Juvenile Primary Fibromyalgia Syndrome Clinical Presentation

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

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 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.