APPROACH TO THE CHILD WITH JOINT PAINS
A BRIEF REVIEW OF THE CHILDHOOD RHEUMATIC DISEASES
FOR PHYSICIANS
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Dr. Lehman is the
author of many textbook chapters and articles on the care of children and young
adults with SLE. He practices in
Much
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Because children are often poor historians the examining physician must have a clear knowledge of the differential diagnosis when he approaches a child with limb pain. Nonetheless a careful history and physical examination are key to establishing the correct diagnosis. A useful algorithm is illustrated in Figure 1. The examining physician must determine whether objective inflammation is present (pain, swelling, warmth, or limitation of motion), and if inflammation is present whether it is articular or periarticular.
A. No inflammation:
1. Growing pains: the most common and most misused diagnosis for
musculoskeletal pain in childhood. True "growing pains" occur in
young children peaking at four to five years of age. The pain classically
occurs in the popliteal fossa.
It is relieved by gentle massage or reassurance and occurs only at night.
Pain during the day is not due to 'growing pains'. The condition is benign and selflimited, it does not require specific therapy, but may
be relieved by q HS Tylenol.
2. Psychogenic Rheumatism: Vague joint pains and fatigue are
frequent manifestations of 'somatization' disorders.
The child who is unable to attend school despite a normal physical exam and
laboratory evaluation is often demonstrating psychological distress. Many will
respond to gentle reassurance, but in others the complaints mask a significant
psychological disorder. Children with persistent complaints despite normal
findings should be evaluated carefully for family problems. An immediate
recommendation of psychological counseling is frequently rejected, but
physicians who establish a trusting relationship with the family by thoroughly
investigating the child for medical illness before suggesting a psychological
origin may be able to bring about gradual resolution.
3. Reflex Neurovascular Dystrophy: This represents a more severe
form of psychogenic rheumatism in which the somatization
has progressed to include hyperaesthesias, often with
mottled skin coloring and vascular instability. The condition often begins with
a well documented injury which failed to improve. The syndrome typically occurs
in 'perfect' children under excessive parental pressure. Performance activities
and sexual abuse are well recognized causes of this syndrome.
B. Periarticular inflammation:
Periarticular inflammation may be the
result of rheumatic disorders, but osseous disorders must be carefully excluded..
1. Orthopedic disorders: Acute periarticular
pain may result from a fracture or osteomyelitis.
Small children with fractures may not report trauma. Battering must be
considered when a child presents with unsuspected fractures.
2. Neoplastic
disorders: Neoplastic diseases associated with
infiltration of the bone marrow include leukemia, lymphoma, and neuroblastoma. All may initially present with
'joint pains.' Disproportionate anemia, thrombocytopenia, hyperuricemia,
lymphadenopathy, or hepatosplenomegaly
should prompt careful evaluation including bone marrow aspiration if malignancy
is suspected. Bone scans cannot be relied upon to detect leukemia.
3. Rheumatic disorders: The juvenile spondyloarthropathies often
present with both articular and periarticular
inflammation. The periarticular manifestations may
predominate, but articular inflammation is usually
simultaneously present. Lumbar stiffness and heel pain should be specifically
sought.
C. Articular inflammation:
True articular inflammation may be acute or
chronic (more than three weeks in duration).
1. Acute articular inflammation
a. Infection: An acutely inflamed joint must be considered of
infectious etiology until proven otherwise. Staphylococci, Streptococci, and Hemophilus influenzae are
frequent causes of septic arthritis in childhood. Lyme disease is another
frequent infectious arthritis where Ixodes ticks are
endemic. These arthritides typically present with a
single inflamed joint accompanied by fever and elevated erythrocyte
sedimentation rates.
b. Reactive arthritis: Reactive arthritis may accompany or follow
bacterial, viral, or fungal infection. It is usually polyarticluar
and may be associated with fever, rash and systemic illness. Some cases of Lyme
disease present in this manner. It may also be the presentation of
meningococcemia.
Toxic synovitis is the most common reactive arthritis
in childhood. The typical child is three to five years of age and was well
except for an upper respiratory infection in the evening prior to the onset of
symptoms. The following morning he/she awakes unable to walk, with decreased
range of motion in one hip. Fever is only low grade, without significant
elevation of the white blood cell count, or erythrocyte sedimentation rate.
Unless an experienced physician is comfortable with the clinical picture, the
joint must be aspirated to rule out bacterial infection.
c. Poststreptococcal reactive
arthritis deserves special consideration. In the past these children were
not classified as acute rheumatic fever because they do not fulfill 2 of Jones
major criteria. (However, those with migratory polyarthritis
do fulfill the 1993 revised Jones criteria.) Nonetheless, children with
elevated sedimentation rates and arthritis following a documented streptococcal
infection should receive rheumatic fever prophylaxis. Cardiac damage has been
recorded with subsequent streptococcal infections in children who did not
receive prophylaxis.
C. Acute expression of a collagen vascular disease: Serum
sickness, acute rheumatic fever, Henoch Schonlein purpura, and the
'chronic' collagen vascular diseases may present with the acute onset of
arthritis.
2. Chronic articular inflammation:
a. Infection: Unfortunately chronicity does
not exclude infection. Tuberculosis is a frequent cause of smoldering
arthritis. Other bacterial infections including staphylococcal arthritis may
also present with a smoldering onset. Children with established collagen
vascular disease are not immune to developing complicating septic arthritis or osteomyelitis.
b. Collagen vascular diseases: All of the chronic collagen
vascular diseases may occur in children. Diagnoses of gout or temporal
arteritis must be regarded with extreme suspicion. They are virtually unheard
of in childhood. The chronic collagen vascular diseases with findings unique to
childhood include juvenile rheumatoid arthritis (JRA), plant thorn synovitis, the spondyloarthropathies,
The Collagen vascular diseases of childhood.
A. Juvenile Rheumatoid Arthritis: JRA is the most common chronic synovitis of childhood. [Don't be confused by use of the
term 'juvenile arthritis.' This term was developed in recognition of the many
children with arthritis do not have true JRA. Unfortunately, its use to
describe any arthritis occurring in childhood has added to the failure to
properly differentiate the many different forms of arthritis which occur.]
There are three subtypes which are distinguished by the number of joints
involved during the first six months after onset.
1. Pauciarticular onset JRA: Pauciarticular onset JRA involves
four or fewer joints. It most commonly occurs in young females, but may effect either sex. This group is divided between children
who are antinuclear antibody (ANA) positive and at greater risk of complicating
eye disease (iridocyclitis) and children who are ANA
negative. Young females with early involvement of small joints are at high risk
of progressing to polyarticular involvement with a poor prognosis. Many of
these children have 'sausage digits' and probably 'psoriaform'
arthritis. Adolescents with involvement of four or fewer large joints more likely
have a spondyloarthropathy (see below).
2. Polyarticular onset JRA: Polyarticular onset JRA has two
distinct subtypes. Rheumatoid factor (RF) positive adolescent females have
typical adult type rheumatoid arthritis with early onset. Young children with
polyarticular JRA are typically rheumatoid factor (RF) negative. Both of these
entities carry a guarded prognosis especially if they are associated with
significant anemia and elevation of the ESR. Some children previously labeled
as polyarticular JRA may have 'psoriaform' arthritis.
RF may also be found in children with SLE and mixed connective tissue disease.
3. Systemic onset JRA: Systemic onset JRA
presents with high spiking fevers, rash, and variable joint involvement.
It occurs with a more equal sex ratio that the other forms which have a female
predominance. Children with systemic onset JRA are striking for their ill
appearance during episodes of fever, with a relatively benign appearance
between episodes. The fleeting salmon pink rash and a temperature curve which
falls to normal or below at least once each day are characteristic. While many
children with systemic onset JRA do well, others develop significant internal
organ involvement or progress to chronic destructive arthritis. Amyloidosis is a
rare complication in the
B. Spondyloarthropathies: The spondyloarthropathies are a diffuse
group of conditions occurring in both males and females. Their hallmark is an
asymmetric large joint arthritis associated with limited lumbar flexion and tenosynovitis. These children are most often ANA negative
and rheumatoid factor negative. They are at risk for acute, painful iritis, but (in general) not chronic iridocyclitis.
Psoriaform arthritis is considered a
spondyloarthropathy, but is recognized to have a high frequency of ANA positivity and iridocyclitis.
Genetic studies to determine the relationship of psoriaform
arthritis and pauciarticular JRA are in progress.
1. Ankylosing spondylitis (AS): AS is the 'classic'
spondyloarthropathy. It occurs predominantly in HLA B27 positive males with
abnormal limitation of lumbar flexion. Because definite AS cannot be diagnosed
in the absence of radiographic sacroiliitis many
children who are suspect fail to fulfill the diagnostic criteria. These
children should be carried with a diagnosis of 'juvenile spondyloarthropathy'
(see below).
2. Juvenile spondyloarthropathy (SEA syndrome [seronegative
enthesiopathy/arthropathy]): These are children
with asymmetric large joint arthritis and enthesiopathic
findings who do not meet the criteria for AS. They are easily differentiated
from JRA by their later age at onset (usually age 10 or older), the early
presence of back or hip involvement, and the frequent occurrence of asymmetric
metatarsal joint pain or Achilles tendonitis. Although many of these patients
are HLA B27 positive boys, girls and HLA B27 negative individuals of either
sex, may be affected. It was initially thought that the majority of these
children would develop classical AS upon reaching adulthood, but only a small
percentage do so.
3. Reiter's syndrome: The full
combination of arthritis, urethritis, and
conjunctivitis occurs infrequently in childhood. When it does occur its
manifestations are the same as those seen in adults. It is not important to
differentiate children with 'incomplete' Reiter's syndrome from others with
'juvenile spondyloarthropathy,' since the therapy and prognosis are similar.
4. Psoriatic arthritis/'psoriaform'
arthritis: True psoriatic arthritis with typical skin lesions and boney
changes is infrequent in childhood. However, some children without psoriatic
skin changes present with asymmetric dactylitis
(sausage digits), a family history of psoriasis in a first or second degree relative, and variable degrees of additional joint
inflammation. In contrast to the other spondyloarthropathies this constellation
of findings not only affects adolescents, but often occurs in young females who
would otherwise be labeled as having pauciarticular JRA.
5. Inflammatory bowel disease (IBD): The arthritis accompanying
inflammatory bowel disease is a typical spondyloarthropathy. Because arthritis
may be the initial manifestation of IBD, any child with arthritis who develops
chronic or recurrent abdominal pain should be carefully evaluated for the
presence of ulcerative colitis or regional enteritis.
Dermatomyositis:
Dermatomyositis is an acute inflammatory condition of skin and muscle.
There are several distinct subsets of children with dermatomyositis who may ultimately
prove to have distinct diseases. Most often dermatomyositis presents with
increasing fatigue and loss of proximal muscle strength accompanied by a
heliotrope rash. In some children vasculitic lesions of the nail beds ('nailfold capillary dilatation) and skin overlying the
proximal interphalangeal joints (Gottren's
papules) are a prominent finding. Less frequently the onset of dermatomyositis
can be rapid with fever, widespread vasculitic rash and profound weakness. Both
rapid and gradual onset of disease are often
accompanied by malaise and may be complicated by fever and severe muscle
wasting.
Laboratory evaluation typically shows elevation of the CPK, SGOT, and aldolase,
although in some cases only the CPK or aldolase may be abnormal. The diagnosis
is suggested by marked proximal muscle weakness and confirmed by inflammatory
changes in the muscle biopsy. The characteristic clinical history, however, is
simply of a child who is not able to 'keep up', or who is asking to be carried
more and more often. Difficulty going up and down stairs is often the
manifestation which prompts the family to seek medical attention.
Although not pathologically distinguishable, three distinct patterns of
childhood dermatomyositis are clinically recognizable. The most common group
consists of children with proximal muscle weakness, mild heliotrope rash and no
findings suggestive of vasculitis. These children often have a benign illness
which resolves over a four to six month period with corticosteroid therapy
alone. In contrast, children with a marked vasculitic rash, or typical findings
of vascular involvement in the nail beds (nail-fold capillary abnormalities),
or inflammatory papules overlying the interphalangeal
joints (Gottren's papules) most often have a more
chronic relapsing course. Their disease typically improves with corticosteriod or immunosuppressive therapy, but recurs.
Less frequently seen are children with severe rash, markedly elevated muscle
enzyme levels, but only moderate weakness. This subgroup of children is often
refractory to therapy. A fourth group which may be mistakenly diagnosed as
dermatomyositis consists of children who are incidentally found to have
markedly elevated CPK levels (e.g. 20,000 units) when being
screened for another reason. These children are not weak, do not have a heliotropic rash, and remain well without therapy. Although
the precise explanation for this group is unknown, they most likely have a
simple biochemical defect.
Systemic lupus erythematosus (SLE):
Systemic lupus erythematosus most frequently strikes females entering the
second decade of life. However, it may affect children of either sex at any age
The characteristic malar rash presented in textbooks
is present in only one third of cases and is often mistaken for malar flushing due to fever or other causes. Physicians who
rely on its presence to suspect the diagnosis of SLE will miss the majority of
cases.. Definite SLE can be diagnosed whenever a child
fulfills four the the eleven American
The disease course of systemic lupus erythematosus may be highly
variable. Two thirds of children have evidence of renal involvement at the time
of presentation and these children are at risk for progression to diffuse
proliferative glomerulonephritis and ultimate renal failure. Other children may
have no evidence of renal involvement and a relatively benign course. The key
indicators of prognosis at the time of presentation are the presence or absence
of renal involvement, the degree of anemia, and the persistance
of hypocomplementemia following the intiation of therapy. Rising titers of antibodies to dsDNA and falling serum complement levels (C3, C4 or both)
are often indicators of worsening disease and may precede flares of renal
involvement. The key to proper therapy is prompt recognition of the patient at
high risk and institution of aggressive therapy. Five and ten year survivals
are excellent for those without renal disease and satisfactory for those with
renal disease who are aggressively treated. Nonetheless, complications such as
infection or stroke may strike without warning even in the absence of renal
disease.
Henoch Schoenlein purpura (HSP):
The characteristic presentation of abdominal pain, a vasculitic rash over
the extensor surfaces of the lower extremities and buttocks, and arthritis is
easily recongized in this condition. Because HSP is
most often benign physicians may forget to evaluate for the presence of renal
involvement. Unfortunately renal involvement may be present in one third of
cases and in a small percentage of cases will proceed to renal failure. All
children with HSP should be investigated for the presence of renal involvement
with a proper routine and microscopic urine analysis as well as measurement of
the blood urea nitrogen (BUN) and creatinine. If any abnormalities are present
this should be followed by collection of a twenty-four
hour urine for protein, creatine, and creatinine
clearance.
Miscellaneous conditions:
Several forms of arthritis which occur predominantly in children are not
easily characterized. Recognition of these conditions is important to assure
proper therapy.
A. Plant thorn synovitis: This entity
results from retention of a fragment of plant material within the joint
following a puncture injury. The onset of joint swelling and limitation usually
occurs four to six weeks after the initial injury which may have been forgotten
in the interim. The arthritis is often quite painful and unresponsive to normal
measures. Proper diagnosis is often made following surgical biopsy of an
intractable monoarthritis when the pathologic
specimen reveals plant fibers under polarized light microscopy. Synovectomy is the treatment of choice.
B. Benign hypermobile joint syndrome:
This condition typically occurs in early adolescent girls. Most often they are
gymnasts who practice extensively and have great flexibility due to ligamentous laxity. As a result their joints are subjected
to repeated episodes of 'microtrauma.' Acute episodes
may be treated with NSAIDs, but more prolonged
difficulty should prompt review of the athletic program. Osteochondritis
dessicans (particularly of the knee) may present in a
similar manner and can result in permanent disability in this group of
patients.
C. Immunization associated arthritis: The development of a benign
polyarthritis affecting primarily the small joints of the hands 10 to 14 days
following rubella immunization is well documented. The arthritis is typically
mild and resolves over seven to ten days with only symptomatic therapy. Similar
episodes have been reported less frequently with other immunizations.
D. Arthritis associated with immunoglobulin deficiency: Children
with IgA deficiency are most often asymptomatic.
However, IgA deficiency occurs with a greater than
expected frequency in children with arthritis . The
arthritis often consists of benign recurrent joint effusions, but some children
develop typical JRA. A similar benign arthritis is seen in some children with hypogammaglobulinemia. IgA
deficiency will only be detected if quantitative immunoglobulins are routinely
measured. Panhypogammaglobulinemia may be suspected
if the total protein is decreased with a normal serum albumin.
VI. Arthritis associated with other conditions
Arthritis is a well recognized complication of vasculitis including SLE,
Wegener's granulomatosis, Takayasu's arteritis, and Henoch
Schonlein purpura,
A. Marfans syndrome: Children with Marfans syndrome characteristically are tall with arachnodactyly. They typical have ligamentous
laxity and present with complaints similar to those of the hypermobile
joint syndrome. Characteristic findings are an arm span greater than their
height, and leg length greater than trunk length. These children are vulnerable
to dissecting aortic aneurysms. Aortic root dilatation may be evaluated by
routine echocardiography.
B. Ehlers Dahnlos syndrome: Children
with Ehlers Dahnlos syndrome suffer from an extreme
form of hypermobile joint syndrome due to abnormal
connective tissue. Recurrent joint injury secondary to chronic subluxation and characteristic 'cigarette paper' scarring
are common. Milder cases which lack the cutaneous
manifestations occur.
C. Cystic fibrosis: these children occasionally develop benign
effusions of the large joints which will prompt rheumatologic referral. Hypertrophic osteoarthropathy may
also occur in children with cystic fibrosis.
References:
For
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It
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take the time and give them the detail I would like to. I have to take care of my patients. This book is a distillation of my experience
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pediatrician, or a nurse who cares for children with these diseases it will
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questions families, pediatricians, and other health care providers have asked
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Cassidy JT, Petty RE: Textbook of Pediatric Rheumatology, 2nd Edition.
Churchill Livingston, NY.
Click here if you'd like to order a copy of the latest edition of this book from amazon.com
Hicks RV: Vasculopathies of Childhood, PSG
Publishing Co,
Click here to review other books on childhood rheumatic disease which are available
Systemic
lupus erythematosus, dermatomyositis, Scleroderma, progressive systemic
sclerosis, pss, jra,
juvenile rheumatoid arthritis, childhood arthritis, growing pains, rheumatism,
children with pain, bone pain, pediatric specialists, my child hurts, chronic
disease, chronic childhood illness, the best care, Kawasaki disease, mixed
connective tissue disease, SLE, JCA, JIA, juvenile chronic arthritis, sports injuries, frequent sports injuries,
cyclophosphamide, Methotrexate, diclofenac, voltaren, Relafen, children’s health care, educational
materials, pediatric resources, public health education, health education,
school nurse materials, help for school nurses.