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Juvenile Primary Fibromyalgia Syndrome

  • Author: Eileen R Giardino, RN, MSN, PhD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jul 06, 2016
 

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.

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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
  • 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

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]

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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]

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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.

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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.

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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

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Contributor Information and Disclosures
Author

Eileen R Giardino, RN, MSN, PhD FNP-BC, ANP-BC, Associate Professor of Nursing, Department of Family Nursing, University of Texas Health Sciences Center Houston, School of Nursing

Eileen R Giardino, RN, MSN, PhD is a member of the following medical societies: American College Health Association, American Professional Society on the Abuse of Children, American Association of Nurse Practitioners, American Nurses Association, International Society for the Prevention of Child Abuse and Neglect

Disclosure: Nothing to disclose.

Coauthor(s)

Angelo P Giardino, MD, MPH, PhD Professor and Section Head, Academic General Pediatrics, Baylor College of Medicine; Senior Vice President and Chief Quality Officer, Texas Children’s Hospital

Angelo P Giardino, MD, MPH, PhD is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Harris County Medical Society, International Society for the Prevention of Child Abuse and Neglect, Ray E Helfer Society

Disclosure: Received grant/research funds from Health Resources and Services Administration (HRSA) Integrated Community Systems for CSHCN Grant for other; Received advisory board from Baxter Healthcare Corporation for board membership.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

Herbert S Diamond, MD Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Gregory F Keenan, MD, Director of Medical Affairs, Department of Immunology, Centocor, Inc

Gregory F Keenan, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References
  1. Anthony KK, Schanberg LE. Juvenile primary fibromyalgia syndrome. Curr Rheumatol Rep. 2001 Apr. 3(2):165-71. [Medline].

  2. Bennett RM. The fibromyalgia syndrome. Textbook of Rheumatology. 5th ed. Philadelphia, PA: WB Saunders Co; 1997. 511-9.

  3. McLeod JD. Juvenile Fibromyalgia Syndrome and Improved Recognition by Pediatric Primary Care Providers. J Pediatr Health Care. 2013 Aug 26. [Medline].

  4. Yunus MB. Central sensitivity syndromes: a new paradigm and group nosology for fibromyalgia and overlapping conditions, and the related issue of disease versus illness. Semin Arthritis Rheum. 2008 Jun. 37(6):339-52. [Medline].

  5. Yunus MB. Fibromyalgia and overlapping disorders: the unifying concept of central sensitivity syndromes. Semin Arthritis Rheum. 2007 Jun. 36(6):339-56. [Medline].

  6. Wolfe F, Ross K, Anderson J, Russell IJ, Hebert L. The prevalence and characteristics of fibromyalgia in the general population. Arthritis Rheum. 1995 Jan. 38(1):19-28. [Medline].

  7. Romano TJ. Fibromyalgia in children; diagnosis and treatment. W V Med J. 1991 Mar. 87(3):112-4. [Medline].

  8. Buskila D, Press J, Gedalia A. Assessment of nonarticular tenderness and prevalence of fibromyalgia in children. J Rheumatol. 1993 Feb. 20(2):368-70. [Medline].

  9. Kashikar-Zuck S, Graham TB, Huenefeld MD, Powers SW. A review of biobehavioral research in juvenile primary fibromyalgia syndrome. Arthritis Care Res. 2000 Dec. 13(6):388-97. [Medline].

  10. Durmaz Y, Alayli G, Canbaz S, Zahiroglu Y, Bilgici A, Ilhanli I, et al. Prevalence of juvenile fibromyalgia syndrome in an urban population of Turkish adolescents: impact on depressive symptoms, quality of life and school performance. Chin Med J (Engl). 2013 Oct. 126(19):3705-3711. [Medline].

  11. Yunus MB, Masi AT. Juvenile primary fibromyalgia syndrome. A clinical study of thirty-three patients and matched normal controls. Arthritis Rheum. 1985 Feb. 28(2):138-45. [Medline].

  12. Siegel DM, Janeway D, Baum J. Fibromyalgia syndrome in children and adolescents: clinical features at presentation and status at follow-up. Pediatrics. 1998 Mar. 101(3 Pt 1):377-82. [Medline].

  13. Buskila D, Neumann L, Hershman E. Fibromyalgia syndrome in children--an outcome study. J Rheumatol. 1995 Mar. 22(3):525-8. [Medline].

  14. Gedalia A, Garcia CO, Molina JF; Bradford NJ; Espinoza LR. Fibromyalgia syndrome: experience in a pediatric rheumatology clinic. Clin Exp Rheumatol. 2000 May-Jun. 18(3):415-9. [Medline].

  15. Zernikow B, Gerhold K, Bürk G, Häuser W, Hinze CH, Hospach T, et al. [Definition, diagnosis and therapy of chronic widespread pain and so-called fibromyalgia syndrome in children and adolescents. Systematic literature review and guideline]. Schmerz. 2012 Jun. 26(3):318-30. [Medline].

  16. Olsen MN, Sherry DD, Boyne K, McCue R, Gallagher PR, Brooks LJ. Relationship between sleep and pain in adolescents with juvenile primary fibromyalgia syndrome. Sleep. 2013 Apr 1. 36(4):509-16. [Medline]. [Full Text].

  17. Tayag-Kier CE, Keenan GF, Scalzi LV. Sleep and periodic limb movement in sleep in juvenile fibromyalgia. Pediatrics. 2000 Nov. 106(5):E70. [Medline].

  18. Conte PM, Walco GA, Kimura Y. Temperament and stress response in children with juvenile primary fibromyalgia syndrome. Arthritis Rheum. 2003 Oct. 48(10):2923-30. [Medline].

  19. Neumann L, Smythe HA, Buskila D. Performance of point count and dolorimetry in assessing nonarticular tenderness in children. Arthritis and Rheumatism. 1989. 28(2):138-145.

  20. Calabro JJ. Fibromyalgia (fibrositis) in children. Am J Med. 1986 Sep 29. 81(3A):57-9. [Medline].

  21. Kashikar-Zuck S, Ting TV, Arnold LM, Bean J, Powers SW, Graham TB, et al. Cognitive behavioral therapy for the treatment of juvenile fibromyalgia: a multisite, single-blind, randomized, controlled clinical trial. Arthritis Rheum. 2012 Jan. 64(1):297-305. [Medline]. [Full Text].

  22. Goldenberg D, Mayskiy M, Mossey C, Ruthazer R, Schmid C. A randomized, double-blind crossover trial of fluoxetine and amitriptyline in the treatment of fibromyalgia. Arthritis Rheum. 1996 Nov. 39(11):1852-9. [Medline].

  23. Arnold LM, Russell IJ, Diri EW, et al. A 14-week, randomized, double-blinded, placebo-controlled monotherapy trial of pregabalin in patients with fibromyalgia. J Pain. 2008 Sep. 9(9):792-805. [Medline].

  24. Rossy LA, Buckelew SP, Dorr N, et al. A meta-analysis of fibromyalgia treatment interventions. Ann Behav Med. 1999 Spring. 21(2):180-91. [Medline].

  25. Degotardi PJ, Klass ES, Rosenberg BS, et al. Development and evaluation of a cognitive-behavioral intervention for juvenile fibromyalgia. J Pediatr Psychol. 2006 Aug. 31(7):714-23. [Medline].

  26. McLoughlin MJ, Stegner AJ, Cook DB. The relationship between physical activity and brain responses to pain in fibromyalgia. J Pain. 2011 Jun. 12(6):640-51. [Medline]. [Full Text].

  27. Sherry DD, Brake L, Tress JL, Sherker J, Fash K, Ferry K, et al. The Treatment of Juvenile Fibromyalgia with an Intensive Physical and Psychosocial Program. J Pediatr. 2015 Sep. 167 (3):731-7. [Medline].

  28. Beresford L. Juvenile Fibromyalgia: Intensive Therapy Relieves Pain. Medscape Medical News. Available at http://www.medscape.com/viewarticle/850033. August 25, 2015; Accessed: December 14, 2015.

  29. Walco GA, Ilowite NT. Cognitive-behavioral intervention for juvenile primary fibromyalgia syndrome. J Rheumatol. 1992 Oct. 19(10):1617-9. [Medline].

  30. Kashikar-Zuck S, Vaught MH, Goldschneider KR, et al. Depression, coping, and functional disability in juvenile primary fibromyalgia syndrome. J Pain. 2002 Oct. 3(5):412-9. [Medline].

  31. Tran ST, Guite JW, Pantaleao A, Pfeiffer M, Myer GD, Sil S, et al. Preliminary outcomes of a cross-site cognitive-behavioral and neuromuscular integrative training intervention for juvenile fibromyalgia. Arthritis Care Res (Hoboken). 2016 Jun 22. [Medline].

  32. Kelly JC. Fibromyalgia: CBT/Exercise Training May Get Teens Moving. Medscape Medical News. Available at http://www.medscape.com/viewarticle/865754. July 06, 2016; Accessed: July 06, 2016.

 
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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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