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CURRENT Diagnosis & Treatment: Pediatrics > Chapter 27. Rheumatic Diseases >

Juvenile Idiopathic Arthritis

Essentials of Diagnosis & Typical Features

� Arthritis, involving pain, swelling, warmth, tenderness, morning stiffness, and decreased

range of motion of one or more joints, lasting at least 6–12 weeks.

� May have associated systemic manifestations, including fever, rash, uveitis, serositis,

anemia, and fatigue.

Juvenile idiopathic arthritis (JIA) is characterized by chronic arthritis in one or more joints for at

least 6–12 weeks. There are four main subtypes of JIA: oligoarticular, polyarticular, systemic, and

enthesitis-associated. The exact cause of JIA is not known, but there is substantial evidence that it

is an autoimmune process with genetic susceptibility factors.

Clinical Findings

Symptoms and Signs

The most common type of JIA is the oligoarticular form, which constitutes 50% of patients and is

characterized by arthritis of four or fewer joints. This type of JIA often affects medium to large

joints. Because the arthritis is often asymmetrical, children may develop a leg-length discrepancy in

which the involved leg grows longer due to increased blood flow and growth factors. The synovitis is

usually mild and may be painless. Systemic features are uncommon except for inflammation in the

eye. Up to 30% of children with this type of JIA develop insidious, asymptomatic uveitis, which may

cause blindness if untreated. The activity of the eye disease does not correlate with that of the

arthritis. Therefore, routine ophthalmologic screening with slit-lamp examination must be performed

at 3-month intervals if the antinuclear antibody (ANA) test is positive, and at 6-month intervals if

the ANA test is negative, for at least 4 years after the onset of arthritis, as this is the period of

highest risk.

Polyarticular disease is defined as arthritis involving five or more joints. This type of JIA affects 35%

of patients. Both large and small joints are involved, typically in a symmetrical pattern. Systemic

features are not prominent, although low-grade fever, fatigue, rheumatoid nodules, and anemia

may be present. This group is further divided into rheumatoid factor–positive and rheumatoid

factor–negative disease. The former resembles adult rheumatoid arthritis with more chronic,

destructive arthritis.

The systemic form, also known as Still disease, is the least common form, comprising 10–15% of

patients with JIA. The arthritis can involve any number of joints and affects both large and small

joints, but may be absent at disease onset. One of the classic features is a high fever, often as high

as 39–40°C, typically occurring one to two times per day. In between fever spikes, the temperature

usually returns to normal or subnormal. Ninety percent of patients have a characteristic evanescent,

salmon-pink macular rash that is most prominent on pressure areas and when fever is present.

Other systemic features that may be present, but are not specific for JIA, include

hepatosplenomegaly, lymphadenopathy, leukocytosis, and serositis.

Enthesitis-associated arthritis is most common in males, older than 10 years of age, and is typically

associated with lower extremity, large joint arthritis. The hallmark of this form is inflammation of

tendinous insertions (enthesopathy), such as the tibial tubercle or the heel. Low back pain and

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sacroiliitis are also commonly seen in this form of arthritis.

Laboratory Findings

There is no diagnostic test for JIA. A normal erythrocyte sedimentation rate (ESR) does not exclude

the diagnosis of JIA. However, patients with systemic JIA typically have significantly elevated

markers of inflammation, including ESR, C-reactive protein (CRP), white blood cell count, and

platelets. Rheumatoid factor is positive in about 10–15% of patients and usually when onset of

polyarticular disease occurs after age 8 years. A newer test, anti–cyclic citrullinated peptide (CCP)

antibody, may be detectable prior to the rheumatoid factor and has a very high specificity for

rheumatoid arthritis. ANAs are associated with an increased risk of iridocyclitis in patients with

oligoarticular disease. A positive ANA test is also fairly common in patients with the late-onset

rheumatoid factor–positive form of the disease. Carriage of HLA-B27 antigen is associated with an

increased risk of developing enthesitis-associated arthritis.

Table 27–1 lists the general characteristics of joint fluid in various conditions. The main indication

for joint aspiration and synovial fluid analysis is to rule out infection. A positive Gram stain or

culture is the only definitive test for infection. A leukocyte count over 2000/ L suggests

inflammation; this may be due to infection, rheumatologic diseases, leukemia, or reactive arthritis.

A very low glucose concentration (< 40 mg/dL) or very high polymorphonuclear leukocyte count (>

60,000/ L) is highly suggestive of bacterial arthritis.

Imaging Studies

In the early stages of the disease, only soft tissue swelling and periarticular osteoporosis may be

seen. Magnetic resonance imaging (MRI) of involved joints may show early joint damage and, if

obtained with gadolinium, can confirm the presence of synovitis. Later in the course of the disease,

particularly in patients with rheumatoid factor–positive disease, plain films may demonstrate joint

space narrowing due to cartilage thinning and erosive changes of the bone related to chronic

inflammation.

Differential Diagnosis

Table 27–2 lists the most common causes of limb pain in childhood. JIA is a diagnosis of exclusion;

therefore, it is important to rule out other causes of the clinical signs and symptoms prior to settling

on this diagnosis. The differential diagnosis is often quite broad, including orthopedic conditions,

infectious diseases, and malignancies. A few key features can help distinguish these different

entities, including the timing of the pain and associated signs and symptoms. In inflammatory

conditions, patients frequently have increased symptoms in the morning with associated stiffness.

In contrast, patients with an orthopedic abnormality typically have increased symptoms later in the

day and with activity. Growing pains, a common cause of leg pain in childhood, are characterized by

poorly localized pain at night, which frequently wakes the child from sleep; no objective signs of

Table 27–1. Joint fluid analysis.

Disorder Cells/ L Glucosea

Trauma More red cells than white cells;

usually < 2000 white cells

Normal

Reactive arthritis 3000–10,000 white cells, mostly

mononuclear cells

Normal

Juvenile idiopathic arthritis and other

inflammatory arthritides

5000–60,000 white cells, mostly

neutrophils

Usually normal or

slightly low

Septic arthritis >60,000 white cells, >90%

neutrophils

Low to normal

aNormal value is 75% of the serum glucose value.



inflammation; and no daytime symptoms. Patients with growing pains often ask to be massaged,

which is not typical of those with arthritis.

Table 27–2. Differential diagnosis of limb pain in children.

Orthopedic

Stress fracture

Chondromalacia patellae

Osgood-Schlatter disease

Slipped capital femoral epiphysis

Legg-Calvé-Perthes disease

Hypermobility syndrome

Reactive arthritis

Henoch-Schönlein purpura

Toxic synovitis of the hip

Transient synovitis following viral infection

Rheumatic fever

Poststreptococcal arthritis

Infections

Bacterial

Lyme arthritis

Osteomyelitis

Septic arthritis

Discitis

Viral

Parvovirus

Epstein-Barr virus

Hepatitis B arthritis

Rheumatologic

Juvenile idiopathic arthritis

Systemic lupus erythematosus

Dermatomyositis

Neoplastic

Leukemia

Lymphoma

Neuroblastoma

Osteoid osteoma

Bone tumors (benign or malignant)

Pain syndromes

Growing pains

Fibromyalgia



It is particularly important to establish the diagnosis in the case of monoarticular arthritis. Bacterial

arthritis is usually acute and monoarticular except for arthritis associated with gonorrhea, which

may be associated with a migratory pattern. Fever, leukocytosis, and increased ESR with an acute

process in a single joint demand synovial fluid examination and culture to identify the pathogen.

Pain in the hip or lower extremity is a frequent symptom of childhood cancer, especially leukemia,

neuroblastoma, and rhabdomyosarcoma. Infiltration of bone by tumor and a joint effusion may be

seen. Radiographs of the affected site and examination of the blood smear for unusual cells and

thrombocytopenia are necessary. An elevated lactate dehydrogenase value should also raise

concern about an underlying neoplastic process. In doubtful cases, bone marrow examination is

indicated.

In cases of reactive arthritis, a preceding illness is identified in approximately half of cases. Patients

often have acute onset of arthritis, and there may be a migratory pattern. The duration of

symptoms is a very important distinction between reactive arthritides and JIA. Symptoms

associated with reactive arthritis typically resolve within 4–6 weeks. In contrast, to meet criteria for

chronic arthritis, symptoms must be present for at least 6–12 weeks.

The arthritis of rheumatic fever is migratory, transient, and often more painful than that of JIA.

Rheumatic fever is very rare in children younger than 5 years of age. In suspected cases, evidence

of rheumatic carditis should be sought based on examination and electrocardiographic findings.

Evidence of recent streptococcal infection is essential to the diagnosis. The fever pattern in

rheumatic fever is low grade and persistent compared with the spiking fever that characterizes the

systemic form of JIA. Lyme arthritis resembles oligoarticular JIA, but the former occurs as discrete,

recurrent episodes of arthritis lasting 2–6 weeks. For patients suspected of having Lyme disease,

testing for antibodies against Borrelia burgdorferi should be performed, with confirmatory testing by

Western blot.

Treatment

The objectives of therapy are to restore function, relieve pain, maintain joint motion, and prevent

damage to cartilage and bone.

Nonsteroidal Anti-Inflammatory Medications

First-line therapy is nonsteroidal anti-inflammatory drugs (NSAIDs). A wide range of agents is

available but only a few are approved for use in children, including naproxen (10 mg/kg per dose

twice daily), ibuprofen (10 mg/kg per dose three to four times daily), and meloxicam (0.125 mg/kg

once daily). NSAIDs are generally well tolerated in children, as long as they are taken with food.

The average time to symptomatic improvement is 1 month, but in some patients a response is not

seen for 8–12 weeks.

Disease-Modifying and Biologic Agents

For patients with JIA who fail to respond to NSAIDs, weekly methotrexate is the second-line

medication of choice. Symptomatic response usually begins within 3–4 weeks. The low dosages

used (5–10 mg/m2/wk or 1 mg/kg/wk as a single dose) are generally well-tolerated. Potential side

effects include nausea, vomiting, hair thinning, stomatitis, bone marrow suppression, and

hepatotoxicity. A complete blood count and liver function tests should be obtained every 2–3

months. Several additional disease-modifying agents are available for use in patients with

persistently active disease or those intolerant to methotrexate. Leflunomide is an antipyrimidine

medication that has been shown to be as effective as methotrexate. Side effects may include

diarrhea and alopecia. Medications that inhibit tumor necrosis factor, a cytokine known to play an

important role in the pathogenesis of JIA, include etanercept, infliximab, and adalimumab. These

Complex regional pain syndrome



drugs are generally quite effective in controlling disease and preventing cartilage and bone damage,

and have been associated with healing based on radiologic changes. However, their potential long-

term effects are unknown, and they are very expensive and require parenteral administration.

Newer biologic agents, including anakinra, rituximab, and abatacept, have demonstrated some

preliminary efficacy in patients who have not responded to other treatments.

Corticosteroids

Steroids are reserved for children with severe involvement, primarily patients with systemic disease.

Local steroid joint injections may be helpful in patients who have arthritis in one or a few joints.

Triamcinolone hexacetonide is a long-acting steroid that can be used for injections and is often

associated with at least several months of disease control.

Uveitis

Iridocyclitis should be closely monitored by an ophthalmologist. Typically treatment is initiated with

corticosteroid eye drops and dilating agents to prevent scarring between the iris and the lens. In

patients who fail topical treatments, methotrexate, cyclosporine, and infliximab may be used.

Rehabilitation

Physical and occupational therapies are important to focus on range of motion, stretching, and

strengthening. These exercises, as well as other modalities such as heat, water therapy, and

ultrasound, can help control pain, maintain and restore function, and prevent deformity and

disability. Young children with oligoarticular disease affecting asymmetrical lower extremity joints

can develop a leg-length discrepancy, which may require treatment with a shoe lift on the

unaffected side.

Prognosis

The course and prognosis for JIA is variable, depending on the subtype of disease. Overall, the

prognosis is good; 75–80% of patients remit without serious disability. In children with extended

oligoarticular and polyarticular disease, more joints are involved; these patients may have more

persistent and severe disease. Patients who are rheumatoid factor–positive are at highest risk for

chronic, erosive arthritis that may continue into adulthood. The systemic features associated with

systemic arthritis tend to remit within months to years. The prognosis in systemic disease is worse

in patients with persistent systemic disease after 6 months, thrombocytosis and more extensive

arthritis.

Gensler L, Davis JC: Recognition and treatment of juvenile-onset spondyloarthritis. Curr Opin

Rheumatol 2006;18:507. [PMID: 16896291] [Full Text]

Goldmuntz EA, White PH: Juvenile idiopathic arthritis: A review for the pediatrician. Pediatr Rev

2006;27:e24. [PMID: 16581950] [Full Text]

Hayward K, Wallace CA: Recent developments in anti-rheumatic drugs in pediatrics: Treatment of

juvenile idiopathic arthritis. Arthritis Res Ther 2009;11:216. [PMID: 19291269] [Full Text]

Ravelli A, Martini A: Juvenile idiopathic arthritis. Lancet 2007;369:767. [PMID: 17336654] [Full

Text]

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16651274] [Full Text]

Systemic Lupus Erythematosus


� Multisystem inflammatory disease of the joints, serosal linings, skin, kidneys, blood, and

central nervous system.



� Autoantibodies such as ANA, double-stranded DNA, and anti-Smith antibodies are present

and related to the pathogenesis of disease.

Pathogenesis

Systemic lupus erythematosus (SLE) is the prototype of immune complex diseases; its pathogenesis

is related to the formation of antibody-antigen complexes that exist in the circulation and deposit in

the involved tissues. The spectrum of symptoms is due to tissue-specific autoantibodies, as well as

damage to the tissue by lymphocytes, neutrophils, and complement evoked by the deposition of

immune complexes. Autoreactive T lymphocytes that have escaped clonal deletion and unregulated

B-lymphocyte production of autoantibodies may initiate the disease.

Clinical Findings

Symptoms and Signs

The onset of pediatric SLE is most common in girls between the ages of 9 and 15 years. Signs and

symptoms depend on the organs affected by immune complex deposition. The American College of

Rheumatology has established criteria to aid in the diagnosis of SLE; four of the following 11 criteria

are necessary to establish the diagnosis:

1. Malar rash—photosensitive, so-called butterfly rash on the cheeks and nasal bridge

2. Discoid rash—annular, scaly rash on the scalp, face, and extremities that can lead to scarring

3. Photosensitivity—increased rash or other disease symptoms in response to sunlight exposure

4. Mucous membrane ulcers—painless ulcers on the hard palate or nasal septum (or both)

5. Arthritis—nonerosive arthritis of large and small joints, typically in a symmetrical distribution

6. Serositis—pericarditis or pleuritis (or both), often associated with chest pain and difficulty

breathing

7. Renal abnormalities—proteinuria (> 0.5 g/d) or cellular casts (or both)

8. Neurologic abnormalities—seizures or psychosis (or both)

9. Blood count abnormalities—low white blood cell count (< 4000/mm3), Coombs test–positive

anemia, and/or thrombocytopenia (< 100,000/mm3)

10. Positive ANA—seen in almost 100% of patients with SLE

11. Autoantibodies—positive double-stranded DNA antibody, anti-Smith antibody, anticardiolipin

antibodies, lupus anticoagulant, or false-positive blood test for syphilis

Other common signs and symptoms include fever, fatigue, weight loss, anorexia, Raynaud

phenomenon, myositis, vasculitis, chorea, neuropathies, depression, and cognitive changes.

Laboratory Findings

Complete blood count abnormalities are common, including leukopenia, anemia, and

thrombocytopenia. Approximately 15% of patients are Coombs test–positive, but many patients

develop anemia due to other causes, including chronic disease and blood loss. Patients with

significant renal involvement may have electrolyte disturbances, elevated kidney function tests, and

hypoalbuminemia. The ESR is frequently elevated during active disease. In contrast, many patients

with active SLE have a normal CRP. When the CRP is elevated, it is important to investigate possible

infectious causes, particularly bacterial infections. It is critical to monitor the urinalysis in patients

with SLE for proteinuria and hematuria, as the renal disease may be otherwise clinically silent. In

immune complex diseases, complement is consumed; therefore, levels of C3 and C4 are depressed

with active disease.



The ANA test is positive in almost 100% of patients, usually at titers of 1:320 or above. In patients

with suspected SLE, it is important to obtain a full ANA profile—including antibodies directed against

double-stranded DNA, Smith, ribonucleic protein, and Sjogren's specific antibody A and B

antibodies—to better characterize their serologic markers of disease. Because approximately 50–

60% of pediatric SLE patients have antiphospholipid antibodies and are therefore at increased risk

of thrombosis, it is important to screen all patients with SLE for anticardiolipin antibodies and lupus

anticoagulant.

Differential Diagnosis

Because there is such a wide spectrum of disease with SLE, the differential diagnosis is quite broad,

including systemic JIA, mixed connective tissue disease (MCTD), rheumatic fever, vasculitis,

malignancies, and bacterial and viral infections. A negative ANA test essentially excludes the

diagnosis of SLE. Anti–double-stranded DNA and Smith antibodies are very specific for SLE. The

preceding diagnostic criteria, which are very helpful in establishing the diagnosis of SLE, have a

specificity and sensitivity of 96%.

MCTD, an overlap syndrome with features of several collagen-vascular diseases, shares many

features with SLE. The symptom complex is diverse and often includes arthritis, fever, skin

tightening, Raynaud phenomenon, muscle weakness, and rash. The ANA test is typically positive in

very high titers. The ANA profile is negative except for antibodies directed against ribonucleic

protein.

Treatment

The treatment of SLE should be tailored to the organ system involved so that toxicities may be

minimized. Prednisone is the mainstay of treatment and has significantly lowered the mortality rate

in SLE. Patients with severe, life-threatening, or organ-threatening disease are typically treated with

intravenous pulse methylprednisolone, 30 mg/kg per dose (maximum of 1000 mg) daily for 3 days,

and then switched to 2 mg/kg/d of prednisone. The dosage should be adjusted using clinical and

laboratory parameters of disease activity, and the minimum amount of corticosteroid to control the

disease should be used. Skin manifestations, arthritis, and fatigue may be treated with antimalarials

such as hydroxychloroquine, 5–7 mg/kg/d orally. Pleuritic pain or arthritis can often be managed

with NSAIDs.

If disease control is inadequate with prednisone or if the dose required produces intolerable side

effects, a steroid-sparing agent, such as mycophenolate mofetil, azathioprine, or cyclophosphamide,

should be added. More recently, rituximab, a monoclonal antibody directed against CD20, has been

used for persistent active disease, particularly in patients with hematologic manifestations. Patients

who have evidence of antiphospholipid antibodies should be treated with a baby aspirin every day to

help prevent thrombosis. Thrombotic events due to these clotting antibodies require long-term

anticoagulation.

The toxicities of the regimens must be carefully considered. Growth failure, osteoporosis, Cushing

syndrome, adrenal suppression, and aseptic necrosis are serious side effects of chronic use of

prednisone. When high doses of corticosteroids are used (> 2 mg/kg/d), there is a high risk of

infection. Cyclophosphamide can cause bladder epithelial dysplasia, hemorrhagic cystitis, and

sterility. Azathioprine has been associated with liver damage and bone marrow suppression.

Immunosuppressant treatment should be withheld if the total white blood cell count falls below

3000/ L or the neutrophil count falls below 1000/ L. Retinal damage from hydroxychloroquine has

not been observed with recommended dosages.

Prognosis

The prognosis in SLE relates to the presence of renal involvement or infectious complications of

treatment. Nonetheless, the survival rate has improved from 51% at 5 years in 1954 to 90% today.



The disease has a natural waxing and waning cycle; the disease may flare at any time and

spontaneous remission may rarely occur.

Gottlieb BS, Ilowite NT: Systemic lupus erythematosus in children and adolescents. Pediatr Rev

2006;27:323. [PMID: 16950937] [Full Text]

Ravelli A et al: Outcome in juvenile onset systemic lupus erythematosus. Curr Opin Rheumat

2005;17:568. [PMID: 16093835] [Full Text]

Tucker LB: Making the diagnosis of systemic lupus erythematosus in children and adolescents.

Lupus 2007;16:546. [PMID: 17711886] [Full Text]

Dermatomyositis


� Pathognomonic skin rashes.

� Weakness of proximal muscles and occasionally of pharyngeal and laryngeal groups.

� Pathogenesis related to vasculitis.

Clinical Findings

Symptoms and Signs

The predominant symptom is proximal muscle weakness, particularly affecting pelvic and shoulder

girdle muscles. Tenderness, stiffness, and swelling may be found. Pharyngeal involvement,

manifested as voice changes and difficulty swallowing, is associated with an increased risk of

aspiration. Intestinal vasculitis can be associated with ulceration and perforation of involved areas.

Flexion contractures and muscle atrophy may produce significant residual deformities. Calcinosis

may follow the inflammation in muscle and skin.

Several characteristic rashes are seen in dermatomyositis. Patients often have a heliotrope rash

with a reddish-purple hue on the upper eyelids, along with a malar rash that may be accompanied

by edema of the eyelids and face. Gottron papules are shiny, erythematous, scaly plaques on the

extensor surfaces of the knuckles, elbows, and knees. Nail-fold abnormalities, including dilation,

thrombosis, and dropout of periungual capillaries, may identify patients with a worse prognosis.

Laboratory Findings/Imaging Studies/Special Tests

Determination of muscle enzyme levels, including aspartate aminotransferase, alanine

aminotransferase, lactate dehydrogenase, creatine phosphokinase, and aldolase, is helpful in

confirming the diagnosis, assessing disease activity, and monitoring the response to treatment.

Even in the face of extensive muscle inflammation, the ESR and CRP are frequently normal. An MRI

scan of the quadriceps muscle can be used in equivocal cases to confirm the presence of

inflammatory myositis. Electromyography is useful to distinguish myopathic from neuropathic

causes of muscle weakness. Muscle biopsy is indicated in cases of myositis without the

pathognomonic rash.

Treatment

Treatment is aimed at suppression of the inflammatory response and prevention of the loss of

muscle function and joint range of motion. Acutely, it is very important to assess the adequacy of

the ventilatory effort and swallowing and to rule out intestinal vasculitis. Corticosteroids are the

initial therapy of choice. Treatment is usually initiated with prednisone, 2 mg/kg/d, and continued

until signs and symptoms of active disease are controlled; the dosage is then gradually tapered. In

severe cases, intravenous pulse methylprednisolone for 3 days is indicated. Therapy is guided by

the physical examination findings and muscle enzyme values. Steroid therapy is generally

maintained at the lowest dose possible for at least 2 years to minimize the risk of exacerbations and



calcinosis. If patients continue to have active disease, additional steroid-sparing agents, such as

methotrexate, cyclosporine, and, in severe cases, cyclophosphamide, should be started.

Hydroxychloroquine and intravenous immunoglobulin are particularly helpful in managing the skin

manifestations. As the rashes are photosensitive, sun protection is very important. Physical and

occupational therapy should be initiated early in the course of disease. Initially, passive range-of-

motion exercises are performed to prevent loss of motion. Later, once the muscle enzymes have

normalized, a graduated program of stretching and strengthening exercises is introduced to restore

normal strength and function.

Prognosis

Most patients have a monocyclic course; 10–20% of patients have more chronic or recurrent

symptoms. Factors that influence the outcome include the rapidity of symptom onset, extent of

weakness, presence of cutaneous or gastrointestinal vasculitis, timeliness of diagnosis, initiation of

therapy, and response to treatment. Dermatomyositis in children is not associated with an increased

risk of cancer as it is in adults.

Feldman BM et al: Juvenile dermatomyositis and other idiopathic inflammatory myopathies of

childhood. Lancet 2008;371:2201. [PMID: 18586175] [Full Text]

McCann LJ et al; Juvenile Dermatomyositis Research Group: The Juvenile Dermatomyositis National

Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the

first 5 yr. Rheumatology (Oxford) 2006;45:1255. [PMID: 16567354] [Full Text]

Stringer E, Feldman BM: Advances in the treatment of juvenile dermatomyositis. Curr Opin

Rheumatol 2006;18:503. [PMID: 16896290] [Full Text]

Raynaud Phenomenon

Raynaud phenomenon is an intermittent vasospastic disorder of the extremities. As much as 10% of

the adult population has this disorder, and onset in childhood is not uncommon. The classic triphasic

presentation is cold-induced pallor, then cyanosis, followed by hyperemia, but incomplete forms are

frequent. In adults older than 35 years who are ANA-positive, Raynaud phenomenon may be a

harbinger of rheumatic disease. This progression is rarely seen in childhood. Evaluation should

include a detailed history with review of systems relevant to rheumatic disease and examination for

nail-fold capillary abnormalities. In the absence of positive findings, Raynaud phenomenon is likely

to be idiopathic.

Treatment involves education about keeping the extremities and core body warm and the role of

stress, which may be a precipitant. In very symptomatic patients, treatment with calcium channel

blockers such as nifedipine can be effective.

Nigrovic PA et al: Raynaud's phenomenon in children: A retrospective review of 123 patients.

Pediatrics 2003;111:715. [PMID: 12671102] [Full Text]

Pavlov-Dolijanovié S et al: The prognostic value of nailfold capillary changes for the development of

connective tissue disease in children and adolescents with primary Raynaud phenomenon: A follow-

up study of 250 patients. Pediatr Dermatol 2006;23:437. [PMID: 17014637] [Full Text]

Pope JE: The diagnosis and treatment of Raynaud's phenomenon: A practical approach. Drugs

2007;67:57. [PMID: 17352512] [Full Text]

Noninflammatory Pain Syndromes

COMPLEX REGIONAL PAIN SYNDROME

Complex regional pain syndrome, previously known as reflex sympathetic dystrophy, is a painful



condition that is frequently confused with arthritis. Prevalence and recognition of the condition

appear to be increasing. Severe extremity pain leading to nearly complete loss of function is the

hallmark of the condition. Evidence of autonomic dysfunction is demonstrated by pallor or cyanosis,

temperature differences (with the affected extremity cooler than surrounding areas), and

generalized swelling. On examination, marked cutaneous hyperesthesia to even the slightest touch

is evident. Results of laboratory tests are normal, without evidence of systemic inflammation.

Radiographic findings are normal except for late development of osteoporosis. Bone scans may be

helpful and may demonstrate either increased or decreased blood flow to the painful extremity.

The cause of this condition remains elusive. Treatment includes physical therapy to focus on

restoration of function, maintenance of range of motion, and pain relief. NSAIDs can be helpful for

pain control, and in patients with more chronic disease, gabapentin is frequently effective.

Persistent disease may respond to local nerve blocks. Counseling is helpful to identify potential

psychosocial stressors and to assist with pain management. Long-term prognosis is good if recovery

is rapid; recurrent episodes imply a less favorable prognosis.

FIBROMYALGIA

Fibromyalgia is a chronic pain syndrome characterized by diffuse musculoskeletal pain, fatigue,

sleep disturbance, and chronic headaches. Weather changes, fatigue, and stress exacerbate

symptoms. Patients have normal examination findings except for characteristic trigger points at the

insertion of muscles, especially along the neck, spine, and pelvis.

Treatment centers on physical therapy, non-narcotic pain medications, improving sleep, and

counseling. Low-dose amitriptyline or trazodone can help with sleep and may produce remarkable

reduction in pain. Physical therapy should emphasize a graded rehabilitative approach to stretching

and exercise and promote regular aerobic exercise. Pregabalin recently became the first medication

to be approved by the Food and Drug Administration for the treatment of fibromyalgia. Use of the

drug is associated with decreased pain in adults with fibromyalgia, and future studies are planned to

test the safety and efficacy of its use in children with the condition. The prognosis for children with

fibromyalgia is not clear, and long-term strategies may be necessary to enable them to cope with

the condition.

HYPERMOBILITY SYNDROME

Ligamentous laxity, which previously was thought to occur only in Ehlers-Danlos syndrome or Down

syndrome, is now recognized as a common cause of joint pain. Patients with hypermobility present

with episodic joint pain and occasionally with swelling that lasts a few days after increased physical

activity. Depending on the activity, almost any joint may be affected. Five criteria have been

established: (1) passive opposition of the thumb to the flexor surface of the forearm, (2) passive

hyperextension of the fingers so that they are parallel to the extensor surface of the forearm, (3)

Tan EC et al: Complex regional pain syndrome type I in children. Acta Paediatr 2008;97:848.

[PMID: 18410465] [Full Text]

Wilder RT: Management of pediatric patients with complex regional pain syndrome. Clin J Pain

2006;22:443. [PMID: 16772799] [Full Text]

Buskila D: Pediatric fibromyalgia. Rheum Dis Clin North Am 2009;35:253. [PMID: 19647140] [Full

Text]

Crofford LJ et al: Pregabalin for the treatment of fibromyalgia syndrome: Results of a randomized,

double-blind, placebo-controlled trial. Arthritis Rheum 2005;52:1264. [PMID: 15818684] [Full Text]

Degotardi PJ et al: Development and evaluation of a cognitive-behavioral intervention for juvenile

fibromyalgia. J Pediatr Psychol 2006;31:714. [PMID: 16120766] [Full Text]



hyperextension of the elbow, (4) hyperextension of the knee, and (5) palms on floor with knees

extended. Results of laboratory tests are normal. The pain associated with the syndrome is

produced by improper joint alignment caused by the laxity during exercise. Treatment consists of a

graded conditioning program designed to provide muscular support of the joints to compensate for

the loose ligaments and to train patients on how to protect their joints from hyperextension.

Adib N et al: Joint hypermobility syndrome in childhood. A not so benign multisystem disorder?

Rheumatology (Oxford) 2005; 44:744. [PMID: 15728418] [Full Text]

Tofts LJ et al: The differential diagnosis of children with joint hypermobility: A review of the

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