Juvenile Primary Fibromyalgia Syndrome 

Updated: Nov 14, 2018
Author: Eileen R Giardino, RN, MSN, PhD, ANP-BC, FNP-BC; Chief Editor: Lawrence K Jung, MD 

Overview

Background

Juvenile primary fibromyalgia syndrome (JPFS) is a musculoskeletal pain syndrome characterized by multiple discrete tender points (TPs), fatigue, and sleep disturbance in the child and adolescent. JPFS can be chronic in nature and can necessitate a multidisciplinary approach to the ongoing treatment of the disorder. The pain experienced is modulated by factors such as activity, anxiety, stress, and weather changes.

The diagnosis is made on the basis of the presence or absence of specific criteria that have been found to be consistent with those who experience JPFS (see Differentials). The chronic nature of fibromyalgia in the pediatric population suggests consequences for psychosocial development and implies a more difficult transition from childhood to adulthood.[1]

The core feature of fibromyalgia is widespread musculoskeletal pain, with multiple TPs and other associated features (eg, fatigue, joint stiffness, skin tenderness, postexertional pain, sleep disturbance, irritable bowel symptoms, poor memory, tension headaches, dizziness, fluid retention, paraesthesias, restless legs, bruising, and Raynaud phenomenon). Chronic musculoskeletal pain affects quality of life, whereas fatigability influences motor response and ability to complete activities of daily living within an expedient time frame.[1, 2]

The understanding of fibromyalgia syndrome (FMS) in children is still in its infancy stage; however, great strides have been made in both diagnosis and treatment in the past 10 years. Because the prevalence of FMS in children is increasing, diagnosing the disorder early in its course (see Clinical) and then recommending a multidisciplinary approach to treat the child’s disorder (see Treatment) are important. An approach that involves support for the family and specific recommendations for treatment may help decrease the symptomatology and increase the child’s functioning.[3]

Go to Fibromyalgia and Rehabilitation and Fibromyalgia for complete information on these topics.

Pathophysiology

FMS is a physiologic entity rather than a psychiatric disorder. Yunus proposes a class of disorders called central sensitivity syndromes (CSSs), which result from changes in the central nervous system (CNS).[4] Neurochemical pathology of the CNS (spinal cord and brain) causes areas of the body to become sensitized, so that pain is experienced from even mild touch or pressure.

Proposed members of the CSS family include the following[4, 5] :

  • Fibromyalgia syndrome

  • Chronic fatigue syndrome

  • Irritable bowel syndrome

  • T-T headache (tension type)

  • Migraine

  • Temporomandibular disorders

  • Myofascial pain syndrome

  • Female urethral syndrome/interstitial cystitis

  • Multiple chemical sensitivity syndrome

  • Restless leg syndrome

  • Periodic limb movements in sleep (PLMS)

  • Primary dysmenorrhea

  • Posttraumatic stress disorder

The underlying concept of the CSSs is that the 13 disorders have some similar clinical features and a common pathophysiologic component of central sensitization (CS). (See Etiology.) Yunus disputes the idea that the pain experienced by people with CSSs is merely psychiatric, psychosocial, or psychological in nature. Although depression and stress may contribute to the symptoms of CSS, they are still based on objective changes in the CNS.[4]

Etiology

The physiology of CS involves a process in which inflammation that may be caused by a minor trauma sets off an involved CNS response that results in pain and distress. The response pathway starts with the initial input that causes the release of bradykinin, prostaglandins, serotonin, and substance P to activate A-delta and C-fiber nociceptors at peripheral tissues.[4]

Nociceptive impulses travel through fibers to wide-dynamic-range neurons in the spinal cord. At nerve terminals, activated C-fibers convey neurotransmitters or neuromodulators that generate a barrage of impulses at the synapse and hyperexcite postsynaptic receptors. The changes cause hyperexcitability escalation of other neurons, giving rise to hypersensitivity to peripheral stimuli.[4]

The enhanced nociceptive (spinal) flexion reflex (NFR) is a consistent objective test for CS, obtained by electrically stimulating the sural nerve and then measuring the electromyelographic (EMG) response of the biceps femoris. People with fibromyalgia or other CSSs have demonstrated an accentuated NFR (or decreased stimulus threshold), which is indicative of CS.[4]

Along with the CS response, other physiologic aspects of CSSs include sympathetic overactivity, genetic factors, neuroendocrine dysfunctions, peripheral nociception generators (eg arthritis), environmental stimuli, poor sleep, viral or local infections (may trigger inflammatory mediators that activate nociceptive fibers), nonrestorative sleep, and psychosocial distress.[4, 5]

Epidemiology

United States statistics

Estimates of the prevalence of JPFS using the American College of Rheumatology (ACR) diagnostic criteria (see Differentials) are as high as 6%.[1] JPFS accounts for 7.7% of new diagnoses made among children and adolescents by pediatric rheumatologists. Musculoskeletal pain syndromes, which include JPFS, account for approximately 25% of new referrals to pediatric rheumatologists. JPFS is the diagnosis in 25-40% of children with musculoskeletal pain syndromes.[1]

In the United States, FMS affects approximately 2% of the population.[4] Earlier data for the adult population indicated that 0.5% of men and about 3.4% of women are diagnosed with FMS.[6]

Romano studied 15 children (10 females, 5 males) aged 16 years and younger (mean age, 13 y) in a rheumatology clinic and found that many cases of juvenile FMS go undiagnosed and are confused with other diagnoses.[7] He reported that 67% visited 3 or more doctors before evaluation by a rheumatologist; 60% were diagnosed with juvenile chronic arthritis, and other misdiagnoses included psychological problems, hysteria, and growing pains.[7]

International statistics

Fibromyalgia syndrome occurs in 6.2% of Israeli school children[8] and 1.3% of Mexican school children.[9] The prevalence is high in school-age children in Turkey.[10]

Age-related differences in incidence

Patients with pediatric FMS most frequently present in adolescence (age 13-15 y). The mean age of onset is 12 years. The earliest reported case in pediatrics was a 5-year-old child with FMS.

Sex-related differences in incidence

FMS is diagnosed more commonly in girls than in boys (at least 3-7 times more commonly). Most children diagnosed with JPFS are prepubertal or adolescent girls aged 13-15 years,[11, 12] although boys of the same age or younger have also been diagnosed; reported figures indicate as many as 35% of all children diagnosed with the syndrome are boys.

Race-related differences in incidence

In the United States, FMS is less common among black children.

Prognosis

Improvement in signs and symptoms of FMS is likely in children and adolescents.

In 1995, Buskila and colleagues studied FMS among children aged 9-15 years.[13] Data on 15 of the children showed that 73% (ie, 11 of the 15) no longer met criteria for FMS (see Differentials) at 30 months’ follow-up. The mean number of TPs and the amount of force necessary to elicit pain at each TP showed significant improvement.

Symptoms among the 4 children who still met criteria for FMS included abdominal pain, headache, paresthesias, morning stiffness, and sleep disturbance. Additionally, 7 children were observed who did not progress to the point of meeting the full criteria over the 30 months, and all 7 children had improved.

In 1998, Siegel and colleagues observed 33 patients, with a mean follow-up of 2.6 years. Improvement was observed in most patients during that follow-up time, with all patients showing some positive response to treatment.[12] Given prognostic findings, children with FMS as a whole are more likely to have a favorable outcome than adults diagnosed with FMS.

In 2000, Gedalia and colleagues, after observing children in a rheumatology clinic, collected data on 50 children with an average follow-up period of 18 months.[14] They found that as compared with the initial presentation, 60% of the children had improved, 36% had stayed the same, and 4% had worsened. Nearly all of the children needed to continue medications for up to 4 years after initial presentation.

Patient Education

Health care providers are responsible for educating children and families about every facet of FMS in an effort to improve basic knowledge and coping mechanisms to deal with the long-term aspects of the disease.

All individuals involved must have fully understand the goals of treatment, including exercise regimes, expectations of medication therapy, and overriding aspects of living with chronic pain. Successful treatment and improved outcomes are enhanced when the patient has a multifaceted approach to treatment, including medical care, psychologic interventions, and physical therapy. Education concerning every aspect of care and intervention is a key to successful treatment of FMS.

For patient education resources, see the following:

Muscle Disorders Center

Mental Health and Behavior Center

Back, Ribs, Neck, and Head Center

Fibromyalgia

Chronic Fatigue Syndrome

Chronic Pain

Fatigue

 

Presentation

History

Fibromyalgia syndrome (FMS) is characterized by musculoskeletal pain, stiffness, and aching.[15] The severity of pain at the tender points (TPs) rates 8 on a scale of 10. Symptoms of fatigue, anxiety, and depression are reported.

Adolescents with FMS often describe abnormal sleep patterns that interfere with school and family activities. Descriptions of difficulty falling asleep, frequent awakenings due to discomfort, and feeling unrested in the morning are common.[16]

Tayag-Kier et al reported that children with FMS presented with long sleep latency, shortened total sleep time, decreased sleep efficiency, and increased wakefulness during sleep.[17] Additionally, they found that a subset of children with FMS exhibited periodic limb movement in sleep (PLMS), in which patients experienced significantly higher wakefulness after sleep onset.

A study of 16 children with JPFS, 16 with arthritis, and 16 healthy controls found that children with fibromyalgia demonstrated increased levels of anxiety and depression, more temperamental instability, less family cohesion, and higher pain sensitivity than the other 2 groups. They concluded that the interaction of the associated risk factors of JPFS may explain the severity and breadth of the symptoms of this syndrome.[18]

In addition, other associated symptoms of FMS in children include irritable bowel syndrome, migraines, premenstrual syndrome, Raynaud phenomenon, female urethral syndrome, and restless leg syndrome.

In 1998, Siegel and colleagues found the following symptoms at the initial presentation of 45 children with FMS[12] :

  • Sleep disturbance (96%)

  • Diffuse pain (93%)

  • Headaches (71%)

  • General fatigue (62%)

  • Morning stiffness (53%)

  • Morning fatigue (49%)

  • Depression (43%)

  • Feeling worse with exercise (42%)

  • Subjective swelling (40%)

  • Irritable bowel (38%)

  • Dysmenorrhea (36%)

  • Illness changes with weather (36%)

  • Paresthesias (24%)

  • Global anxiety (22%)

  • Lack of energy (18%)

  • Raynaud phenomenon (13%)

In a 2000 review of 59 children with pediatric FMS, Gedalia and colleagues found the following symptoms[14] :

  • Generalized aches (97%)

  • Headaches (76%)

  • Sleep disturbances (70%)

  • Stiffness (30%)

  • Subjective joint swelling (24%)

  • Fatigue (20%)

  • Abdominal pain (17%)

  • Joint hypermobility(14%)

  • Depression (7%)

The Yunus and Masi criteria for juvenile primary fibromyalgia syndrome (JPFS) state that the child experiences 3 of 10 minor criteria, most of which are subjective findings reported by the child (see Differentials). These 10 minor criteria are as follows[11] :

  • Chronic anxiety or tension

  • Fatigue

  • Sleep disturbance

  • Chronic headaches

  • Irritable bowel syndrome symptoms

  • Subjective soft tissue swelling

  • Numbness or tingling of the extremities

  • Pain modulated by physical activity

  • Pain modulated by weather

  • Pain modulated by stress or anxiety

Questions for patient and family should explore the presence of the following:

  • Widespread pain or aching

  • Headaches

  • Morning stiffness and fatigue

  • Subjective joint swelling

  • Abdominal pain

  • Symptoms of depression

  • Quality and amount of sleep

Assess the quality of pain (eg, when, what, where, how long) with the following questions:

  • When did the pain start?

  • What makes it better?

  • What makes it worse?

  • What is it like (eg, sharp, dull, aching, deep)?

  • What is the appearance of the affected area (eg, swelling, edema)?

  • Where is the pain?

  • How long does it last?

  • Does it vary throughout the day?

  • Does it wake you up at night?

Other questions about associated symptoms include the following:

  • Do the child’s legs move constantly during the night?

  • Do you experience migraines or headaches?

  • Do you have facial pain?

  • Do you have fever?

  • Do you have any change in appetite?

  • Have you lost weight?

  • Can you describe your sleep pattern?

  • Are you disturbed easily during sleep?

  • Do you have frequent awakenings?

  • Do you feel rested in the morning?

  • Do you have any bowel or gastrointestinal (GI) symptoms?

  • Do you feel anxious, sad, or depressed?

  • Are your muscles weak?

Questions about the psychosocial aspect include the following:

  • Are you experiencing any stressors or problems at school?

  • Are you experiencing any stressors or problems in your family?

  • Are you tired in school?

  • Are you able to keep up with the other children at school and outside activities?

  • What impact has the pain had on routine activities?

  • How has your family responded to the pain?

  • Does anyone at home have similar problems?

Common aggravating factors of FMS include the following:

  • Anxiety and stress

  • Cold weather

  • Humid weather

  • Inactivity

  • Physical overactivity

  • Poor sleep

Common alleviating factors of FMS include the following:

  • Hot shower or bath

  • Moderate activity

  • Stretching and exercising

  • Warm weather

  • Massage

Physical Examination

A standard physical examination to diagnose FMS is essential. Examination skill in palpating tender points (TPs) is important in establishing a diagnosis. The diagnostic criteria for JPFS, according to Yunus and Masi (see Differentials), include pain at 5 or more well-defined TPs (see the image below).[11]

Illustration of 9 paired tender points identified Illustration of 9 paired tender points identified in the 1990 statement of the American College of Rheumatology on fibromyalgia. They are as follows: (a) insertion of nuchal muscles into occiput, (b) upper border of trapezius, (c) muscle attachments to upper medial border of scapula, (d) anterior aspects of the C5–C7 intertransverse spaces, (e) second rib space 3 cm lateral to the sternal border, (f) muscle attachments to lateral epicondyle 2 cm below bony prominence, (g) upper outer quadrant of gluteal muscles, (h) muscle attachments just posterior to greater trochanter, and (i) medial fat pad of knee proximal to joint line.

Perform thumb palpitation of 18 specific TP sites with a force of 4 kg per unit area. This force is approximately the pressure necessary to blanch the examiner’s nail. Note that this criterion is suggested but not agreed on among practitioners. Neumann et al suggest a criterion of 3 kg rather than 4 kg in pediatric patients because the threshold in children is different from that in adults.[19] In children, palpation elicits tenderness in TPs at 5 of 11 of the following locations (most of them bilateral):

  • Occiput - Bilateral, at the suboccipital muscle insertions

  • Low cervical - Bilateral, at the anterior aspects of the intertransverse spaces at C5-C7

  • Trapezius - Bilateral, at the midpoint of the upper border

  • Supraspinatus - Bilateral, at origins, above the scapula spine near the medial border

  • Second rib - Bilateral, at the second costochondral junctions just lateral to the junctions on upper surfaces

  • Lateral epicondyle of humerus - Bilateral, 2 cm distal to the epicondyles

  • Gluteal - Bilateral, in the upper outer quadrants of the buttocks in the anterior fold of muscle

  • Greater trochanter - Bilateral, posterior to the trochanteric prominence

  • Knee - Bilateral, at the medial fat pad proximal to the joint line

In 1986, Calabro observed that the examination of joints in juvenile FMS revealed normal findings despite tenderness and spasms in soft tissue on palpation.[20] Classic signs of joint swelling, heat, or redness are not seen on examination. Physical findings to explore include joint hypermobility (using criteria developed by Carter and Wilkerson and modified by Bird), swelling or joint edema, abdominal tenderness, and joint range of motion to determine stiffness. Skin palpation may also reveal changes in the texture of both skin and subcutaneous tissue.

 

DDx

Diagnostic Considerations

In adults, diagnosis of fibromyalgia syndrome (FMS) is based on history, physical examination, laboratory study findings, and exclusion of other potential conditions.

In 1990, the American College of Rheumatology (ACR) defined 2 major diagnostic criteria for classifying FMS in adults. The first is a history of widespread pain for at least 3 months that involves both sides of the body above and below the waist. Specific areas include the cervical skeleton (eg, spine, anterior chest), the shoulders or buttocks (considered for each involved side), and the lower back (considered below the waist).

The second criterion requires pain on 11 of 18 defined tender points (TPs) upon digital palpation with approximately 4 kg per unit area of force. For a positive result, the patient must indicate that palpation is painful (see Physical Examination).

In 1985, Yunus and Masi first compared juvenile primary fibromyalgia syndrome (JPFS) with FMS in adults.[11] The 1990 criteria for adult FMS were found to be less sensitive to the events that occur in childhood FMS. Yunus and Masi proposed FMS criteria that are slightly different for children and adolescents. Their criteria take into consideration a more variable presentation, along with a dependence on adult input to make the diagnosis.

The ACR criteria for adults have not been validated in children; thus current diagnostic criteria for JPFS are based on the Yunus and Masi guidelines.[11]

By these criteria, a JPFS patient should have had 3 or more months of widespread musculoskeletal pain, with 5 or more well-defined TP sites. No other underlying medical conditions that could have caused the symptoms should be present, and routine laboratory test results should be in the normal range. In addition, at least 3 of the following 10 minor criteria (or associated symptoms) should be present (see History):

  • Chronic anxiety or tension

  • Fatigue

  • Poor sleep

  • Chronic headaches

  • Irritable bowel syndrome

  • Subjective soft tissue swelling

  • Numbness

  • Pain modulation by physical activities

  • Pain modulation by weather conditions

  • Pain modulation by anxiety or stress

Although children and adults with FMS experience similar symptoms, children seem to have more sleep disturbances. Compared with adults, children experience pain in fewer body areas (3 versus 5) and have fewer TPs (5 out of 11 versus 11 out of 18). Children have less lower back pain, hand pain, and paraspinal TPs; however, they experience ankle pain and increased pain associated with overactivity.

Go to Fibromyalgia and Rehabilitation and Fibromyalgia for complete information on these topics.

Other conditions to be considered include the following:

  • Anterior chest wall syndrome

  • Benign rheumatoid nodules

  • Bursitis

  • Dysautonomia

  • Early spondyloarthropathy

  • Growing pains

  • Hypermobility syndrome

  • Inflammatory bowel disease

  • Malingering

  • Multiple sclerosis

  • Reflex sympathetic dystrophy

  • Restless leg syndrome

  • Tendinitis

  • Thyroid disease

  • Syndrome of multiple chemical sensitivities

Differential Diagnoses

 

Workup

Approach Considerations

The patient’s history and physical examination guide the laboratory workup for fibromyalgia syndrome (FMS). Because the presentation and diagnosis by exclusion of other physical problems are often confusing, children with FMS may be evaluated by numerous physicians who perform various batteries of tests. Studies may include laboratory tests, diagnostic imaging, and polysomnography.

Lab Studies

Most laboratory tests are expected to produce findings within the reference range when FMS is diagnosed. Studies to consider in a child presenting with a clinical picture consistent with fibromyalgia syndrome and expected findings are discussed below.

  • Complete blood count (CBC) - Findings are normal.

  • Erythrocyte sedimentation rate (ESR) - 15 mm/h.

  • Rheumatoid factor - Findings are negative.

  • C-reactive protein and antinuclear antibody (ANA) - Findings may be positive. However, because of the high incidence of ANA in the general population, ANA testing should be avoided unless the history and physical examination indicate features and abnormalities not found in FMS.

  • Serum prolactin - Findings are negative.

  • Serum electrolytes - levels are within the reference range.

  • Liver function tests - Results are normal.

  • Muscle enzymes - levels are within the reference range.

  • Purified protein derivative - Findings are negative.

  • Blood and urine cultures - Results are negative.

  • Thyroid function tests - Results are normal.

  • Serotonin, substance P growth hormone, and cortisol - Characteristic changes in serotonin, substance P growth hormone, and cortisol suggest autonomic and neuroendocrine system dysregulation.

Diagnostic Imaging

Plain radiography of the chest, ribs, and back reveals normal findings, as does ultrasonography of the abdomen, pelvis, and paravertebrae. Bone scanning also reveals normal findings, and the results of computed tomography (CT) scanning and magnetic resonance imaging (MRI) are typically normal as well.

Polysomnography

Polysomnography, including periodic limb movement in sleep (PLMS) assessment, which is used to evaluate possible sleep disorders, reveals normal findings.

 

Treatment

Approach Considerations

Because of the multifaceted problems that develop, effective treatment of fibromyalgia syndrome (FMS) requires a multidisciplinary approach, which may include medication, physical therapy, exercise, support groups, and psychological therapy. No surgical treatment is indicated. The goals of treatment are to reduce pain and depression, to decrease sleep disturbances, and to promote physical activity. A number of cognitive-behavioral interventions may help to mitigate the disorder.[21] Activity is a mainstay in the treatment of FMS.

Go to Fibromyalgia and Rehabilitation and Fibromyalgia for complete information on these topics.

Pharmacologic Therapy

The medication treatment for juvenile primary fibromyalgia syndrome (JPFS) is inferred for what has been studied and used in adult fibromyalgia. Typical medication regimens for pediatric FMS primarily include skeletal muscle relaxants, low-dose tricyclic antidepressants, and selective serotonin reuptake inhibitors (SSRIs). Some evidence reports that pain and symptom management with nonsteroidal anti-inflammatory drugs (NSAIDs) in combination with antidepressants and nonaddictive analgesics is effective.

Low-dose antidepressants, such as amitriptyline (Elavil), and skeletal muscle relaxants, such as cyclobenzaprine (Flexeril), help decrease the hyperarousal mechanisms in fibromyalgia and, in turn, help the child and adolescent sleep better. Both medications are administered at bedtime or 1-2 hours before bedtime. Some debate surrounds which medication should be initially used. Some suggest the use of cyclobenzaprine first in treatment, whereas others suggest beginning medication therapy with low-dose tricyclic antidepressants.[14]

An adult study found the combination of a low-dose tricyclic antidepressants (amitriptyline) and the SSRI fluoxetine (Prozac) to be effective in improving sleep quality and alleviating pain and fatigue.[22] A current level 3 clinical trial of fluoxetine to test its efficacy in treating JPFS is under way.

Depending on which medication is started first, either skeletal muscle relaxants or low-dose tricyclic antidepressants have been used when the child or adolescent does not respond to the initial medication. An NSAID or acetaminophen is used in conjunction with the muscle relaxants or antidepressants in some cases that are unresponsive to the mainstay therapies alone. Active investigation is underway to look at the potential role for S-adenosylmethionine (SAMe) and the SSRIs in the adult population.

A double-blind placebo controlled study of pregabalin in adults older than 18 years found reduction in pain intensity ratings as well as an improvement in reported sleep outcomes and an improvement in the quality of sleep.[23] In 2007, the US Food and Drug Administration (FDA) approved pregabalin for treatment of fibromyalgia in adults, and this drug became the first FDA-approved treatment for fibromyalgia.

Pregabalin monotherapy is used to treat pain and other fibromyalgia-related symptoms in adults. Evidence suggests that pregabalin improves daily function for some patients with fibromyalgia. The FDA states that the manufacturer of pregabalin (Pfizer) may conduct studies of pregabalin in children with fibromyalgia and in breast-feeding women, but such studies have not been completed at this time.

Bennett and Tayag-Kier et al suggested that a sleep analysis in children is helpful in determining treatable causes of sleep disturbance and periodic limb movement in sleep (PLMS).[2, 17] Few studies have involved children; however, low-dose tricyclic antidepressants or cyclobenzaprine has been used to help promote deeper sleep. Gedalia et al first tried cyclobenzaprine at bedtime to help promote sleep and then switched to low-dose antidepressants when 25% of the patients did not respond to the muscle relaxant.[14]

Exercise and Activity

The goal of an exercise regime is to improve cardiovascular health and musculoskeletal fitness through nonimpact aerobic activity. An essential component of the treatment regimen, routine exercise consists of moderate exercise, such as brisk walking for 20 minutes 3 times per week and progression as tolerated. In 2000, Gedalia et al recommended physical therapy guidance to low-impact exercises, such as stretching, walking, biking, and swimming, for at least half an hour per day, to improve cardiovascular fitness.[14]

Returning to normal activity is imperative for the child who has stopped sport and social activities because of pain; this helps to modulate the pain. A physical therapist may be extremely helpful in establishing a reasonable exercise and activity regime. Maintaining the child’s physical conditioning is imperative for achieving the best possible long-term outcome of FMS.

The literature supports the therapeutic use of exercise and activity as an important treatment aspect of JPFS. A meta-analysis by Rossy et al found better outcomes with the use of cognitive-behavioral interventions (see Psychotherapy) and exercise than with medication.[24] Degotardi et al studied JPFS using exercise and a cognitive-behavioral approach and also found that cognitive-behavioral therapy strategies helped children effectively manage musculoskeletal pain associated with the disorder.[25]

One study evaluated 34 participants to determine whether physical activity was predictive of brain responses to experimental pain in FMS using functional magnetic resonance imaging (fMRI). Using self-reported measures of physical activity and fMRI of painful heat stimuli, the results found that greater physical activity was significantly associated with decreased pain ratings, suggesting that physically active FMS patients appear to maintain their ability to modulate pain while those who are less active do not.[26]

A study by Sherry et al concluded that children with fibromyalgia can be successfully treated without medications with a very intensive physical and occupational therapy and psychotherapy program.[27, 28]  A pilot trial that included 40 adolescents with juvenile fibromyalgia evaluated a program that combines cognitive-behavioral therapy with specialized neuromuscular exercise training and reported significant decreases in pain in the combined group.[33]

 

Patient Support

Although a better understanding of what causes FMS would be helpful in determining treatment options, a holistic approach to the child and family living with this problem is the current recommendation.

Supporting the child and family to maintain as normal a lifestyle as possible is important because they live with a potentially chronic disorder. Emphasis on both the child’s and the family’s understanding of the disorder is helpful in learning to live with and overcome the problems. Attendance at school and other usual activities is imperative. Modifying participation or attendance may be necessary in light of the child’s ability to keep up with the expected activities.

Psychotherapy

Cognitive-behavioral therapy has proven helpful in some cases. Conte et al compared children who had JPFS with healthy children and those who had arthritis.[18] The children and adolescents with JPFS showed increased levels of anxiety and depression, greater temperamental instability, higher pain sensitivity, and less family cohesion than healthy children or those with arthritis.

Degotardi et al studied 67 children with JPFS by using an educational and behavioral approach that addressed sleep difficulties, pain management, and exercise. They found significant differences in all physicals between preintervention status and postintervention status.[25]

Walco and Ilowite found that the use of a cognitive-behavioral program showed improvement in symptoms over a 4- to 24-month period.[29] Likewise, Vereker studied the use of counseling, behavioral techniques, and physical activity in 5 children who had shown improvement in symptoms.

Kashikar-Zuck et al found that children with JPFS had higher levels of depression than children with nonmalignant back pain, possibly because of the longer time taken for those with JPFS to receive specialty care and treatment for the problem.[30]

A study by Tran et al suggested that a combination of cognitive-behavioral therapy that emphasizes pain coping strategies and a specially tailored exercise program reduces fears that exertion will worsen pain in teenagers with juvenile fibromyalgia.[31, 32]

Other Modalities

Other modalities found to be helpful in modulating pain include hypnotherapy and transcutaneous electrical nerve stimulation (TENS). Using palliative measures to treat symptoms and minimizing physical disability is an important treatment mainstay.

Consultations

Because of the multifaceted symptoms that present, the patient may be referred to the following subspecialists for evaluation and treatment:

  • Physical medicine and rehabilitation specialist

  • Rheumatologist

  • Psychiatrist/psychologist

  • Pulmonary medicine specialist for evaluation of sleep disorders that may cause fatigue and the presence of PLMS

  • Orthopedist

 

Medication

Medication Summary

Pharmacologic treatment of juvenile primary fibromyalgia syndrome (JPFS) is inferred from what has been studied and used in adult fibromyalgia. Typical medication regimens for pediatric fibromyalgia syndrome (FMS) primarily include skeletal muscle relaxants, low-dose tricyclic antidepressants, and selective serotonin reuptake inhibitors (SSRIs). Some evidence reports that pain and symptom management with nonsteroidal anti-inflammatory drugs (NSAIDs) in combination with antidepressants and nonaddictive analgesics is effective.

Antidepressants

Class Summary

Antidepressant agents help decrease pain intensity and improve sleep quality. They counteract the hyperarousal mechanism in FMS and promote deeper sleep in children and adolescents. Both medications are administered at bedtime or 1-2 hours before bedtime. SSRIs have been found useful for treating chronic pain states in adults.

Amitriptyline

Amitriptyline is used for analgesia for certain chronic and neuropathic pain conditions.

Imipramine (Tofranil)

These agents have been suggested to act by inhibiting reuptake of noradrenaline at synapses in central descending pain modulating pathways located in the brainstem and spinal cord.

Doxepin (Silenor)

Doxepin increases the concentration of serotonin and norepinephrine in the CNS by inhibiting their reuptake by the presynaptic neuronal membrane. It inhibits histamine and acetylcholine activity and has proven useful in the treatment of various forms of depression associated with chronic and neuropathic pain.

Nortriptyline (Pamelor)

Nortriptyline has demonstrated effectiveness in the treatment of chronic pain.

Desipramine (Norpramin)

This is the original TCA used for depression. These agents have been suggested to act by inhibiting reuptake of noradrenaline at synapses in central descending pain modulating pathways located in the brainstem and spinal cord.

Skeletal Muscle Relaxants

Class Summary

Skeletal muscle relaxants may act centrally by a selective action on the central nervous system (CNS) and are principally used for relieving painful muscle spasms or spasticity that occurs in musculoskeletal and neuromuscular disorders. Their mechanism of action may be due, in part, to their CNS-depressant activity.

Cyclobenzaprine (Flexeril)

Cyclobenzaprine helps decrease the hyperarousal mechanisms in FMS and, in turn, helps the child sleep better. It is structurally related to the tricyclic antidepressants and exhibits similar pharmacologic effects. It primarily acts on the CNS at the brainstem level.

Nonsteroidal Anti-Inflammatory Drugs and Miscellaneous Analgesics

Class Summary

NSAIDs and miscellaneous analgesics are used for their anti-inflammatory, analgesic, and antipyretic effects. They are useful for the relief of mild-to-moderate pain.

Ibuprofen (Advil, Motrin)

Ibuprofen may help achieve analgesia when used in combination with skeletal muscle relaxants or tricyclic antidepressants. It inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

Ketoprofen

Ketoprofen is used for relief of mild to moderate pain and inflammation. Small dosages initially are indicated in small and elderly patients and in those with renal or liver disease. Doses greater than 75 mg do not increase therapeutic effects. Administer high doses with caution and closely observe the patient for response.

Naproxen (Aleve, Anaprox, Naprosyn)

Naproxen is used for relief of mild to moderate pain; it inhibits inflammatory reactions and pain by decreasing the activity of cyclo-oxygenase, which is responsible for prostaglandin synthesis. NSAIDs decrease intraglomerular pressure and decrease proteinuria.

Acetaminophen (Tylenol, FeverAll, Tempra)

Acetaminophen is the drug of choice for pain in patients with documented hypersensitivity to aspirin or NSAIDs, patients with upper gastrointestinal disease, or those who are taking oral anticoagulants.

 

Questions & Answers

Overview

What is juvenile primary fibromyalgia syndrome (JPFS)?

What is the pathophysiology of juvenile primary fibromyalgia syndrome (JPFS)?

What causes juvenile primary fibromyalgia syndrome (JPFS)?

What is the US prevalence of juvenile primary fibromyalgia syndrome (JPFS)?

What is the global prevalence of juvenile primary fibromyalgia syndrome (JPFS)?

At what age does juvenile primary fibromyalgia syndrome (JPFS) typically present?

What are the sexual predilections of juvenile primary fibromyalgia syndrome (JPFS)?

What are the racial predilections of juvenile primary fibromyalgia syndrome (JPFS)?

What is the prognosis of juvenile primary fibromyalgia syndrome (JPFS)?

What is included in patient education about juvenile primary fibromyalgia syndrome (JPFS)?

Presentation

Which clinical history findings are characteristic of juvenile primary fibromyalgia syndrome (JPFS)?

What are the signs and symptoms of juvenile primary fibromyalgia syndrome (JPFS)?

What are the Yunus and Masi diagnostic criteria for juvenile primary fibromyalgia syndrome (JPFS)?

What is the focus of clinical history to evaluate juvenile primary fibromyalgia syndrome (JPFS)?

What are the aggravating factors of juvenile primary fibromyalgia syndrome (JPFS)?

What are the alleviating factors of juvenile primary fibromyalgia syndrome (JPFS)?

What is included in the physical exam to evaluate juvenile primary fibromyalgia syndrome (JPFS)?

DDX

How is juvenile primary fibromyalgia syndrome (JPFS) diagnosed?

Which conditions are included in the differential diagnoses of juvenile primary fibromyalgia syndrome (JPFS)?

What are the differential diagnoses for Juvenile Primary Fibromyalgia Syndrome?

Workup

What is included in the workup of juvenile primary fibromyalgia syndrome (JPFS)?

What is the role of lab tests in the workup of juvenile primary fibromyalgia syndrome (JPFS)?

What is the role of imaging studies in the workup of juvenile primary fibromyalgia syndrome (JPFS)?

What is the role of polysomnography in the workup of juvenile primary fibromyalgia syndrome (JPFS)?

Treatment

How is juvenile primary fibromyalgia syndrome (JPFS) treated?

What is the role of medications in the treatment of juvenile primary fibromyalgia syndrome (JPFS)?

What is the role of exercise in the treatment of juvenile primary fibromyalgia syndrome (JPFS)?

What type of support should be provided to families and patients with juvenile primary fibromyalgia syndrome (JPFS)?

What is the role of cognitive-behavior therapy (CBT) in the treatment of juvenile primary fibromyalgia syndrome (JPFS)?

Which palliative interventions are used in the treatment of juvenile primary fibromyalgia syndrome (JPFS)?

Which specialist consultations are beneficial to patients with juvenile primary fibromyalgia syndrome (JPFS)?

Medications

Which medications are used in the treatment of juvenile primary fibromyalgia syndrome (JPFS)?

Which medications in the drug class Nonsteroidal Anti-Inflammatory Drugs and Miscellaneous Analgesics are used in the treatment of Juvenile Primary Fibromyalgia Syndrome?

Which medications in the drug class Skeletal Muscle Relaxants are used in the treatment of Juvenile Primary Fibromyalgia Syndrome?

Which medications in the drug class Antidepressants are used in the treatment of Juvenile Primary Fibromyalgia Syndrome?