AUTHOR INFORMATION

Section 1 of 11

Authored by Angelo P Giardino, MD, PhD, Clinical Associate Professor, Department of Pediatrics, Baylor College of Medicine; Medical Director, Texas Children's Health Plan, Inc

Coauthored by Eileen R Giardino, PhD, RN, CRNP, Associate Professor of Nursing, Department of Acute and Continuing Care, University of Texas Health Sciences Center Houston School of Nursing; Gregory F Keenan, MD, Director of Medical Affairs, Department of Immunology, Centocor, Inc

Angelo P Giardino, MD, PhD, is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, American College of Physician Executives, American Professional Society on the Abuse of Children, and International Society for Prevention of Child Abuse and Neglect

Edited by Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital at Houston; Associate Professor, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Thomas JA Lehman, MD, Clinical Professor of Pediatrics, Weill-Cornell University; Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Hospital for Special Surgery; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; and Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital at Houston; Associate Professor, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Baylor College of Medicine

Author's Email:	Angelo P Giardino, MD, PhD
Editor's Email:	Barry L Myones, MD

eMedicine Journal, December 5 2006, VOLUME 7, Number 12

INTRODUCTION

Section 2 of 11

Background: Juvenile primary fibromyalgia syndrome (FMS) is characterized by musculoskeletal pain of unknown origin; multiple soft tissue tender points (TPs); pain modulated by factors such as activity, anxiety, stress, and weather changes; stiffness in multiple areas; fatigue from sleep disturbance; absence of other systematic manifestations; and normal findings on routine laboratory tests.

Although children and adults with FMS experience similar symptoms, children seem to experience more sleep disturbances and fewer TPs than adults. Other associated symptoms include chronic headaches, soft tissue swelling, tension, and anxiety.

In 1985, Yunus and Masi first compared juvenile FMS with FMS in adults. FMS is diagnosed based on history, physical examination, and laboratory study findings and exclusion of other causes of findings. The 1990 American College of Rheumatology (ACR) diagnostic criteria for FMS include diffuse pain and 11 or more TPs. The goal of treatment is to control the symptoms using multiple therapies that include medication, physical therapy, exercise, support groups, and psychologic therapy. The incidence of FMS in children may be as high as 6.2% of the general pediatric population. The prognosis of FMS in children is more favorable than that in adults.

The 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 TPs when digitally palpated with approximately 4 kg per unit area of force. For a positive result, the patient must indicate that palpation is painful (see Physical).

The 1990 criteria for adult FMS were found to be less sensitive to the events that occur in childhood FMS. The term juvenile primary FMS results from a better understanding of the child's experience with 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. Pediatric FMS criteria include the presence of 2 major criteria and some minor diagnostic symptomatology.

The 1985 Yunus and Masi diagnostic criteria are similar to the latter ACR criteria and include the following:

• Three months of widespread pain in the absence of other underlying causes for the symptoms

• Severe pain in 5-11 TPs with palpation of less than 4 kg per unit area of force

In FMS, routine laboratory test results are, by definition, in the reference range and 3-10 of the following minor criteria are present: chronic anxiety or tension; fatigue; poor sleep; chronic headaches; irritable bowel syndrome; subjective soft tissue swelling; numbness; and pain modulation by physical activities, weather conditions, or anxiety and stress.

Bennett describes FMS in the 1997 Textbook of Rheumatology as involving a core feature of pain (eg, widespread musculoskeletal pain, multiple TPs), typical features (eg, fatigue, stiffness, skin tenderness, postexertional pain, sleep disturbance), and associated features (eg, irritable bowel symptoms, poor memory, tension headaches, dizziness, fluid retention, paraesthesias, restless legs, bruising, Raynaud phenomenon). The chronic musculoskeletal pain affects quality of life, while fatigability influences motor response and ability to complete activities of daily living in an expedient time frame.

As opposed to adults with FMS, children with the condition may have the following symptoms:

• Increased frequency of pain aggravated by overactivity (with pain being relieved by moderate activity)

• Increased subjective swelling

• Decreased pain modulation by anxiety and weather

Children have less lower back pain, hand pain, and paraspinal TPs; however, children experience ankle pain and increased pain associated with overactivity.

Pathophysiology: FMS is a physiologic entity rather than a psychiatric disorder. Although the physiologic cause of FMS in children is unknown, studies suggest possibilities such as abnormalities in muscle structure or repair, changes in neuroendocrine transmitters, endocrine abnormalities, psychologic components, or biochemical changes in the lower spine or upper back. FMS may be either primary or secondary to hypothyroidism, malignancy, osteoarthritis, rheumatic diseases, sports-related overactivity, or trauma.

Some authors also describe a reactive FMS, which arises after a discrete illness or after a specific episode of trauma. Also, more than 30% of cases involve psychologic comorbidity. The ACR recommends against the use of primary and secondary designations, but these continue to prove useful in clinical and research settings. A number of abnormalities have been suggested as a possible pathogenesis, including abnormalities in CNS neurotransmitter levels, delta sleep disturbance, muscle metabolic aberrations, and various psychopathologies.

Frequency:

• In the US: FMS accounts for 7.5% of new diagnoses made among children and adolescents by pediatric rheumatologists. Musculoskeletal pain syndromes, such as juvenile primary FMS, account for approximately 25% of new referrals to pediatric rheumatologists.

• Internationally: FMS occurs in 6.2% of Israeli school children and 1.3% of Mexican school children.

Mortality/Morbidity:

• In a 2000 review of 59 children with pediatric FMS, Gedalia and colleagues found the following symptoms:
  
  
  • Generalized aches (97%)
    
    
  • Headaches (76%)
    
    
  • Sleep disturbances (70%)
    
    
  • Stiffness (30%)
    
    
  • Subjective joint swelling (24%)
    
    
  • Fatigue (20%)
    
    
  • Abdominal pain (17%)
    
    
  • Joint hypermobility(14%)
    
    
  • Depression (7%)

• In 1998, Siegel and colleagues found the following symptoms at the initial presentation of 45 children with FMS:
  
  
  • 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%)

• Studies of children with FMS have documented a high association of sleep disturbances. Tayag-Kier et al reported in 2000 that children with FMS presented with long sleep latency, shortened total sleep time, decreased sleep efficiency, and increased wakefulness during sleep. Additionally, Tayag-Kier et al 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.
  
  
• 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.

Race: In the United States, FMS is less common among African American children.

Sex: FMS is diagnosed more commonly in girls than in boys. Studies show that girls are at least 3-7 times more likely than boys to be diagnosed with FMS.

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

CLINICAL

Section 3 of 11

History: Fibromyalgia syndrome (FMS) is characterized by musculoskeletal pain, stiffness, and aching. The severity of pain at the 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.

• 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 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: A standard physical examination to diagnose FMS is essential. Examination skill in palpating tender points (TPs) is important in establishing a diagnosis.

• 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 using a 3-kg criterion rather than 4 kg in children because their threshold is different from that in adults. In the child, palpation elicits tenderness in TPs at 5 of 11 of the following locations:


• • 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 mid point 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 upper outer quadrants of buttocks in 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 described that the examination of joints in juvenile FMS revealed normal findings despite tenderness and spasms in soft tissue on palpation. Therefore, 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.

Causes: Various etiologies of FMS have been proposed, although the actual cause of the syndrome is unknown. In 1989, Pellegrino et al studied evidence of inherited primary FMS and found an autosomal dominant mode of inheritance; therefore, FMS may be an inherited condition.

Some findings indicate a relationship in the disturbances in the neuroendocrine axis that may, in turn, affect sleep. Findings of sleep electroencephalograms in patients with FMS indicate disturbance of the non–rapid eye movement (REM) sleep phase by alpha wave intrusions that, in turn, inhibits progression of stage 3 and stage 4 non-REM sleep. These findings correlate with reports of frequent awakenings and feeling unrefreshed after sleeping.

Other proposed etiologies include neurotransmitter abnormalities, immune disorders, endocrine abnormalities, allergic factors, viral infections, and structural muscle changes.

DIFFERENTIALS

Section 4 of 11

Hepatitis C

Other Problems to be Considered:

Anterior chest wall syndrome
Benign rheumatoid nodules
Bursitis
Depression
Dysautonomia
Early spondyloarthropathy
Growing pains
Hypermobility syndrome
Hypochondriasis
Inflammatory bowel disease
Malingering
Multiple sclerosis
Reflex sympathetic dystrophy
Restless leg syndrome
Tendinitis
Thyroid disease
Syndrome of multiple chemical sensitivities

WORKUP

Section 5 of 11

Lab Studies:

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


• • CBC count: Findings are normal.
  
  
  
  • Erythrocyte sedimentation rate (ESR): The mean ESR is 15 mm/h.
  
  
  
  • Rheumatoid factor (RF): Findings are negative.
  
  
  
  • C-reactive protein and antinuclear antibody (ANA) titer: 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.
  
  
  
  • Prolactin serum levels: Findings are negative.
  
  
  
  • Electrolytes: Levels are within the reference range.
  
  
  
  • Liver function tests: Results are normal.
  
  
  
  • Muscle enzymes: Levels are within the reference range.
  
  
  
  • Purified protein derivative (PPD): Findings are negative.
  
  
  
  • Blood and urine cultures: Culture results are negative.
  
  
  
  • Thyroid function tests: Results are normal.
  
  
  
• Characteristic changes in serotonin, substance P growth hormone, and cortisol suggest autonomic and neuroendocrine system dysregulation.

Imaging Studies:

• Plain radiography including the chest, ribs, and back reveals normal findings.



• Ultrasonography of the abdomen, pelvis, and paravertebrae reveals normal findings.



• Bone scanning reveals normal findings.



• CT scanning and/or MRI study results are normal.



• Polysomnography, including PLMS assessment, which is used to evaluate possible sleep disorders, reveals normal findings.

TREATMENT

Section 6 of 11

Medical Care: Effective treatment of fibromyalgia syndrome (FMS) requires a multidisciplinary approach because of the multifaceted problems that develop. The goals of treatment are to reduce pain and depression, to decrease sleep disturbances, and to promote physical activity. In addition, a number of cognitive-behavioral interventions may help to improve the disorder. Activity is a mainstay in the treatment of FMS (see Activity).

• 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.

• Sleep: Bennett in 1997 and Tayag-Kier et al in 2000 suggested that a sleep analysis in children is helpful in determining treatable causes of sleep disturbance and periodic limb movement in sleep (PLMS). Few studies have involved children; however, low-dose tricyclic antidepressants or cyclobenzaprine has been used to help promote deeper sleep. In 2000, 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.

• Psychologic treatment


• • The use of cognitive-behavioral therapy has proven helpful in some cases. Conte et al (2003) compared children with juvenile primary FMS with healthy children and those living with arthritis. Findings showed that children and adolescents with juvenile primary FMS 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 (2005) studied 67 children with juvenile primary FMS using an educational and behavioral approach that addressed sleep difficulties, pain management, and exercise. They found significant differences in all physicals between preintervention and postintervention.
  
  
  
  • Walco and Ilowite (1992) found that the use of a cognitive-behavioral program showed improvement in symptoms over a 4- to 24-month period.

  • 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 (2002) found that children with juvenile primary FMS had higher levels of depression than children with nonmalignant back pain, possibly because of the longer time taken for those with juvenile primary FMS to receive specialty care and treatment for the problem.
  
  
  
• Pharmacologic pain control: See Medication.



• Cognitive behavioral approaches including activity and exercise: The literature supports the therapeutic use of exercise and activity as an important treatment aspect of juvenile primary FMS. A meta-analysis by Rossy et al (1999) found better outcomes with the use of cognitive-behavioral interventions and exercise than with medication. Degotardi et al (2005) studied juvenile primary FMS 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.

Surgical Care: No surgical treatment is indicated.

Consultations: Because of the multifaceted symptoms that present, refer the patient to other 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

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, at least half an hour per day, to improve cardiovascular fitness.



• The goal of an exercise regime is to improve cardiovascular health and musculoskeletal fitness through nonimpact aerobic activity.



• 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.



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



• Maintaining the child's physical conditioning is imperative in the long-term outcome of FMS.
  

MEDICATION

Section 7 of 11

Typical medication regimens for pediatric FMS primarily include skeletal muscle relaxants and low-dose tricyclic antidepressants. Some evidence reports that pain and symptom management with nonsteroidal anti-inflammatory drugs (NSAIDs) in combination with antidepressants and nonaddictive analgesics is effective. The most well-described medications used in the treatment of pediatric FMS include low-dose antidepressants, skeletal muscle relaxants, and NSAIDs. Low-dose antidepressants, such as amitriptyline (Elavil), and skeletal muscle relaxants, such as cyclobenzaprine (Flexeril), help decrease the hyperarousal mechanisms in FMS and, in turn, help the child and adolescent sleep better. Both medications are administered at bedtime or 1-2 hours before bedtime. Some debate exists in the literature as to which medication should be used initially. Some authorities, such as Gedalia et al, suggest the use of cyclobenzaprine first in treatment, while other authorities suggest beginning medication therapy with low-dose tricyclic antidepressants.

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 selective serotonin reuptake inhibitors (SSRIs) in the adult population.

Drug Category: Tricyclic antidepressants -- These 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.

Drug Name	Amitriptyline (Elavil) -- Used for analgesia for certain chronic and neuropathic pain.
Adult Dose	30-100 mg PO hs
Pediatric Dose	<2 years: Not recommended Children: 0.1 mg/kg PO qhs, may increase as tolerated over 2-3 wk to 0.5-2 mg/kg hs Adolescents: 5-40 mg qhs or 2 h before bedtime
Contraindications	Documented hypersensitivity; MAOI use in past 14 d; seizures; cardiac arrhythmias; glaucoma; urinary retention
Interactions	Phenobarbital may decrease effects; coadministration with CYP2D6 enzyme system inhibitors (eg, cimetidine, quinidine) may increase levels; inhibits hypotensive effects of guanethidine; may interact with thyroid medications, alcohol, CNS depressants, barbiturates, and disulfiram
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in cardiac conduction disturbances and history of hyperthyroidism and renal or hepatic impairment

Drug Category: Skeletal muscle relaxants -- These agents may act centrally by a selective action on the 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.

Drug Name	Cyclobenzaprine (Flexeril) -- Helps decrease the hyperarousal mechanisms in FMS and, in turn, helps the child sleep better. Is structurally related to tricyclic antidepressants and exhibits similar pharmacologic effects. Acts primarily on the CNS at the brain stem level.
Adult Dose	20-40 mg/d PO divided bid/qid; not to exceed 60 mg/d
Pediatric Dose	<15 years: Not established >15 years: 5-30 mg PO qhs
Contraindications	Documented hypersensitivity; concomitant use of MAOIs; MAOI use in last 14 d; depression; hyperthyroidism; urinary retention; cerebral palsy; QT prolongation
Interactions	Coadministration with MAOIs and tricyclic antidepressants may increase toxicity; cyclobenzaprine may have additive effect when used concurrently with anticholinergics; effects of alcohol, CNS depressants, and barbiturates may be enhanced with cyclobenzaprine
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Caution in urinary retention, angle-closure glaucoma, or increased intraocular pressure; may cause drowsiness, dizziness, and xerostomia

Drug Category: Nonsteroidal anti-inflammatory drugs -- These agents are used for their anti-inflammatory, analgesic, and antipyretic effects. They are useful for the relief of mild-to-moderate pain.

Drug Name	Ibuprofen (Motrin, Ibuprin) -- May help achieve analgesia when used in combination with skeletal muscle relaxants or tricyclic antidepressants. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
Adult Dose	200-400 mg PO q4-6h while symptoms persist; not to exceed 3.2 g/d
Pediatric Dose	4-10 mg/kg/dose PO q6-8h; not to exceed 2.4 g/d
Contraindications	Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Interactions	Coadministration with aspirin increases risk of inducing serious NSAID-related side effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in anticoagulation abnormalities or during anticoagulant therapy

Drug Name	Acetaminophen (Tylenol, Feverall, Tempra) -- DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, patients with upper GI disease, or those who are taking PO anticoagulants.
Adult Dose	325-650 mg PO q4-6h or 1000 mg tid/qid; not to exceed 4 g/d
Pediatric Dose	<12 years: 10-15 mg/kg/dose PO q4-6h prn; not to exceed 2.6 g/d >12 years: 325-650 mg PO q4h; not to exceed 5 doses in 24 h
Contraindications	Documented hypersensitivity; known G-6-P deficiency
Interactions	Rifampin can reduce analgesic effects of acetaminophen; coadministration with barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Hepatotoxicity possible with overdose or long-term high doses; severe or recurrent pain or high or continued fever may indicate a serious illness; contained in many OTC products and combined use with these products may result in cumulative doses exceeding recommended maximum dose

FOLLOW-UP

Section 8 of 11

Prognosis:

• Improvement in signs and symptoms of fibromyalgia syndrome (FMS) is likely in children and adolescents. 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. They found that 60% of the children had improved, 36% stayed the same, and 4% worsened compared with their initial presentation. Nearly all of the children needed to continue medications for up to 4 years after initial presentation.



• In 1995, Buskila and colleagues studied FMS among children aged 9-15 years. Data on 15 of the children showed that 73% (ie, 11 of the 15) no longer met criteria for FMS at 30 months’ follow-up. The mean number of TPs and the amount of force necessary to elicit pain at each point 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. Given prognostic findings, children with FMS as a whole are more likely to have a favorable outcome than adults diagnosed with FMS.

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.



• In summary, the understanding of FMS in children is still in its infancy stage; however, strides in both diagnosis and treatment modalities have progressed in the past 10 years. Because prevalence of FMS in children is increasing, diagnosing the disorder early in its course and then recommending a multidisciplinary approach to treat the child's disorder is 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.



• For excellent patient education resources, visit eMedicine's Muscle Disorders Center, Mental Health and Behavior Center, and Back, Ribs, Neck, and Head Center. Also, see eMedicine’s patient education articles Fibromyalgia, Chronic Fatigue Syndrome, Chronic Pain, and Fatigue.

TEST QUESTIONS

Section 9 of 11

CME Question 1: The 2 primary diagnostic criteria of the American College of Rheumatology (ACR) for fibromyalgia syndrome (FMS) include pain on palpation of specific tender points (TPs) and which of the following?

A: Widespread pain for 3 months in absence of other underlying causes
B: Pain aggravated by overactivity
C: Pain secondary to an injury
D: Routine laboratory test results in the reference range
E: Pain modulation by physical activities

The correct answer is A: In adults, the ACR criteria are composed of 2 primary findings, which are tenderness on palpation of 11 of 18 defined TPs and widespread pain for longer than 3 months. FMS has other associated findings, such as sleep disturbance, but these are not part of the ACR criteria.

CME Question 2: In addition to the widespread pain in pediatric fibromyalgia, children most frequently present with which of the following symptoms?

A: Raynaud phenomena
B: Female urethral syndrome
C: Joint hypermobility
D: Sleep disturbance
E: Swelling of small joints

The correct answer is D: According to studies by Gedalia and colleagues, a high percentage of children with pediatric fibromyalgia (70-96%) also have sleep disturbances. The other symptoms noted occurred less than 40% of the time in pediatric studies.

Pearl Question 1 (T/F): On average, children with fibromyalgia syndrome (FMS) have a better prognosis than adults.

The correct answer is True: Follow-up studies demonstrate that children are more likely to improve in their symptoms of FMS when under treatment than adults.

Pearl Question 2 (T/F): Fibromyalgia syndrome (FMS) has been proven to be solely psychologic in origin.

The correct answer is False: No general agreement exists regarding the cause of fibromyalgia in children. Studies have implicated possibilities such as abnormalities in muscle structure or repair, endocrine abnormalities, psychologic components, or biochemical changes in the lower spine or upper back.

Pearl Question 3 (T/F): Few laboratory study results are abnormal in a patient diagnosed with fibromyalgia syndrome (FMS).

The correct answer is True: Most laboratory tests are expected to reveal results that are within the reference range.

Pearl Question 4 (T/F): A mainstay of treatment for pediatric fibromyalgia syndrome (FMS) is regular aerobic activity.

The correct answer is True: An essential component of the treatment regimen, routine exercise consists of a moderate exercise, such as brisk walking for 20 minutes 3 times per week, and progression as tolerated.

PICTURES

Section 10 of 11

Caption: Picture 1. 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.
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BIBLIOGRAPHY

Section 11 of 11

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