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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 more information for physicians is available in my book† (click here for a complete table of contents and information on getting a copy.)
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.
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.
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.
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
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.
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,
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 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
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 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.
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.
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.
Arthritis is a well recognized complication of vasculitis including SLE,
Wegener's granulomatosis, Takayasu's arteritis, and Henoch
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.
For a much more detailed discussion of this information see my book below.† Itís 432 pages packed with the latest information on how to diagnose and treat childhood rheumatic diseases.
Itís not just
A guide to childhood muscle, bone, and joint pain,
rheumatic diseases and the latest treatments
It has always been a frustration trying to answer the many questions I have received from people over the web.† I canít 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 answering questions for parents and health professionals over 25 years of practice.† If you want to know about the diseases, the tests, the medications, or how to be sure you are getting the best careĖ If you are the family member of a child with joint pains, this book will give you the answers.† If you are a general physician, a pediatrician, or a nurse who cares for children with these diseases it will answer many of the questions families ask you, and you can recommend it to them.† It will also answer many of your questions about what shots to give, what precautions to take, and the other questions families, pediatricians, and other health care providers have asked me over the years.
Cassidy JT, Petty RE: Textbook of Pediatric Rheumatology, 2nd Edition. Churchill Livingston, NY.
Hicks RV: Vasculopathies of Childhood, PSG
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, childrenís health care, educational materials, pediatric resources, public health education, health education, school nurse materials, help for school nurses.