Classification of Chronic Pain Syndromes

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While chronic pain and pain-associated disability can complicate any chronic illness, there are certain pain syndromes that are recognized in children. These have been mentioned previously.

Diffuse Idiopathic Pain Syndromes (Juvenile Fibromyalgia)

The onset of pain in diffuse idiopathic pain (DIP) syndromes (47) is often gradual. There may have been an initial insult (infection) or hypermobility (22), but there is often no obvious trigger and vague recollections of the time of onset. The pain, as suggested, is generalized. There may be areas of allodynia, but there is often an absence of the autonomic changes that we see in more localized pain conditions (2). What is striking in the young people with diffuse pain is the fatigue, poor sleep pattern, and extremely low mood (48). It is widely believed, however, that the low moods in adolescents are reactive (to the pain-associated disability) rather than a primary depression. This is in contrast to adults with fibromyalga, where primary depression is frequently seen. This distinction is important when looking at rehabilitation and prognosis in these two populations (49). There are no DIP criteria for adolescents so the American College of Rheumatology criteria for adult fibromyalgia are still referred to. This has to be used with a little caution (48), not only because the outcome in adolescents is so much better but also there has been no definitive work looking specifically at tender points on children.

Localized Idiopathic Pain Syndrome, Including Complex

Regional Pain Syndromes

Localized idiopathic pain syndrome simply describes pain that remains in a localized area (such as a limb). Within this descriptive diagnostic group are the complex regional pain syndromes (CRPS 1 or reflex sympathetic dystrophy).

The diagnosis of CRPS 1 remains a clinical one. There is often a precipitating trauma (not always). The pain should be out of proportion to the inciting event and usually accompanied by allodynia. Autonomic changes are present; these include swelling, reduced cutaneous perfusion, and thermodynamic instability. There is also a marked reduction in range of movement and, in severe cases, trophic changes. In adolescents the legs are more commonly affected. The International Association for the Study of Pain (IASP) has diagnostic criteria for adults with CRPS (Table 1). Although these diagnostic criteria hold true for children and adolescents, it is widely believed that the dystrophic changes and long term disability are less common when compared with adults.

Occasionally more than one limb may be affected at presentation. It is not unusual for a hand or other leg to develop CRPS months after a leg has been affected. This may be due to the use of crutches and subsequent pain amplification but may also have no obvious trigger. Young people with CRPS may also develop low mood and overwhelming fatigue. This further complicates the clinical picture.

Table 1 International Association for the Study of Pain (IASP) Diagnostic Criteria for Complex Regional Pain Syndrome

Presence of an initiating noxious event, or cause of immobilization Continuing pain, allodynia, or hyperalgesia with which the pain is disproportionate to any inciting event

Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of the pain This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction Type 1: Without evidence of major nerve damage Type II: With evidence of major nerve damage

Source: Adapted from Ref. 78.

Although the emphasis in this text is on pain syndromes, it must not be forgotten that chronic pain can complicate any other musculoskeletal diseases, including JIA, muscular dystrophies, and dysplasias.

If the chronic pain condition is not recognized and treated, many of these adolescents develop associated disabilities that continue into adult life. They are unable to continue education; peer relationships fall away; family life remains disrupted/dysfunctional; and physical and emotional well-being is lost. Chronic fatigue and secondary depression become as disabling as the pain.

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