RENEW OR SUBSCRIBE TO PPM
Subscription is FREE for qualified healthcare professionals in the US.
13 Articles in Volume 11, Issue #7
Fibromyalgia: Practical Approaches To Diagnosis and Treatment
Juvenile Fibromyalgia: Diagnostic Challenges and Treatment Options
Aqua Therapy Helpful in Treatment Of Systemic Lupus Erythematosus
Axial Neck Pain, Radiculopathy, and Myelopathy: Recognition and Treatment
Early Treatment of TMD May Prevent Chronic Pain and Disability
Identifying Psychological Factors That Influence Surgical Outcomes
Managing Morton’s Entrapment
Premedicated Mask May Hold Promise for Migraine Patients
Mother With Low Back Pain
The Hip Replacement Patient
Evidence-based Medicine: Losing the Patient’s Voice?
What Is Going Wrong With Research?
Risk for Sedation and Car Accidents

Juvenile Fibromyalgia: Diagnostic Challenges and Treatment Options

Early recognition of symptoms, awareness, and education, including training in pain-coping skills along with targeted treatments to improve sleep, mood, and physical activity, are crucial to improve quality of life in children with fibromyalgia.

Juvenile fibromyalgia (JFM) is a chronic condition of widespread musculoskeletal pain, fatigue, and nonrestorative sleep. The prevalence of JFM among school-aged children is approximately 1% to 6%.1,2 As is the case with adults, JFM appears to occur more commonly in females. JFM typically affects adolescents, but it can occur even in younger children.

This condition can be frustrating and often difficult to recognize and manage for everyone involved—from patients and families to physicians. Patients with JFM often present with additional symptoms, such as chronic daily headaches, irritable bowel syndrome (IBS), recurrent abdominal pain, dysmenorrhea, and associated mood disorders of anxiety and/or depression. In fact, the average time to diagnosis is approximately two years.3,4 Evidence-based pharmacologic treatment for JFM has been limited, although clinical drug trials are ongoing. Current therapies are multidisciplinary. To date, outcomes of JFM remain uncertain.

Pathogenesis
Although the true cause of JFM is still unknown, there are multiple theories that seek to explain the potential pathophysiology of fibromyalgia (FM). These proposed explanations are centered on the common observation that patients suffering from FM have an overall heightened sensitivity to pain. In 1965, Melzack and Wall5 described the gate control theory of pain, in which a “gate” monitors pain signals to and from the brain.5 In susceptible individuals, such as those with FM, pain sensations are heightened because the gate remains wide open, even to minimal stimuli. The major theory at present is based on a phenomenon known as central sensitization, whereby patients have a lower threshold for pain because of an overall increased responsiveness to pain signals within the brain.6 It is believed that a defect of the descending inhibitory pathway prevents normal inhibitory responses, such that signals from even mildly noxious and non-noxious stimuli trigger excessive and persistent pain. Similarly, hyperalgesia and allodynia, at times features of JFM, are explained by this central pathway theory.

Many patients with chronic pain suffer from a type of “memory” for painful stimuli, in which recurrent pain is rapidly and increasingly interpreted as severe pain. This concept is known as temporal summation or wind-up7 and is felt to involve C-fiber–evoked responses of the dorsal horn. Other proposed theories include the possibility of hormonal imbalances of insulin-like growth factor 1 (IGF-1) and growth hormone,8,9 substance P,10 and neurotransmitters,11,12 particularly serotonin and norepinephrine receptors, which have been the target of many of the newer medications.

Infectious Trigger?
Other researchers propose that an infectious trigger causes FM. Viral illnesses like mononucleosis often have been identified at the onset of a patient’s symptoms—although no clear link has been discovered. It is much likelier that the virus was a trigger for altering one’s lifestyle and the subsequent vicious cycle, but is not necessarily the underlying cause. Recent reports in 2009 suggested that a retrovirus known as XMRV (myalgic encephalitis virus) was linked to patients with chronic fatigue syndrome (CFS)13; however, more recent studies do not support this link.14,15

Genetic Link
It has been found that there is a strong familial clustering of FM symptoms among family members, especially female relatives. Arnold et al found that the odds of developing FM in an adult relative of a person with FM vs. the odds of FM in a relative with rheumatoid arthritis (RA) was 8.5 (95% confidence interval [CI], 2.8-26, P = 0.0002).16 These findings might indicate the real possibility that susceptibility to chronic pain or heightened pain sensitivity is an inherited trait, although specific genetic links have yet to be identified.

Diagnosis
A thorough history and review of symptoms is used to guide the diagnosis of JFM and rule out other possible medical conditions with similar symptom presentation (see Table). According to the Yunus and Masi classification criteria, symptoms of widespread musculoskeletal pain must be present for at least three months, and patients must have five or more tender points on examination.17 Exclusion of other medical conditions and otherwise normal laboratory tests also are necessary to classify a patient as having JFM. Other associated symptoms include fatigue, IBS, headaches, poor sleep, anxiety, numbness, subjective soft tissue swelling, and modulation of symptoms due to anxiety or stress, weather changes, and/or physical activity. Often, symptoms are preceded by a history of physical injury or acute illness that leads to a disruption in daily life. Subsequently, symptoms of pain, poor sleep, and fatigue arise and continue on a vicious cycle, leaving patients with persistent pain, altered mood, and poor functioning.

Upon examination, one would not expect to find abnormalities other than pain at sites of tender point locations (see Figure 1). These sites have been suggested to have greater pain sensitivity in patients with FM,18 and among children, the presence of five of 18 tender points is suggestive of JFM.

Most pediatric rheumatologists use the Yunus and Masi criteria; however, the 1990 American College of Rheumatology (ACR) criteria for FM also have been used for classification purposes.19 The ACR criteria are more specific to musculoskeletal pain, requiring widespread pain in at least three different locations for at least three months along with a total of 11 of 18 tender points. The newer, recently endorsed 2010 ACR criteria for FM evaluate widespread pain and symptom severity without the need for a tender point exam by a trained clinician.20 They have yet to be validated in a pediatric population.

Differential Diagnoses
As noted, patients with JFM often suffer from associated conditions like chronic migraines or headaches, chronic abdominal pain, IBS, restless leg syndrome, sleep disorders like narcolepsy and sleep apnea, postural orthostatic tachycardia syndrome, CFS, and mood and anxiety disorders. Differential diagnosis involves ruling out other illnesses, including thyroid disease and autoimmune diseases like chronic juvenile idiopathic arthritis, systemic lupus erythematosus, and juvenile dermatomyositis, which should be considered if symptoms are highly suggestive or if there is a strong family history of thyroid disease or autoimmunity.

As mentioned previously, viral symptoms often precede or are present at onset of JFM, but typically patients readily recover from these illnesses while symptoms of JFM persist. Some viral illness, including Epstein-Barr virus, cytomegalovirus, and HIV, could mimic certain features of JFM, but the latter is likely to present with other worrisome findings of fever, lymphadenopathy, anorexia, and weight loss. Generalized anxiety and depression or other mood disorders should be screened for, and patients should be referred for psychiatric evaluation and treatment if indicated.

Laboratory and Radiologic Testing
Depending on the constellation of symptoms present, basic laboratory testing may be helpful, primarily to exclude other diagnoses. Laboratory tests should include a complete blood count with differential, erythrocyte sedimentation rate, complete metabolic panel, thyroid studies, and perhaps an antinuclear antibody (ANA) if any additional symptoms such as alopecia, photosensitive rash, arthritis, ulcerations, and/or presence of color changes of the fingertips and toes (Raynaud’s phenomenon) are present. In JFM, laboratory tests should be normal. Keep in mind that low-titer ANA results could be false-positives and do not necessarily suggest an underlying rheumatologic condition. Consultation with a rheumatologist may be helpful if there are any abnormal symptoms or laboratory tests that cannot be explained by other conditions.

There is little role for imaging studies in the diagnosis of JFM; however, if a clinician has a high degree of suspicion, then imaging should be used to rule out other conditions. Unfortunately, patients with FM frequently undergo multiple, expensive tests that often are unnecessary. Therefore, careful follow-up with patients to ensure that excessive testing does not occur is important.

Treatment
Although the initial thought would be to directly treat the musculoskeletal pain, analgesic medications are typically ineffective in the treatment of JFM.21 Generally, treatment for JFM is threefold and aimed at improving sleep; increasing physical activity and endurance; and providing coping skills for patients. Treatment is therefore multidisciplinary in nature (see Figure 2).

Medications
As noted above, very few medications have been proven efficacious in the treatment of JFM.22,23 However, in recent years, three medications have been approved for adult FM, including the serotonin norepinephrine reuptake inhibitors (SNRIs) duloxetine (Cymbalta)24 and milnacipran (Savella),25 and the γ-aminobutyric acid (GABA)–mediated drug pregabalin (Lyrica).26 Clinical trials across the United States are ongoing to determine both the efficacy and safety of these medications in the treatment of JFM.

Specific pain medications have not been studied in JFM and historically have not been shown to provide benefit. Tricyclic antidepressants like amitriptyline are used in low doses in clinical practice but are equivocal in efficacy and have not been studied in the treatment of JFM. Nonsteroidal anti-inflammatory agents may be used in the setting of acute pain but have little role in the treatment of the chronic pain of JFM. Other classes of medications that have been used include sleep aids, tramadol, and muscle relaxants. Topical analgesics like capsaicin may be beneficial for patients with hyperalgesia or allodynia for desensitization and relaxation. There is no evidence for the use of narcotics or antibiotics in the treatment of JFM.

Physical Therapies
Early evidence suggests benefit with targeted aerobic physical activity programs for the improvement of symptoms, quality of life, and functional disability in JFM.27 In fact, the pain gate control theory describes the phenomenon that routine physical activity or non-noxious stimuli could slowly help to close the pain gate, allowing for an overall decrease in widespread pain and fatigue. Directed physical and occupational therapy may be helpful in providing additional resources for guidance on targeted exercise programs for patients with JFM. Transcutaneous electrical nerve stimulation treatment may provide both desensitization and muscle relaxation and can be performed at home; however, limited data exist pertaining to physical treatments and their benefits in JFM.

Coping Skills
Cognitive-behavioral therapy (CBT) has been identified as an effective method for improving functional disability and quality of life among patients with JFM in a randomized controlled trial.28 The basis of CBT is to improve coping skills for pain triggers, including guided imagery, deep breathing techniques, and distraction. In addition to providing coping skills, the addition of trained psychologists and counselors is often critical to the treatment plan of JFM patients, particularly if mood disorders of anxiety and/or depression are contributory.

Alternative Therapies
A large number of pediatric patients with chronic pain use complementary and alternative medicine (CAM) therapies, including biofeedback, yoga, massage, acupuncture, vitamin and/or herbal supplements, art therapy, and craniosacral therapy.29 Although there are few studies assessing the use of CAM for the treatment of JFM, patients and families have indicated benefit and a strong interest in pursuing its use.

Outcomes
Longitudinal data on patients with JFM are limited. Studies generally indicate variable prognoses ranging from complete remission to persistence of symptoms into adulthood.30-32 It has been shown that patients with JFM suffer from social isolation from peers,33 increased missed school days,34 poorer overall family functioning,35 lower quality of life, and increased mood-related disorders and internalizing.35-37 With such potential for negative outcomes on adolescents’ lives, it remains extremely important to recognize, diagnose, and implement strategies early on with the hope of preventing worse outcomes and potentially changing the long-term trajectory of the condition.

Our longitudinal follow-up study of a cohort of patients with JFM followed in tertiary care centers by pediatric rheumatologists indicated that a majority (approximately 60%) had persistent symptoms of widespread pain and fatigue, whereas 20% were symptom-free at approximately 4-year follow-up.32 By contrast, longitudinal studies of community populations of youth with JFM were likelier to find generalized improvement (up to 73%) over time.30,31 Indeed, there are likely to be individual differences in long-term outcomes, and more work is needed to determine the varying trajectories among children and adolescents diagnosed with JFM.

Monitoring
Although making a diagnosis can be rather difficult, referrals to specialists like pediatric rheumatologists, neurologists, and pain physicians can be helpful in establishing the presence of JFM and setting goals and expectations for patients. However, the majority of patients with JFM are likely best followed and monitored long term by their primary care physicians, who often have built and established good rapport and are most aware of local resources. Others familiar with treating JFM, including pain specialists, pediatric pain psychologists, and physical and occupational therapists, are critical members of the multidisciplinary treatment team and can be of substantial benefit to patients. Those patients with refractory symptoms are best followed by a multidisciplinary pain team when one is available. Although there is no cure for JFM at this time, it is most definitely a manageable condition, and patients should be reassured that it is possible to lead a relatively normal lifestyle with the right healthcare team in place. 

Conclusion
There is a growing recognition that FM can occur in children and adolescents, and that it can be a very distressing, chronic condition for patients and families to deal with. Although JFM affects only a small proportion of children and adolescents, it can have significant functional and psychosocial implications. Indeed, the potential for negative outcomes can be great, yet there are limited evidence-based treatment options. Future studies certainly are warranted, and ongoing clinical drug trials may prove to be beneficial. Early recognition of symptoms, awareness, and education, including training in pain-coping skills along with targeted treatments to improve sleep, mood, and physical activity, are crucial.

Last updated on: November 1, 2011
close X
SHOW MAIN MENU
SHOW SUB MENU