Juvenile non- articular rheumatic disorders (FM...)

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I have been doing some research on getting information regarding Juvenile FM and

other subjects. I found this article written by my ex rheumatologist. I found it

at www.fmily.com so I hope that it helps.

Iz

Enjoy the article everyone...

Source: Archives of Pediatrics & Adolescent Medicine, July 1996 v150 n7

p740(8).

Musculoskeletal pain syndromes that affect adolescents. Ilona S. Szer

Abstract: Diagnosing the cause of muscle and joint pain in adolescents may be

difficult. Obstacles to diagnosis include mixed physical and psychological

components and the many different potential causes. Diagnosis and treatment are

described for psychogenic rheumatism, reflex sympathetic dystrophy,

fibromyalgia, chronic fatigue syndrome, benign hypermobility syndrome, night

cramps, shin splints, various forms of inflammatory arthritis, and disorders

affecting the knee. Diagnosis demands a careful history, a thorough general

physical examination, laboratory tests as appropriate. Treatment may involve

medication, physical therapy, and psychological counselling.

Author's Abstract: COPYRIGHT American Medical Association 1996

Musculoskeletal pain is one of the most common pains of adolescence, along with

headache and abdominal pain, and arthralgia is the single most common reasons

for referral to the pediatric rheumatologist.) Not surprisingly, the pediatric

rheumatologist is frequently called to distinguish organic from functional

symptoms. During the past decade, the pediatric rheumatology community has been

evaluating increasing numbers of adolescents and preadolescents who experience

musculoskeletal symptoms presumably as a defense against emotional stress from

achievement either in academic work or in sports. To complicate the challenge

further, coexistent organic and psychologic disturbance is not rare. Clearly,

organic illness does not protect a patient from emotional pain, and it may be

most difficult to differentiate nonorganic pain in a patient with a known

organic illness. Conversely, adolescents with organic illness may use their

disease for secondary gain. Fear of misdiagnosis of phy!

sical illness as psychiatric and the notion that all of the patient's complaints

should be explained by a unifying diagnosis cause diagnostic error in both

psychogenic illness with physical manifestations and physical illness with

psychogenic symptoms.

Full Text COPYRIGHT American Medical Association 1996

While arthralgia is a subjective complaint, arthritis is defined as swelling or

limitation of motion of a joint with pain. Approximately one half of all

children and adolescents who are referred to the pediatric rheumatologist do not

have a rheumatic condition.[1] Similarly, many youths who are thought to have

arthritis suffer joint pain alone without objective symptoms. In this article,

various causes of pain encountered in adolescents will be discussed, with

particular emphasis on conditions that cause pain but have few physical

findings.

PSYCHOGENIC RHEUMATISM

Psychogenic rheumatism refers to several conditions that produce bodily pain and

disability that cannot be adequately explained by an objective assessment of the

painful site(s). The clue to any somatoform condition is the virtually universal

presence of disability out of proportion to physical findings.[2] In contrast to

adolescents with organic conditions, patients with somatoform disorders become

gradually dysfunctional; they withdraw from attending school and participating

in peer-related activities, while they are seeking medical attention from a

multitude of physicians. If the pain does not successfully excuse the adolescent

from participating in the expected function or provide secondary gain, it is

usually handled easily by the primary care physician. Unfortunately, the family

and the physician may limit the adolescent's activity or give in to the demands

of inactivity, and the rheumatologist is then confronted with a totally disabled

individual without any evidence !

of organic illness. When disability exceeds visible signs of organic disease,

the patient must be directed back to full activities. A multidisciplinary

approach that includes the family and/or admission to the hospital may be

required if there is resistance to resume normal activities. Multiple studies

have demonstrated that prolonged school absence is associated with poor later

productivity and achievement.[3]

REFLEX SYMPATHETIC DYSTROPHY (RSD)

Hysterical edema (RSD or reflex neurovascular dystrophy) is not rare in

adolescence. The important diagnostic clue is that the patient suddenly assumes

a bizarre immobile posture of an extremity that is soon accompanied by diffuse

swelling and continuous burning pain (causalgia) that is greatly intensified by

the slightest touch. The diffuse swelling is often associated with color and

temperature (trophic) changes. The patient refuses to move the affected

extremity although significant strength is required to maintain the abnormal

posture.[4]

There is great controversy over the cause and pathogenesis of RSD. In adults,

the syndrome is often associated with a minor nerve injury, but in young

patients, trauma does not explain the resultant disability although traumatic

episodes may precede the onset of RSD. Initially, results of laboratory studies

and imaging modalities are normal. With time, plain radiographs may show

demineralization (Sudeck atrophy), and ultrasonography or a bone scan may

document increased autonomic activity and abnormal (either increased or

decreased) blood flow. It is important not to misinterpret the findings from

these studies because continued investigation into possible causes will

reinforce the dysfunctional status. A prompt, confident diagnosis hastens

improvement and prevents harmful treatment and procedures.

The cause of RSD is not understood, but certain psychologic trends have been

observed.[5] Most patients with RSD are girls who are frequently involved in

multiple extracurricular activities, particularly sports. The girls are

described as " perfect children, " always compliant, and never " acting out " or

causing trouble. There is an unusual " enmeshment " with the mother and an

inability to make decisions without the mother's help. The adolescent's symptoms

are often described by the mother as " our pain. " A recent study suggested that

RSD is a conscious or unconscious effort to relieve stress by avoiding

emotionally painful activities. The patient simply cannot verbalize that she can

no longer participate in a given sport. Interestingly, the activity is often

described by the mother and daughter as the one that she loves and cannot live

without. The syndrome therefore offers a way out without the need to confront a

parent who is invested in participation but could accept nonparti!

cipation if it is secondary to illness. The family of a child with RSD is

usually stable but may be chaotic and psychologically disjointed. Marital

discord, overt or covert, is frequently present, and the enmeshment by 1 parent,

usually the mother, is almost universal.[6]

The best approach to RSD is familiarity with the disorder and a perceived

comfort level when a physician is reassuring the family. The treatment plan must

be well organized and carried out by occupational and physical therapists who

are experienced in the care of adolescents with RSD. Prohibition of the activity

from which the patient initially withdrew is essential; however, at the same

time, the patient should be enrolled in a gradual but consistent physical

therapy program and returned to school. The rehabilitation program typically

includes desensitization of the painful area, biofeedback, and other similar

modalities, as well as a strengthening and conditioning program (weight-bearing)

and a gradual return to normal physical function. If the patient is unable to

resume school attendance, is either wheelchair-bound or using crutches, an

admission may be necessary with prompt removal of all assistive devices and an

extensive therapy program with increasing weekly goals an!

d appropriate rewards. All forms of immobilization are contraindicated, the pain

is acknowledged but not solicited, and psychotherapy is provided to the entire

family. Virtually all patients respond to this form of management if the

syndrome is of less than several weeks' duration. Occasionally, the disability

is long-standing and " fixed, " and other modalities, including transcutaneous

nerve stimulation and sympathetic nerve blocks, need to be tried. Above all, the

need for long-term family and patient counseling cannot be underestimated.

FIBROMYALGIA (FIBROMYOSITIS)

Fibromyalgia is defined as a chronic musculoskeletal syndrome that is manifested

by diffuse pain and the following tender discrete anatomic points:

occiput--bilateral, at the sub-occipital muscle insertions; low

cervical--bilateral, at the anterior aspects of the intertransverse spaces at

C5-7; trapezius--bilateral, at the midpoint of the upper border;

supraspinatus--bilateral, at origins, above the scapula, near the medial border;

second rib--bilateral, at the second costochondral junctions, just lateral to

the junctions of upper surfaces; lateral epicondyle-bilateral, 2 cm distal to

the epicondyle; gluteal--bilateral, in upper outer quadrants of buttocks in

anterior fold of muscles; greater trochanter--bilateral, posterior to the

trochanteric prominence; and knee--bilateral, at the medial fat pad proximal to

the joint line. In patients with fibromyalgia, there is no evidence of synovitis

or muscle inflammation, and otherwise, findings from physical examination and

laborato!

ry or radiologic studies are normal.[7] (Pain on digital palpation must be

present in at least 11 of 18 tender point sites. ) The syndrome affects females

much more frequently than males. Other clinical features may include fatigue,

sleep disturbance, headaches, irritable bowel syndrome, parasthesias,

Raynaudlike symptoms, depression, and anxiety.

The cardinal symptom of fibromyalgia is diffuse, chronic pain. The pain often

begins in 1 location, particularly the neck or shoulders, but then becomes more

generalized. Generally, the patients state that " it hurts all over " and have

difficulty in locating the site of pain. They describe the pain as burning,

radiating, and intense. Most patients report fatigue both in the morning and at

the end of the day. In the morning, stiffness and lack of restful sleep are

prominent. Most patients report light sleep with many episodes of awakening and

difficulty in getting back to sleep. Other pain syndromes (headache and

abdominal pain) are frequent. Dysmenorrhea is also common among women with

fibromyositis. Function is greatly affected, and school absence is common and

should alert the clinician to the possibility of fibromyalgia although school

absence is common among patients with other chronic pain syndromes as well.

Results of the physical examination (save for tender trigger po!

ints) are invariably normal and confirm that the degree of disability is out of

proportion to physical findings. Although many clinicians are reluctant to

confirm the diagnosis of fibromyalgia by using tender points alone, there is

little question that this pain syndrome exists and has been objectively

documented by using dolorimetry and pressure-loaded gauges that accurately

measure force per area.

THE DIFFERENTIAL diagnosis of fibromyositis is extensive. The somewhat vague and

nebulous symptoms of chronic generalized pain and fatigue are present in many

rheumatic and nonrheumatic diseases. These conditions should be considered early

in the differential diagnosis of fibromyalgia.[8] Of these, systemic lupus

erythematosus may present with fatigue and diffuse joint or bone pain before

other clinical manifestations become obvious. The presence of specific clinical

and abnormal laboratory parameters allows the clinician to confirm relatively

easily the presence of multi-organ involvement and abnormal immune phenomena.

Juvenile rheumatoid arthritis (JRA) calls for the presence of frank arthritis

and therefore can be distinguished from fibromyalgia on clinical grounds alone.

Nevertheless, fibromyalgia may coexist with a rheumatic disorder, and palpation

of pertinent sites should be incorporated into the physical evaluation.

Enthesopathy syndrome (discussed below) may be diff!

icult to distinguish from fibromyalgia, except that it predominantly affects

adolescent boys and not girls, and the sites of enthesis are not those of tender

points. Dermatomyositis can be differentiated on the basis of characteristic

skin changes, but polymyositis and other myopathies may be difficult to

differentiate if the patient is not clinically weak. Demonstration of elevated

serum muscle enzyme levels and/or characteristic changes in an electromyogram

rule out fibromyalgia. Hypothyroidism may present with fatigue and diffuse pain;

abnormal findings from thyroid studies confirm this entity.

The most confusing conditions in the differential diagnosis include a group of

poorly understood disorders that may in fact be closely related to fibromyalgia.

These include chronic fatigue syndrome, depression, irritable bowel syndrome,

migraine, and myofascial pain syndrome. All of these may co-exist with

fibromyalgia.

Most patients with fibromyositis improve with treatment with nonsteroidal

anti-inflammatory drugs despite the lack of evidence for inflammation. Sleep

disturbance often responds to therapy with low-dose tricyclic antidepressants,

and normalization of the sleep cycle is associated with improvement in pain and

function. Muscle relaxants (eg, cyclobenzaprine hydrochloride [Flexeril]) may be

useful. Psychologic intervention is recommended for adolescents who continue to

be disabled and nonfunctional. A regular exercise program (eg, rhythmic dancing

and aquatics) may be associated with improvement.[9]

CHRONIC FATIGUE SYNDROME (CFS)

The history of CFS is quite similar to that of fibromyalgia. The causal

relationship between chronic fatigue and viral infection was reported commonly

during the polio epidemics in the 1930s and 1940s, with multiple outbreaks of

apparent postviral chronic fatigue. Despite the clinical association, extensive

serologic, virologic, and epidemiologic studies failed to demonstrate an

infectious etiology. During the mid-1980s, the concept of chronic Epstein-Barr

viral infection as a cause of CFS became popular. However, despite elevated

anti-body titers to Epstein-Barr viral antigens in many patients, there is no

evidence that Epstein-Barr virus is causally associated with the vast majority

of cases of CFS.[10]

A working definition of CFS has been developed recently to aid in diagnosis and

further studies. This definition includes (1) self-reported, and fully

evaluated, persistent or relapsing fatigue that lasts 6 or more consecutive

months and causes significant impairment of normal activities and does not

resolve with bed rest and (2) concurrent occurrence of 4 or more of the

following symptoms (all of which must have persisted or recurred during 6 or

more consecutive months of illness and must not have predated the fatigue):

self-reported impairment in short-term memory or concentration that contributes

to an inability to perform daily tasks; sore throat; tender cervical or axillary

Iymph nodes; muscle pain; polyarthralgia; headaches of a new type, pattern, or

severity; unrefreshing sleep; and postexertional malaise that lasts more than 24

hours.[11] Many of the associated symptoms appear to resemble those of

fibromyalgia.[12] A recent study demonstrated that CFS is a common rea!

son for referral of adolescents, with overrepresentation of white children from

middle and upper socioeconomic status. Whereas the natural history is favorable,

chronic fatigue resulted in major quality-of-life changes, and was associated

with a significant level of psychosocial distress.[13] Management includes

reassurance, treatment with nonsteroidal anti-inflammatory drugs, antidepressant

therapy, rehabilitation, and family therapy.

DIFFERENTIAL DIAGNOSIS OF PSYCHOGENIC RHEUMATISM

Psychogenic rheumatism is clearly a diagnosis of exclusion. Before these

conditions are considered, the clinician must rule out other causes of

musculoskeletal pain.

Benign Hypermobility Syndrome

The benign hypermobility syndrome is common in childhood and in adolescence. It

represents one of the heritable disorders of the connective tissues with Marfan

and Ehler-Danlos syndromes, which represent the more severe end of the

spectrum.[14] Adolescents with benign hypermobility syndrome tend to be girls

who excel at gymnastics. They present with chronic, diffuse joint pain that is

usually worse at the end of the day or following strenuous activity. These girls

do not withdraw from activity or school; however, because there are no abnormal

physical findings and no evidence of systemic illness, the physician may

erroneously ascribe the symptoms to psychogenic pain. Findings from laboratory

studies are normal, but results of physical examination confirm joint

hypermobility that is usually associated with skin laxity. Diagnostic criteria

for benign hypermobility syndrome call for the presence of arthralgia and 4 of 9

sites of laxity: passive bilateral knee and elbow extensio!

n beyond 180 degrees, apposition of the thumb to the forearm while the wrist is

flexed, hyperextension of the fifth metacarpophalangeal joint past 90 degrees,

and placing palms flat on the floor while bending forward with knees

extended.[15] Most authors stipulate a minimum of 10[degrees] of hyperextension

at the elbows and knees. Occasionally, laxity is complicated by recurrent

sprains, dislocations, and long-term overuse. Patients should be counseled

accordingly, and management should be supportive. Adolescents are told not to

curtail their activities unless they choose to do so. Most patients carry an

excellent prognosis and become less flexible as they get older.

Night Cramps

Many individuals complain of cramps in their legs and feet that are unrelated to

exertion and often interrupt sleep. The etiology of night cramps is unknown.

Walking on cement floors or prolonged sitting may worsen the symptom. The

diagnosis rests on an appropriate history and a pattern of pain and a

demonstration of cramped muscles.

Shin Splints

This term is applied to localized pain in the lower extremities that is

associated with prolonged walking or jogging. In mild cases, the pain may be

relieved by rest; in more severe cases, pain may be sustained.[16] Palpation of

the anterior aspect of the leg will reproduce the symptom. The syndrome is

reportedly due to small tears at the origin of involved muscles. Stress

fractures or compartment syndromes must be excluded. Shin splints may be avoided

or minimized with the use of proper footwear or orthotics and avoidance of

running on hard surfaces.

Inflammatory Conditions

Inflammatory conditions with musculoskeletal symptoms usually produce objective

findings of joint swelling and limitation of motion with pain. However,

spondyloarthropathy syndromes may present with enthesopathy (painful insertion

of tendon, ligament, or muscle into the bone) only. Spondyloarthropathies are

related to ankylosing spondylitis and share a familial predisposition,

predilection for low back pain, and inflammation in the synovial lining of

joints, tendons, and (sites of tendon insertions into bones). These conditions

are the most common cause of chronic arthritis in the adolescent age group.[17]

The characteristic pattern of joint involvement in spondyloarthritis includes

asymmetric lower-extremity joint swelling, which may follow a febrile or

diarrheal illness (reactive arthritis) or significant skeletal trauma. The most

frequently affected joint is the knee, followed by the hip; 10% to 20% of

patients initially experience metatarsophalangeal joint swelling and sacroiliac

pain with late radiologic changes (ankylosing spondylitis). Fever is not usually

present during flares of arthritis enthesitis, except in boys who, in addition

to arthritis, have conjunctivitis and urethritis (Reiter syndrome). These

patients may appear quite ill, and their condition may resemble systemic-onset

JRA. The presence of low-grade fever, weight loss, decreased linear growth, and

anemia underscore the need to search for underlying inflammatory bowel disease

even in the absence of overt gastro-intestinal symptoms (arthritis associated

with inflammatory bowel disease). Last, patients who h!

ave psoriasis or a positive family history of psoriasis may develop arthritis or

inflammation of soft tissues in the same pattern as spondyloarthritis (psoriatic

arthritis).

Most adolescents with spondyloarthritis do not have findings that are consistent

with one of the spondyloarthropathy syndromes (italicized above). Most patients

tend to have asymmetric peripheral arthritis with or without enthesopathy, a

positive family history of low back pain in the morning, and a positive HLA-B27

antigen. In the past, these boys were thought to have pauciarticular onset JRA

type II, while young girls with asymmetric arthritis of the lower extremities,

positive anti-nuclear antibody, and iritis were classified as having

pauciarticular-onset JRA type I. It has now been widely accepted that adolescent

boys do not have pauciarticularJRA, and the term seronegative arthritis and

enthesopathy syndrome has been coined to describe their illness. In addition to

the typical pattern of arthritis, with a particular predilection for the hips

and lumbosacral spine, cardiovascular lesions have been reported; the

proliferation of small blood vessels of the aortic wall, le!

ading to a subvalvular aortic ridge, is most common.

A thorough evaluation includes the measurement of low back flexion (Schober

test), which is often decreased in patients with spondyloarthritis: while the

patient stands with his or her back to the examiner, a reference point is marked

on the lower spine that corresponds to the dimples of Venus. A 15-cm span is

then measured: 10 cm above and 5 cm below the reference mark. While the patient

is instructed to bend forward and reach his or her toes with knees extended, the

15-cm span is remeasured. An increase of at least 6 cm (15-21 cm) indicates

normal low back flexion.

The typical course of sero-negative arthritis and enthesopathy syndrome is

marked by recurrent flares of synovitis, periostitis, enthesopathy, and low back

pain. The overall prognosis is excellent, with a relatively low risk of future

disability; however, some patients may develop progressive hip disease and may

need joint replacement. The risk of eventual ankylosis of the spine (ankylosing

spondylitis) appears to be low; exact data are lacking because of the relatively

recent realization that this group of disorders is indeed different from JRA.'8

The identification of HLA-B27 antigen as a susceptibility marker for the

spondyloarthrtopathy syndromes has led to the recognition of these disorders as

distinct and relatively common causes of inflammatory, musculoskeletal pain in

adolescence.

Treatment is aimed at control of pain with nonsteroidal anti-inflammatory drugs,

and physical therapy is used to maintain strength and range of motion and

prevent contractures and low back limitation.

LOCALIZED CAUSES OF MUSCULOSKELETAL PAIN

Knee pain is common in adoles cence, and syndromes that are as sociated with

knee pain deserve in dividual discussion. Intra-articular causes are usually

related to osteo chondritis dissecans or tears that in volve a discoid lateral

meniscus. In girls, the anterior knee pain syn drome and patellar maltracking

are the most common causes of iso lated knee pain. Inflammatory causes include

spondyloarthropathies and infection. Periarticular pain is usu ally due to

traction apophysitis--Osgood-Schlatter or Sinding-Larsen Johansson syndrome. A

slipped upper femoral epiphysis in boys fre quently presents with referred knee

pain and a limp, but it is less common in the adolescent age group than in

younger children. The hip should be carefully examined as loss of internal

rotation is an early sign of a slipped upper femoral epiphysis. The slip is

confirmed by radiography.

In older adolescents, particularly boys, the most common cause of knee pain is

injury. Intra-articular causes include meniscal tears and pain that is

associated with instability following a ligamentous injury. Periarticular causes

include overuse syndromes (eg, jumper's knee). Referred pain in this age group

is relatively rare.

SPECIFIC DISORDERS ASSOCIATED WITH ISOLATED KNEE PAIN

Osteochondritis Dissecans

Osteochondritis dissecans occurs most commonly in the knee joint. A fragment of

cartilage with its underlying subchondral bone becomes detached and may become

loose in the joint. Boys are affected more commonly than girls. The second

decade is the usual time of presentation. The patients describe an aching pain

that occurs during and after activity with or without locking and giving way. An

antalgic gait may be present. There is often a mild effusion and tenderness over

the site of the lesion. Wasting of the quadriceps muscles is an early sign. If

the fragment separates, symptoms of locking occur, and a loose body may be

palpable--usually in the supra-patellar pouch. sign may be positive; the

test involves straightening the internally rotated knee from a flexed

position-pain at 30[degrees] of flexion that is relieved by externally rotating

the tibia is said to be diagnostic of osteochondritis dissecans.[19]

Osgood-Schlatter Disease

Osgood-Schlatter disease is a traction apophysitis of the tibial tuberosity. It

occurs most commonly in boys (age range, 10-14 years) and is associated with

overuse.[20] The patients present with pain over the tibial tuberosity,

particularly during activity. The clinical findings are of tenderness over the

tibial tuberosity, which is often enlarged. Lateral radiographs show

characteristic fragmentation of the tibial tubercle, and there may be a loose

ossicle. The symptoms usually respond to rest and disappear at the time of

fusion of the tibial tuberosity. Occasionally, a loose ossicle remains and

causes symptoms that necessitate removal.

Sinding-Larsen-Johansson Syndrome

This is a condition similar to Os good-Schlatter disease that occurs at the

distal pole of the patella. It is a traction apophysitis and is related to

overuse. The patient complains of pain at the distal pole of the patella,

usually during exercise. The area is tender, and radiographs show fragmentation.

The condition re sponds to rest but may persist into late adolescence as

jumper's knee.

PATELLOFEMORAL DISORDERS

These disorders are common in adolescents and include patella instability

syndromes, anterior knee pain syndrome, chondromalacia patella, and plica

syndrome.[21]

Recurrent Subluxation and Dislocation

Intermittent subluxation or dislocation of the patella is particularly common in

adolescent girls. It is associated with a variety of developmental

abnormalities, including generalized or localized hypermobility, patella alta,

and hypoplasia of the lateral femoral condyle. Recurrent subluxation of the

patella is more common than dislocation and is caused by the same factors, but

the diagnosis may be more difficult to make. The majority of cases are secondary

to weakness of the vastus medialis obliquus.

Anterior Knee Pain Syndrome

Pain that arises from the anterior aspect of the knee joint is common in

adolescents. Specific conditions (eg, patella alta, osteochondritis dissecans,

Osgood-Schlatter disease, and trauma) may be responsible. However, there is a

group of patients in whom no cause for the pain can be found, despite careful

investigation. It is this idiopathic condition that should be called anterior

knee pain syndrome. It usually occurs in adolescent girls and is commonly

bilateral. The patient has pain in the anterior aspect of the joint around the

patella. It may be present during a sporting activity and necessitate its

cessation. The pain often occurs at night and may wake the patient from sleep.

In the past, this condition was wrongly labeled as " chondromalacia patella. "

Management includes strengthening of the quadriceps muscles, stretching

exercises for the hamstring muscles, and avoidance of high-heeled shoes.

Chondromalacia Patella

A diagnosis of chondromalacia patella means softening of the articular cartilage

of the patella, and can only be confirmed at arthroscopy or arthrotomy. It is a

pathologic diagnosis and should not be used to describe the clinical syndrome of

pain that arises from the anterior knee joint. However. patients with anterior

knee pain may be found to have chondromalacia patella. Chondromalacia is thought

to be due to impact loading and shearing of the articular cartilage as the

patella is compressed against the lateral femoral condyle. Articular cartilage

is poor at resisting shear forces, and repetitive impact loading and shear have

both been shown to produce cartilage softening. Idiopathic chondromalacia

patella usuallly responds to isometric strengthening of the quadriceps muscles

and stretching of the hamstring muscles.

Plica Syndrome

The medial patellar plica is the great imitator of symptoms in the knee, and can

be a diagnostic problem. It may present with pain, typically over the medial

femoral condyle, but the pain may be generalized. A snapping sensation or giving

way has also been reported. The diagnosis may be made clinically by palpating a

tender, thickened band over the medial femoral condyle; however, commonly, it is

made at arthroscopy when an inflamed plica is seen to impinge on the medial

femoral condyle. Once the plica is removed, the symptoms subside.

Osteoid Osteoma

Osteoid osteoma causes a boring or aching pain that is commonly worse at night

and may interrupt sleep. It is usually unilateral and may cause a sympathetic

effusion in a neighboring joint. The results of physical examination, laboratory

studies, and plain x-ray films may be normal, but a technetium bone scan or a

computed tomographic scan is a sensitive diagnostic tool. Osteoid osteoma is a

benign osteoblastic bone tumor, and intervention may not be necessary. The pain

is so exquisitely responsive to salicylates that pain relief with aspirin is

considered a diagnostic feature of the disease.[22]

EVALUATION OF MUSCULOSKELETAL PAIN IN ADOLESCENTS

History of Present Illness

An evaluation of an adolescent with unexplained musculoskeletal pain begins when

the physician first greets the family at the door and proceeds while the history

is being taken. This initial interaction is important for several reasons; the

interview should be focused, indicating the physician's interest in the details

of the problem. A skillful interview with a thorough approach to organization

and analysis of detail, in the effort to learn just what is wrong, indicates

that the patient is in good hands and encourages cooperation.

The history of the present illness should include all details; nothing is

considered irrelevant. Special attention is paid to signs and symptoms of

rheumatic disorders, including joint pain, swelling, limitation of motion,

stiffness, and weakness. Rashes, eye manifestations, and genitourinary and bowel

symptoms are specifically inquired about. Injury or illness before the onset of

symptoms is important in patients with RSD. History of travel may provide clues

to certain endemic conditions (eg, Lyme disease). Family history, as well as

social history, may provide important information, and school absence is a key

clue for somatoform disorders. For the pediatric rheumatologist, the history

often reveals a constellation of symptoms that seem to be mysterious to other

physicians but may represent a recognizable pattern to an experienced clinician.

An optimal history-taking interview generally provides the diagnostic

hypothesis, which is further confirmed by the results of physic!

al examination and laboratory or imaging studies.

Physical Examination

A thorough physical examination is the most important aspect of evaluation for

patients with musculoskeletal complaints. Generally, adolescents with systemic

illness appear ill, while those with somatoform of localized causes of pain look

well. The general examination may reveal diffuse lymphadenopathy or involvement

of multiple organs that suggests a systemic disease. Special attention should be

paid to the patient's nutritional status and an incremental graph of height and

weight. In addition to noting any evident rashes, the skin is searched for

dermatographism, livedo reticularis, subcutaneous nodules or swelling, changes

in dermal thickness, tightening or contractures, pigmentation, ungual or dermal

telangiectasias, nail changes, and alopecia with broken frontal hairs, as these

are common cutaneous manifestations of rheumatic illnesses that commonly affect

the adolescent population. If the history revealed the presence of Raynaud

phenomenon, fingertips should be evaluat!

ed for the presence of skin thinning, ulcerations, or slow capillary refill.

Mucous membranes should be examined for the presence of aphthous stomatitis,

which is frequently seen in young people with spondyloarthritis, systemic lupus

erythrematosus, mixed connective tissue disease, and inflammatory bowel disease.

Ulcers should also be searched for in genital areas; if found, these ulcers may

signify the presence of Behcet syndrome or Crohn disease. The evaluation of

heart and lungs must focus on the presence of arrhythmia, rubs, and murmurs. An

examination of the lungs may reveal decreased breath sounds that signify a

pleural effusion. An abdominal evaluation may reveal organomegaly, in addition

to other masses that represent a tumor or abscess, as well as localized or

diffuse tenderness. Muscle strength is ascertained quickly by placing the

adolescent in a supine position and asking him or her to lift his or her head,

to perform an unassisted sit-up, and finally to rise fro!

m a squatting position without hand assistance. Inability to perform these

maneuvers independently calls for a directed manual muscle evaluation.

The physical examinahon of the joints begins with the observation of the gait

for any sign of limping or bizarre posturing. The remainder of the examination

may be done actively by the patient or passively by the examiner. Each joint is

evaluated for swelling, warmth, redness, and range of motion. The examiner may

move his or her joint to ascertain the expected range of motion. Symptoms of

limping should be followed by measurement of both extremities to document muscle

atrophy and leg length discrepancy. It is extremely important to do a general

examination of all joints and not to limit the evaluation to the area of

complaint; it is common to discover a decreased range of motion in joints that

are affected by inflammation but not producing symptoms or to find diffuse

hypermobility in a patient who has a localized complaint. The examination of the

back should include palpation of the spine and the sacroiliac joints, as well as

an evaluation of low back flexion, particularly !

in adolescent boys who may have spondyloarthritis. The back and extremities are

then palpated for the presence of " tender " points (Figure) and enthesopathy.

Laboratory Evaluation

Laboratory evaluation of adolescents with musculoskeletal complaints should be

tailored to the intensity and duration of symptoms. If the findings from the

physical examination are abnormal, the cause of the complaint may be obvious and

not require any laboratory studies. If the cause of illness is not clear for the

examination alone, screening laboratory studies should be performed, including a

complete blood cell count, chemistry panel with liver and renal function, and

routine urinalysis. Adolescents whose primary complaint is fatigue may need to

be evaluated for thyroid dysfunction and chronic viral infections (Epstein-Barr

virus). Determination of the antinuclear antibody should be done in adolescents

whose constitutional symptoms and multiorgan involvement are usually evident in

the initial screening tests. An isolated positive antinuclear antibody in an

adolescent who presents with fatigue and no other abnormal objective symptoms or

findings does not correlate with a !

future development of systemic lupus erythematosus.[23] Such patients are often

referred for extensive evaluations that can be avoided. Imaging modalities

should be utilized in patients with isolated or objective findings. It is

important to keep in mind that repeating a negative evaluation usually does not

produce any useful information. Once the workup has been done, intervention can

be initiated. This is of particular importance in somatoform disorders that must

be treated promptly and confidently.

WHEN TO REFER

Referral to other consultants is dictated by the intensity of symptoms and the

presence of associated disability. Adolescents who are out of school despite the

primary physician's intervention should be referred promptly because they may

require an admission for rehabilitation under the direction of an experienced

rheumatologist.

Accepted for publication December 19, 1995.

Corresponding author: llona S. Szer, MD, Division of Pediatric Rheumatology,

Children's Hospital of San Diego, 3030 Children's Way, Suite 202, San Diego, CA

92123-4227.

[Figure ILLUSTRATION OMITTED]

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