The existence of systemic autoimmune diseases not fulfilling classification criteria for defined connective tissue diseases (CTDs) is a common clinical experience. These conditions have been variably defined as incomplete lupus erythematosus, early undifferentiated connective tissue diseases and undifferentiated connective tissue diseases (UCTDs). However, the definition of UCTD includes a wide spectrum of diseases ranging from 'organ-dominant' conditions (e.g., idhiopatic non-specific interstitial pneumonia) to simplified conditions (stable UCTD), to early CTDs or mild forms of CTDs. In the present article, the literature data on undifferentiated diseases and their clinical spectrum as well as the importance of the definition of new classificative criteria are discussed.
Tumours, infections, aneurysms and metabolic and inflammatory diseases are rare causes of neck pain. Most cases involve neck pain of unknown origin or a whiplash-associated disorder. Neck pain is common in the general community and more common in certain occupations. The natural history is relatively benign, but some 10% of patients will suffer chronic, severe symptoms. Psychosocial factors have been refuted as risk factors; the cardinal risk factors relate to occupation. In whiplash, the severity of initial symptoms is the cardinal determinant of chronicity. History is the major factor when considering diagnosis, physical examination adding little to the diagnosis. Imaging is not indicated in the vast majority of cases. The available evidence does not support most of the physical, medical and surgical therapies currently practised. Confident reassurance is paramount and justified for acute cases. Proven options for chronic neck pain are few.
Pain is a subjective experience that is unique to the individual. Although pain is usually associated with tissue injury or inflammation, it is strongly influenced by changes within the nervous system as well as by psychological and social factors. This review focuses on those 'nociceptive' mechanisms which contribute to pain and demonstates how an appreciation of underlying molecular and cellular mechanisms can lead to improved diagnosis and treatment.
Pain is a subjective experience that is unique to the individual. Although pain is usually associated with tissue injury or inflammation, it is strongly influenced by changes within the nervous system as well as by psychological and social factors. This review focuses on those 'nociceptive' mechanisms which contribute to pain and demonstates how an appreciation of underlying molecular and cellular mechanisms can lead to improved diagnosis and treatment.
Two main groups of soft-tissue disorders are identified: specific soft tissue syndromes and a non-specific disorder as yet not fully characterized. Specific soft tissue syndromes occur in joint, muscle or nerve and are associated with characteristic symptoms and physical signs. These include epicondylitis at the elbow, tendon disorders at the wrist and nerve entrapments such as carpal tunnel syndrome. The non-specific soft tissue syndrome is primarily described by symptoms of pain but may include muscular symptoms (such as weakness and cramp) or nerve symptoms (such as numbness, pins and needles, and burning). Agreed, validated diagnostic criteria for use in surveillance are urgently required. Biological markers are still sought; both muscle and nerve seem promising for future studies. The multifactorial aetiology of these disorders will be discussed, and guidelines to management will embrace the principles of this model. Treatments aimed at the pain in isolation are most often studied. Psychosocial and environmental influences on the presentation and persistence of pain need further evaluation.
Decisions about the best treatment options for patients with regional musculoskeletal pain must be made despite a lack of clear diagnostic criteria, an absence of robust outcome measures by which to assess response, and a paucity of evidence from high-quality randomized controlled trials. Although the randomized controlled trial is considered to be the 'gold standard' research design, it does not lend itself easily to the evaluation of all management strategies. This chapter explores these issues from a research perspective and reviews the various types of evidence available to help practitioners make informed decisions. The current evidence from systematic reviews in this area is summarized in the final part of the chapter.
There is a general lack of new evidence on which to base practical and useful diagnostic classifications of regional musculoskeletal pain for primary care. However, the ‘red flag’ system, developed and now disseminated successfully in the management of low back pain, seems to be applicable to the initial management of other regional pain. In this scheme, signs and symptoms of serious disease are sought, and their presence is the indication for the investigation or referral of individual patients. In their absence, the low predictive value of many signs, tests and even diagnostic labels argues against their routine use in primary care. Simple decisions based on separation into articular versus non-articular, and acute versus chronic, are favoured by the most recent expert consensus statements. In addition, the presence or absence of more widespread pain, and of other non-specific features of chronic pain, is likely to be helpful in managing regional syndromes. Prospective studies of the prognostic value of symptoms, signs and diagnostic labels remain a research priority in this field.
Regional musculoskeletal disorders are a major cause of morbidity both in the community and in the workplace. They comprise a heterogeneous group of conditions that are, for the most part, poorly characterized. Consequently, agreed diagnostic criteria have not existed for many of these disorders, and epidemiological investigations have used varied or ill-defined approaches to case definition. This chapter describes our current understanding of the epidemiology of regional pain disorders and details the strengths and weaknesses of the available data. Pain syndromes can be divided anatomically into those which cause generalized pain, such as fibromyalgia syndrome and myofascial pain syndromes, and those which are confined to one regional anatomical area. The latter group comprise those of the neck, shoulder, elbow, wrist/hand, hip, knee and ankle/foot. Current information is considered on the known risk factors for disorders at these sites, in addition to their impact upon both the individual and society.
This chapter deals with four main questions: what is the evidence that 'myofascial pain' syndromes exist?; what is the evidence that the myofascial pain concept is clinically useful?; what is the evidence that managing patients in terms of the myofascial pain diagnosis confers benefits?; and what is the evidence-based management of myofascial pain! The purpose of a diagnosis is to provide boundaries around subgroups of illness in a population since each subgroup presumably has a different mechanism, natural history, prognosis, course and response to treatment. The current literature is divided in its conceptual approach to the problem of regional musculoskeletal pain. Some authors regard myofascial pain as being distinct from regional musculoskeletal pain while others regard these as synonymous. A postulated theory of the pathophysiology of myofascial pain is discussed. This contrasts with a view that regional myofascial pain represents a non-specific localized pain arising from multiple regional, systemic and psychosocial factors. In order to consider myofascial pain as a distinct diagnosis, it would be necessary to resolve reliability issues in the identification of its critical diagnostic features. Beyond reliability issues, there are also problems of sensitivity and specificity-i.e. of the patient population that it identifies-which must be resolved if controlled trials are to be conducted. The clinical usefulness of the myofascial pain diagnosis is considered with regard to what is believed about the course of healing, the determinants of disability, the course of regional versus widespread musculoskeletal pain, the relationship of musculoskeletal injury to pain, and the evidence-based management of musculoskeletal pain. An epidemiological perspective is proposed with regard to regional musculoskeletal pain. This allows for the identification of operationally defined strata of regional musculoskeletal pain and permits studies in course, prognosis and treatment, even though some conceptual issues such as the 'myofascial pain diagnosis' remain to be clarified.
This paper provides an up-to-date overview of the occurrence, diagnosis, risk factors, prognostic indicators and outcome of shoulder disorder (SD), and of the validity and reproducibility of diagnostic classifications and diagnostic imaging techniques for SD. Furthermore, the available evidence on the effectiveness of non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroid injections and physiotherapy for SD is summarized on the basis of randomized controlled trials with an acceptable quality of their methods. The annual incidence of SD is estimated at about 7%, its 1-year period prevalence at about 51% and its lifetime prevalence at about 10%. While approximately 50% of all patients with SD seek medical care, about 95% are treated in primary health care. Of all new episodes of SD presenting to primary care, approximately 50% seem to resolve within 6 months, while about 40% seem to persist for up to 12 months. Several prognostic indicators for a favourable or a poor outcome of SD have been identified, but a comprehensive prognostic model is not available. While evidence for the prognostic validity of popular diagnostic classifications of SD is lacking, their reproducibility has been shown to be poor. The accuracy and clinical usefulness of diagnostic imaging techniques appear to be sufficiently verified for SD in secondary care, while their clinical usefulness in primary care and prognostic validity are not. NSAIDs and steroid injections for SD have been shown to be effective within 6 weeks, but their effect on long-term outcome remains unclear. There is very limited evidence for the effectiveness in SD of physiotherapy, including exercise therapy, ultrasound, electrotherapy, laser, mobilization and manipulation.
Chronic knee pain is common at all ages, particularly in the elderly, among whom it has its greatest impact. Chronic knee pain is often ascribed to osteoarthritis in adults and to chondromalacia patellae in children and adolescents. Pathological findings in both these conditions correlate poorly, however, with the severity of knee pain and disability. Psychometric variables correlate better with the impact of knee osteoarthritis, suggesting that this disorder has characteristics of a regional pain syndrome. This perception may reflect our lack of understanding of the biological mechanisms in these disorders. This possibility has been highlighted by the advent of magnetic resonance imaging, and by recent studies of muscle function, reflex quadriceps inhibition and proprioception in people with knee osteoarthritis. Established risk factors for knee osteoarthritis include increased body weight, knee injury and aspects of occupational activity. Recent studies have also suggested a possible role for oestrogens and vitamins C and D in the secondary prevention of this disorder. The emergence of 'nutraceuticals' such as glucosamine as treatments for osteoarthritis has captured the public imagination and merits further study.
Tumours, infections, aneurysms and metabolic and inflammatory diseases are rare causes of neck pain. Most cases involve neck pain of unknown origin or a whiplash-associated disorder. Neck pain is common in the general community and more common in certain occupations. The natural history is relatively benign, but some 10% of patients will suffer chronic, severe symptoms. Psychosocial factors have been refuted as risk factors; the cardinal risk factors relate to occupation. In whiplash, the severity of initial symptoms is the cardinal determinant of chronicity. History is the major factor when considering diagnosis, physical examination adding little to the diagnosis. Imaging is not indicated in the vast majority of cases. The available evidence does not support most of the physical, medical and surgical therapies currently practised. Confident reassurance is paramount and justified for acute cases. Proven options for chronic neck pain are few.
The seronegative spondyloarthropathies appear to be the genetically predisposed host's clinical expression to acute, subacute or chronic reaction to the invasion by environmental microorganisms. In the ensuing days or weeks, depending on the infectious load, clinical manifestations may occur ranging from constitutional complaints such as fever, to a variety of symptoms and/or signs related to the portal of entry—intestinal, genitourinary or respiratory. Within weeks or months, the initial or other target organs, such as the mucocutaneous, ocular and cardiovascular systems, may develop an acute reaction of greater or lesser specificity regarding the triggering agent (oral ulcers, circinate balanitis, erythema nodosum, acute anterior uveitis, pericarditis, heart blocks). Lastly, many years later, a minority of patients, probably those with a large genetic component, exhibit a spectrum of clinical manifestations related to those organs, with a chronic or recurrent course. Acute clinical manifestations—reactive arthritis—are prominent in the initial phase of the clinical spectrum, while chronic manifestations—ankylosing spondylitis—are seen at the other end of the spectrum.
Chronic childhood arthritis impairs joint function and may result in severe physical handicap. Joint pain and inflammation trigger a vicious cycle that often ends in joint damage and fixed deformities. A comprehensive rehabilitation programme must start early to restore loss of function and prevent permanent handicap. It is dominated by a physiotherapeutic regimen consisting of pain relief, movement expansion, training of muscular coordination and finally re-integration of a physiological movement pattern. The approaches of occupational therapy become integrated into the treatment programme, concentrating on joint protection and self-care training. Additional aids support the aim of joint restoration. They include individual splinting, adapted footwear and walking aids. Depending on the child's age and developmental status different aspects of rehabilitation dominate. Small children need adequate mobility to promote their psychosocial development. In later years integration into school life and the peer group becomes important. Adolescents require help for an adequate vocational training and self-care support. Last but not least, parental education and integration of the whole family into the rehabilitation programme markedly improve the patient's prognosis.
Among several animal models, HLA-B27 transgenic rodents proved useful for investigating the interplay between genetic factors and the bacterial environment in the aetiopathogenesis of the spondyloarthropathies (SpA). HLA-B27 transgenic rats spontaneously develop a multisystemic inflammatory disease resembling human SpA. This disease is dependent on the presence of a normal bacterial flora and implicates the immune system. The presence of both T cells and antigen-presenting cells expressing high levels of HLA-B27 seems of critical importance in its pathogenesis. HLA-B27 transgenic mice also develop arthritis, under the influence of the bacterial flora. In both types of model, CD8+ T cells seem not to be necessary, arguing against the ‘arthritogenic peptide’ hypothesis. In vitro models have been used to study the immune response against bacterial agents and the role of HLA-B27 in human SpA. It appears that an impaired immune response against bacteria could be involved in the triggering of human SpA. HLA-B27 could be implicated at the level of interaction between host cells and bacteria in the driving of a specific immune response against bacterial antigens or as a target of an autoimmune response.
Problem situations in the patient-health care relationship may relate to the patient or to the health care provider characteristics or to the way they interact; they may also relate to the general social context. Such situations force the clinician dealing with non-specific low back pain patients to look beyond the traditional biomedical model that assumes a linear connection between pathology and symptomatology. The introduction of the biopsychosocial model approximately 10 years ago has improved the understanding of common low back pain. This chapter gives some insight into areas relating to factors that may hamper the patient-therapist relationship and thus complicate treatment and recommendation outcomes. It emphasizes the necessity to involve the patient in the decision-making. Recognizing the patients' psychological, social and cultural background as well as the level of education and employability are important to make successful recommendations. This knowledge is not new but the difficulty is to implement it in today's cost effectiveness driven society. However the benefit at the end may be the decrease of chronicity and/or permanent disability, suffering for the patient and frustration for the clinician. Identifying the underlying cause of non-compliance or of unexpected delayed recovery is an exciting issue. The cause may or may not be biomedical. If a specific cause can be identified, it has to be diagnosed and evaluated. If the clinical examination has ruled out specific or emergency conditions, another perspective may be needed and the course of action could then be determined.
Enthesitis is a distinctive pathological feature of spondyloarthropathy and may involve synovial joints, cartilaginous joints, syndesmoses and extra-articular entheses. This review focuses on peripheral extra-articular enthesitis which is a clinical hallmark of spondyloarthropathy. The entheses of the lower limbs are more frequently involved than those of the upper limbs, and heel enthesitis is the most frequent. Entheseal pain may be mild or moderate as well as severe and disabling. Peripheral enthesitis may be observed in all forms of spondyloarthropathy, including the undifferentiated ones, and may for a long time be the only long-standing clinical manifestation of the B27-associated disease process. Various imaging methods have been suggested for studying peripheral enthesitis. Ultrasonography and magnetic resonance imaging are the most useful because they may show alterations of the structures involved. Therapy of peripheral enthesitis consists of NSAIDs, orthoses and physical therapy. Steroid injections, second line drugs such as sulphasalazine and radiotherapy are reserved for more severe cases.
Inflammatory joint disease can develop following an extra-articular infection. The term reactive arthritis was coined in order to differentiate this arthritis, which is often characterized by lack of culturable organisms in the joint, from septic arthritides. Bacteria known to trigger reactive arthritis include Campylobacter, Chlamydia, Salmonella, Shigella and Yersinia. Demonstration of bacteria or bacterial macromolecules in the joint has elicited the idea that reactive arthritis is a sterile process induced and maintained by antigenic material in the synovium. Continued synthesis of antigens to maintain synovial inflammation probably requires establishment of persistent bacterial infection in the joint, or at the primary site of infection. In the case of Chlamydia trachomatis, viable, metabolically-active organisms have been demonstrated to exist for extended periods in the joints of patients with reactive arthritis. In this chapter, we review the aetiological agents, and their molecular biology and phagocyte-host interactions, that are involved in reactive arthritis and spondylarthropathy.
