Patient Presentation
A 15-year-old female came to clinic for her health supervision visit. She had recently moved to the area and was known to have fibromyalgia. She said she had already seen a local rheumatologist, psychologist and physical therapist and was happy with her care as was her mother.
The past medical history showed she had been diagnosed at age 13 after about 15 months of extremity pain, fatigue, academic/concentration problems and depressed mood. The family history was positive for hypermobility, general musculoskeletal problems, migraine, gastrointestinal issues and juvenile rheumatoid arthritis.
The pertinent physical exam showed a smiling female with normal vital signs. Her growth was 50% for weight and length. Her examination was normal except for pain in her lower extremities and wrists. There was no specific warmth or edema, and there was full range of motion and strength.
The diagnosis of of a healthy teenager with fibromyalgia was made. “I usually try hard not to complain but thought as I was meeting you, you should know where I hurt. It’s not bad today. It gets bad if I don’t exercise. If I do that then things go better. Sometimes it is too much and I have to stay home from school but that doesn’t happen a lot. I like playing softball and swimming so I do those. The girls on my old teams were super supportive. I hope the girls are here too,” she commented. “I hope they are too. If you are having more problems please let me know and then I’ll see what I can do to help,” the pediatrician offered.
Discussion
Juvenile fibromyalgia syndrome (JFM) is a “…noninflammatory condition and typically presents as a primary chronic pain disorder, but can also present as a secondary pain syndrome in patients who have an underlying autoimmune condition such as juvenile rheumatoid arthritis or systemic lupus erythematosus….it is characterized as a centralized or nociplastic (ie, altered nociception in the absense of tissue damage or lesion) pain condition.” Basically, it is a chronic, diffuse, musculoskeletal pain that is non-inflammatory and out of proportion to the stimuli. It is a clinical diagnosis and can be quite challenging as the symptoms are non-specific, can change overtime, and overlap with other diagnoses.
Patients are more often female (84%), have a family history of chronic pain (possible genetic component), and symptoms may be preceding by an environmental trigger such as illness, injury or stressful event. Prevalence is unknown for JFM but in adults it is about 2.5%. Age range is as low as 5 years, but average age when diagnosed is 15 ± 2 years for JFM. It can be difficult to diagnose JFM so the patients take longer to diagnose (average symptoms of > 1 year).
The etiology is unclear. It is hypothesized that the neuroregulation of pain is disrupted for one or many reasons including genetic variation. While externally this is a non-inflammatory condition, there could be neuroinflammation.
Pain is the promient symptom, but other cardinal symptoms include fatigue, sleep disturbance and cognition problems. Many other symptoms have also been reported by patients. JFM impacts patients’ lives and overall prognosis is that many patients have a chronic course but only a small proportion have worsening symptoms.
Other conditions to consider instead of JFM include inflammatory conditions (juvenile idiopathic arthritis), thyroid disease, inflammatory bowel disease, hypermobility, unrecognized fracture or soft tissue problem, Raynaud’s disease, myopathies and others.
Treatment is multidisciplinary. Education to better understand the condition (for example: it is real pain, movement will not make it worse), physical therapy to increase movement, flexibility and strength, and cognitive behavioral therapy to assist with coping and skill building often provide a great deal of relief. A variety of other treatments have been used including psychiatric medications to assist with anxiety/depression that patients may also have.
Learning Point
JRM is a clinical diagnosis therefore a patient should have widespread pain for a period of time (some use > 3 months), with normal lab testing and an alternative diagnosis not being appropriate. A 2016 study of adolescent female patients using the 2010 American College of Rheumatology (ACR) Adult Fibromyalgia Criteria found very high sensitivity (89.4%) and specificity (87.5%) for diagnosing JFM. “…[T]he positive predictive value was 87.5%, and the negative predictive value 89.4%. Positive and negative likelihood ratios were 7/14 and 0.12, respectively.” While there have been updated guidelines since this time, there has not been a study specifically for adolescents using these criteria.
The questionnaires below are relatively quick to use and could be helpful gathering data to help in diagnosing a patient. They can be found here.
The ACR criteria uses the Widespread Pain Index questionnaire which asks about having any pain in the last week in any of 19 body areas. One point is given for each body area affected. The criteria also use the Symptom Severity Score questionnaire which has two parts. The first part asks about how much of a problem the three cardinal symptoms of JFM of fatigue, waking still feeling tired and concentration or memory problems the patient has experienced in the past week on a 0-3 scale (0 = no problem, 3 = severe, score range = 0-9). The second part asks about having any of 40 different symptoms that the patient may have had in the past 3 months. Examples include diarrhea, rash, blurred vision, thinking problem, oral ulcers, etc. This is scored as 0 = no symptoms to 3 = a great deal of symptoms. Fibromyalgia is diagnosed if widespread pain index is ≥ 7 and symptom score is ≤ 5, OR widespread pain index = 3-6 and symptom score ≥ 9.
Tender point examination has also been used as an evaluation method and can be good. However it can be difficult to consistently perform the examination and specific pressure needs to be applied, making it easy to be inconsistent with this examination. In the study above, the authors note that tender point examination does not need to be completed to make the diagnosis. They do note that patients with JFM had more tender points than controls though (average of 11 vs 2-3, total tender points available = 18).
Questions for Further Discussion
1. What is chronic fatigue syndrome?
2. What other chronic pain syndromes have you encountered in your practice?
3. What is the criteria for diagnosing juvenile rheumatoid arthritis?
Related Cases
- Disease: Fibromyalgia
- Symptom/Presentation: Pain | Fatigue | Sleep Disturbance
- Age: Teenager
To Learn More
To view pediatric review articles on these topics from the past year check PubMed – Fibromyalgia,
and PubMed – Chronic Fatigue Syndrome.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Fibromyalgia and Chronic Pain.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Ting TV, Barnett K, Lynch-Jordan A, Whitacre C, Henrickson M, Kashikar-Zuck S. 2010 American College of Rheumatology Adult Fibromyalgia Criteria for Use in an Adolescent Female Population with Juvenile Fibromyalgia. J Pediatr. 2016;169:181-7.e1. doi:10.1016/j.jpeds.2015.10.011
Weiss JE, Kashikar-Zuck S. Juvenile Fibromyalgia. Rheum Dis Clin North Am. 2021;47(4):725-736. doi:10.1016/j.rdc.2021.07.002
Coles ML, Weissmann R, Uziel Y. Juvenile primary Fibromyalgia Syndrome: epidemiology, etiology, pathogenesis, clinical manifestations and diagnosis. Pediatr Rheumatol Online J. 2021;19(1):22. doi:10.1186/s12969-021-00493-6
Coles ML, Uziel Y. Juvenile primary fibromyalgia syndrome: A Review- Treatment and Prognosis. Pediatr Rheumatol Online J. 2021;19(1):74. doi:10.1186/s12969-021-00529-x
Author
Donna M. D’Alessandro, MD
Professor of Pediat