailed descriptions of polymyalgia rheumatic and giant cell arteritis, polyarteritis nodosa (systemic and cutaneous), granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Takayasu arteritis, childhood-onset vasculitis, Kawasaki disease, Henoch–Schönlein purpura, and leucocytoclastic vasculitis. Other antineutrophil cytoplasmic antibody-associated vasculitides and primary angiitis of central nervous system in children are also covered. The epidemiology, aetiopathogenesis, classification criteria, presenting features, clinical manifestations, and management of these diseases are presented. In particular, diagnostic criteria for and mimics of polymyalgia rheumatic and giant cell arteritis are discussed in detail to permit rheumatologists and non-rheumatologists to confidently manage them.

The Oxford Handbook of Rheumatology, 4th edition, includes a chapter on spondyloarthritis (SpA) conditions. These conditions are axial spondyloarthritis, including ankylosing spondylitis, psoriatic arthritis, reactive arthritis, and inflammatory bowel disease-related arthritis. It summarizes evidence for pathogenetic mechanisms either common to all conditions, or specific for each condition, highlights current disease classifications, and emphasizes clinical features common to all SpAs, such as inflammatory back pain and enthesitis. There is an expanded section on treatments including biologic disease-modifying antirheumatic drugs (bDMARDs; ‘biologics’) such as anti-tumour necrosis factor-α‎, ustekinumab, and secukinumab, and new to this edition of the Handbook, an expanded section on juvenile spondyloarthritis.

The Oxford Handbook of Rheumatology, 4th edition, chapter on ‘Spinal disorders and back pain’ brings together a pragmatic approach to categorizing back pain, how to investigate disorders presenting with back pain, and how to treat adults, children, and adolescents with back pain. In the chapter, the management of back pain is separated into acute, subacute, and chronic presenting scenarios and the text makes reference to other chapters in the book where there may be some additional useful information (e.g. spondyloarthritis-associated back pain in Chapter 8 and chronic pain syndromes in Chapter 22). Notably the chapter is best read in conjunction with relevant sections in Chapter 3 (‘Regional musculoskeletal symptoms: making a working diagnosis’). The chapter makes reference to the need for a rigorous diagnostic approach to all the conditions which cause back pain, and at whatever age. There are subsections on how to assess paediatric spinal disorders and how to take a broad holistic approach to managing chronic back pain where there is no specific diagnosis.

The Oxford Handbook of Rheumatology, 4th edition, includes a chapter on metabolic bone diseases with an extensive review of the modern management of all forms of osteoporosis and fracture risk assessment including glucocorticoid-induced osteoporosis, idiopathic juvenile osteoporosis, and DXA scanning. The chapter outlines the salient features of other less common and occasionally encountered bone diseases found in rheumatological practice including vitamin D deficiency, osteomalacia and rickets, hypercalcaemia, parathyroid disease, Paget’s disease of bone, SAPHO syndrome, osteochondroses, osteonecrosis, fibrous dysplasia, sclerosing bone dysplasias, and bone tumours. New to this Handbook edition is a section on the main childhood autoinflammatory conditions manifest as skeletal disease under the umbrella term chronic non-bacterial osteomyelitis.

This chapter covers commonly encountered rheumatological emergencies, including septic arthritis (for both adults and paediatrics), infections in patients taking biologic therapy, acute systemic lupus erythematosus (SLE), systemic vasculitis, systemic sclerosis-related crises, methotrexate-induced pneumonitis, macrophage activation syndrome (MAS), and paediatric osteomyelitis. Malignancies presenting with musculoskeletal symptoms in children are also covered. Several of these topics are covered in more detail in other chapters of this handbook, with links provided from this chapter as appropriate.

Infectious agents have been linked directly and indirectly to a number of acute and chronic inflammatory rheumatological diseases. This chapter introduces some examples of the organism-specific and autoimmune responses leading to rheumatic disease. The epidemiology, aetiopathogenesis, classification criteria, clinical presentation, and management of septic arthritis (pyogenic non-gonococcal arthritis and gonococcal arthritis), Mycobacterium tuberculosis arthritis, osteomyelitis, Lyme disease, rheumatic fever, septic bursitis, and atypical mycobacterial infection are presented.

This chapter provides a systematic approach to rare autoimmune and autoinflammatory or monogenetic genetic conditions. These conditions typically present with characteristic fevers such as familial Mediterranean fever, TRAPS, cryopyrin-associated periodic syndromes, and adult-onset Still’s disease; characteristic skin manifestations such as panniculitis, neutrophilic dermatoses, and eosinophilic fasciitis; or with refractory synovial disease or polychondritis. Rare multisystem conditions of unknown aetiology including sarcoidosis, Behçet’s disease, amyloidosis, and immunoglobulin G4-related disease are described in terms of organ or system involvement.

The Oxford Handbook of Rheumatology, 4th edition, includes a chapter on glucocorticoid (‘steroid’) injection therapy. Soft tissue, tendon sheath, tendon insertion (enthesis), and joint injections are commonly carried out by rheumatologists in outpatient clinics. The chapter outlines the principles of injection techniques referring to the utility of both image-guided and non-imaging-guided injection procedures and cross-refers to line drawings of joint and tendons elsewhere in the book. The chapter focuses also on the use of injections in the context of managing specific disorders such as tennis elbow, plantar fasciitis, carpal tunnel syndrome, and greater trochanter pain syndrome, thus placing injection therapy in the context of alternatively available therapies.

The Oxford Handbook of Rheumatology, 4th edition, emphasizes the importance of evidence-based therapeutics and therefore devotes a whole chapter to medications used to treat rheumatological and bone disease (‘Drugs used in rheumatological disease’). Details about treatments are cross-referenced to specific use of medications for specific diseases and conditions elsewhere in the book. There are chapter sections on analgesics including opiate-based medications, NSAIDs, conventional synthetic disease-modifying antirheumatic drugs (DMARDs), biologic DMARDs, intravenous immunoglobulin, and other immunosuppressants. There is a specific focus in the chapter on drugs used for Raynaud’s disease, osteoporosis (e.g. bisphosphonates), pulmonary hypertension, gout, and hyperuricaemia. Overall there is reference to both adult and paediatric disease treatments in the chapter.

This chapter describes the presenting features, clinical manifestations, and management of rheumatoid arthritis. Commonly used investigations, their indication and interpretation, as well as the holistic treatment of rheumatoid arthritis are outlined. A summary of the commonly used drugs for the treatment of rheumatoid arthritis, their mechanism of action, and key points for starting and monitoring their use is included. Special circumstances including the management of rheumatoid arthritis in pregnancy, surgery, vaccinations, and during infections are also discussed.

This chapter focuses on systemic sclerosis, a generalized autoimmune connective tissue disease. It describes the epidemiology and classification of systemic sclerosis, along with its pathophysiology and potential triggers of disease. It also discusses the condition’s broad spectrum of clinical manifestations and highlights its potential for multiorgan involvement and life-threatening complications. Furthermore, the chapter provides insights into the definitions for related conditions such as morphoea and its different subtypes. Complexities around the treatment and a discussion on the prognosis and outcomes of systemic sclerosis and related conditions are also included.

The key to a rheumatological diagnosis and formulation of an effective management plan is a structured history and thorough examination that assesses the extent or impact of disease, screens for asymptomatic components, and tests for any red flags and incongruence. This, in turn, focuses the use of investigations, which alone rarely provide a diagnosis. Our structured approach aims to identify (1) those conditions not be missed; (2) normal variants; (3) inflammatory and non-inflammatory causes of pain, including chronic pain; (4) mono/oligo articular diagnoses; (5) multi/polyarticular or widespread presentations; and (6) diagnoses with systemic features. Assessment of the child and adolescent acknowledges the impact of musculoskeletal development on presentation and a parental-derived history. All assessment includes evaluation of infectious causes of musculoskeletal pain, gait, limp, muscle weakness and polysymptomatology.

This chapter describes crystal-induced musculoskeletal disease, which encompasses gout, hyperuricaemia, calcium pyrophosphate dihydrate disease, basic calcium phosphate-associated disease, and calcium oxalate arthritis. Their epidemiology, aetiopathogenesis, classification criteria, presenting features, clinical manifestations, and management are discussed. Diet, lifestyle, non-pharmacological and pharmacological approaches to their management are considered. User-friendly algorithms for the management of gout and calcium pyrophosphate dihydrate disease are provided, allowing both rheumatologists and non-rheumatologists to confidently manage these diseases in clinic, ward, or community settings.

The presentation and natural history of juvenile idiopathic arthritis (JIA) differs from that of the adult inflammatory arthritides and this chapter describes the key features and differentials that need to be considered. In making a diagnosis, this chapter should be used in association with Part I of the Handbook. The chapter conveys information useful for explaining JIA to parents, national guidelines and other routine therapeutic practices, analysis of subtypes of JIA, and uveitis commonly associated with JIA. The description of systemic-onset JIA includes its differential and association with macrophage activation syndrome. As an archetypal long-term musculoskeletal condition, the key features of transition and age-appropriate care are also discussed here.