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CHAPTER 106  Pediatric Rheumatologic Disease

around 70%.23 SLE should always be considered in a patient
presenting with pulmonary hemorrhage. Presenting symptoms
and signs typically include dyspnea and hemoptysis or a sudden
decline in hemoglobin. Acute pulmonary hemorrhage can be
rapidly progressive and catastrophic, leading to acute hypoxemic
respiratory failure and hemorrhagic shock. Evaluation for concomitant bacterial, viral, and fungal pathogens is essential, as
pulmonary infections may trigger hemorrhage. Chest radiography
typically reveals diffuse alveolar airspace-filling defects. However,
in milder cases, there may be only focal abnormalities. High-resolution computed tomography (HRCT) is more sensitive than
chest radiography and typically reveals ground-glass opacities.
Bronchoscopy with bronchoalveolar lavage may reveal gross blood
and hemosiderin-laden macrophages.24
Pulmonary Embolism

Pulmonary embolism may occur secondary to antiphospholipid
antibodies (aPLs) or other hypercoagulable states, such as nephrotic syndrome from renal involvement. Smaller and recurrent
emboli may contribute to the development of pulmonary hypertension, another severe SLE complication. Patients typically present
with pleuritic chest pain, tachypnea, tachycardia, and hypoxia but
can sometimes be asymptomatic. Chest CT with angiography is a
very helpful diagnostic test. Early intervention with systemic
thrombolytic agents improves survival in pediatric patients.25

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respiratory distress, with rales and hepatomegaly on examination.
Chest radiography may demonstrate cardiomegaly with pulmonary edema and echocardiography can show diminished ventricular systolic function. Management involves diuresis, inotropic
support, and aggressive immunosuppressive therapy.30
Valvular Disease

Libman-Sacks endocarditis (LSE) involves the development of
sterile vegetations on the cardiac valves and is usually associated
with the presence of aPLs.31 It is a verrucous endocarditis, in
which fibrinoid nodules form on the cardiac valves, most frequently on the mitral valve.29 Vegetations and valvular thickening
can lead to severe regurgitation and stenosis. Echocardiography is
the best diagnostic study; in some cases, a transthoracic study may
not be sensitive enough to detect valvular disease and a transesophageal study is necessary.32,33 Many patients respond to
medical treatment of the underlying SLE; in severe cases, valve
replacement surgery may be indicated. Infective endocarditis (IE)
can mimic LSE in an immunosuppressed SLE patient presenting
with fever and new-onset murmur. Thus, it is important to differentiate between the two conditions.34
Arrhythmias

Interstitial Lung Disease

SLE is associated with an increased risk for conduction abnormalities, including premature atrial beats, supraventricular tachycardia, atrioventricular block, and right bundle branch block. This
is best diagnosed by ECG and echocardiography. Patients usually
respond to pharmacologic antiarrhythmics. Pacemakers are indicated for complete atrioventricular block, particularly in neonatal
lupus, discussed later.

Pulmonary Hypertension

There is a well-recognized increased risk for acute myocardial infarction in SLE secondary to premature atherosclerosis, probably
related to the combined effect of disease-related dyslipidemia,
coronary artery inflammation, and long-term corticosteroid therapy.35 Patients with SLE are at higher risk for premature cardiovascular disease, such as hyperlipidemia and hypertension, which
may relate to chronic inflammation and side effects of long-term
steroid use. Vasculitis in the coronary vessels increases the risk of
myocardial infarction.36 In any child with SLE presenting with
chest pain or dyspnea, myocardial infarction should be considered. ECG may demonstrate ST-segment elevation; in that circumstance, laboratory testing, including serum troponin, may be
helpful. The underlying SLE should be treated; cardiology consultation is beneficial for management and long-term cardiac

monitoring.

Pediatric SLE patients routinely undergo pulmonary function
tests (PFTs) for monitoring and may develop interstitial lung
disease (ILD). Patients may be asymptomatic or present with fever, cough, respiratory distress, or pleuritic chest pain. Chest radiographs may show interstitial changes but are not always sensitive enough to detect ILD. HRCT is a more sensitive study and
may reveal characteristic findings, such as basilar ground-glass
opacities, honeycombing, subpleural nodules, and bronchiectasis.26 Patients with severe ILD are treated with aggressive immunosuppression and supportive therapy. In some cases, they may
require extracorporeal membrane oxygenation.
Pulmonary hypertension may occur in SLE patients secondary to
vasculitis, vasospasm, advanced ILD, and/or recurrent thromboembolism, with the latter observed in patients with aPL positivity.
Patients may present with dyspnea or signs of right-sided heart
failure.27 Treatments include vasodilator therapy in conjunction
with immunosuppressive agents.

Cardiovascular Manifestations
Pericarditis and Pericardial Tamponade

21,28

Pericarditis is the most common cardiac manifestation of SLE.
Acute pericarditis with effusion may lead to cardiac tamponade.
In severe cases or if there is concern for an underlying infection,
pericardiocentesis may be necessary.29 Chronic pericarditis may
cause restrictive cardiac disease. Evaluation for pericarditis includes electrocardiography (ECG) and echocardiography. Symptoms should improve with medical management of the underlying systemic inflammatory disease.

Myocarditis

Although rare, patients with SLE may present with myocarditis,
manifested by poor cardiac output and congestive heart failure.29,30
Symptoms include fatigue, exercise intolerance, tachycardia, and

Acute Coronary Syndrome

Renal Manifestations
Renal disease is estimated to occur in 50% to 80% of children
with SLE and is often more severe than renal disease in adult patients.37 Renal disease may be present at the time of initial diagnosis or may evolve later. Laboratory evaluation includes determination of serum electrolytes, blood urea nitrogen, creatinine, and
albumin; urinalysis; spot urine protein/creatinine ratio (ideally,
first morning void); and 24-hour urine protein and creatinine
collection. Lupus nephritis can manifest in various ways, with
oliguria or anuria, hematuria, hypertension, nephrotic syndrome,
and renal failure requiring dialysis. Hypertension can be severe,
leading to neurologic manifestations such as posterior reversible
encephalopathy syndrome and other end-organ complications,
and must be managed with aggressive antihypertensive therapy.


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S E C T I O N X I   Pediatric Critical Care: Immunity and Infection

Renal biopsy helps to determine the severity, type, and extent of
lupus nephritis, which has major implications for treatment.
However, in cases complicated by significant thrombocytopenia,
hypercoagulability, or severe hypertension, the risks and benefits
of renal biopsy must be weighed closely.38
The severity of the renal disease often dictates the intensity of
the overall treatment approach for a given patient. Response times
to treatment vary—it often takes 3 to 6 months of intensive treatment for the kidney disease to become quiescent. Despite aggressive treatment, a subset of patients will have ongoing refractory
renal disease activity, leading to decline in renal function. Acute
flares over time may also contribute to renal insufficiency and

need for dialysis acutely or more chronically. Renal transplantation has been successfully performed in pediatric patients, but
there is a risk of disease recurrence.39,40

Central Nervous System Manifestations
Seizures and psychosis are the CNS manifestations included in
the SLE classification criteria, but the neuropsychiatric features of
SLE are diverse and of variable severity. These features may be the
initial presentation of SLE or occur at any point during the disease course, even when other features of the disease are under
good control.41 In addition, the presence of aPLs is highly associated with neurologic symptoms, especially stroke, seizure, headache, and movement disorders.42 It is important to carefully
evaluate behavioral changes in critically ill SLE patients, as
changes may be subtle and must be differentiated from medication side effects or ICU psychosis. When SLE patients present
with neurologic symptoms, CNS infection (viral, bacterial, and
fungal) must also be considered. Testing for aPLs should be repeated whenever new neurologic symptoms present. Additional
diagnostic studies—such as brain magnetic resonance imaging
(MRI)/magnetic resonance angiography (eFig. 106.4), electroencephalography, and evaluation of spinal fluid should be performed and may be helpful to guide response to therapy. However, there is no gold standard test to confirm or refute CNS
involvement in SLE. Aggressive immunosuppression is generally
indicated for suspected CNS involvement. Concurrent use of
anticonvulsant and/or antipsychotic medications may be necessary. In the setting of thrombotic stroke, anticoagulation is generally indicated.
Hematologic Involvement
Anemia and thrombocytopenia are common and are often presenting features of SLE. Lymphopenia is also frequent and in
many patients may be an indicator of disease activity. Antibodymediated cytopenias (such as Coombs-positive hemolytic anemia
or microangiopathic hemolytic anemia [MAHA] syndromes) are
frequent. However, other factors—such as blood loss, bone
marrow suppression, medications, or infection—should also be
considered.
Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare, lifethreatening MAHA spectrum hematologic disorder that can be
inherited or acquired. It is strongly associated with SLE, especially
in pediatric patients. Diagnosis is based on five clinical features:

(1) thrombocytopenia and disseminated platelet aggregation,
(2) MAHA, (3) neurologic abnormalities, (4) fever, and (5) renal
disease.43,44 The pathophysiology is based on dysfunction of the
ADAMTS-13 enzyme, which normally cleaves von Willebrand
factor (vWF) multimers. In TTP, there is a lack of vWF cleavage

leading to an accumulation of vWF multimers, which bind
to platelets leading to clot formation and end-organ damage.
In congenital TTP, there is a deficiency of ADAMTS-13, while
in acquired TTP an inhibitory autoantibody inactivates
ADAMTS-13. In patients with clinical suspicion for TTP, demonstrating reduced levels of ADAMTS-13 activity (,5%) is confirmatory. Treatment for TTP includes plasmapheresis acutely along
with immunosuppression. The early use of plasmapheresis significantly decreases the mortality rate, which otherwise is high.43

Immune Dysfunction
Patients with SLE are immunosuppressed by the disease itself due
to abnormal B- and T-cell function and low complement levels
that impair opsonization of encapsulated organisms. The immunosuppressive agents used to control the disease additionally impair the ability to fight infection. Infection is thus a common
reason for ICU admission and a leading cause of death in patients
with SLE.45,46 Children being treated for SLE may not manifest
typical signs of infection, such as fever, due to chronic immunosuppression. As SLE patients may have baseline hypertension, a
relative decline in blood pressure is concerning for sepsis, even if
the child remains normotensive. Similarly, SLE patients often
have baseline leukopenia; thus, a relative rise in WBC count may
indicate infection even in the absence of true leukocytosis. Given
the underlying inflammatory disease, inflammatory markers may
also be difficult to interpret. Patients with SLE are also at risk of
developing MAS, discussed elsewhere, which is often triggered in
the setting of acute infections. Therefore, a high index of suspicion for infection and early initiation of broad-spectrum antimicrobial therapy are warranted in acutely ill SLE patients.
Acute Abdominal Manifestations: Peritonitis, Serositis,
Pancreatitis, and Intestinal Perforation

Nonbacterial peritonitis and serositis of the abdominal organs are
common manifestations of SLE. Patients may present with severe
symptoms, including a surgical abdomen. However, immunosuppressive therapy can also mask clinical signs and symptoms, leading to delayed diagnosis.47 Patients are also at risk for infectious
pathology, such as bacterial peritonitis with abscess formation,
and for intestinal perforation. In cases in which infection and
perforation have been excluded and symptoms are thought to relate to noninfectious serositis, the underlying disease should be
treated aggressively to bring symptoms under control.
Severe acute pancreatitis may complicate SLE.48 It may be
disease related or secondary to medications, such as high-dose
corticosteroids and azathioprine. In patients with nonlocalizing
abdominal pain, pancreatitis should be considered and appropriate laboratory screening and imaging performed to look for inflammation, stones, and pseudocysts. Inciting drugs should be
avoided if possible.
Laboratory Studies
Auto-Antibody Testing

More than 95% of patients with SLE have persistent evidence of
ANAs, a positive ANA test. Thus, a negative ANA test has a good
negative predictive power for the diagnosis of SLE in particular.
However, a positive ANA test is not specific for SLE; ANAs can
be induced transiently in the setting of infection and can be detected in many other conditions, including malignancies, other
rheumatologic diseases, and in healthy people. Unlike the ANA,
anti–double-stranded DNA (dsDNA) antibodies are much more
specific for SLE and are elevated in 80% to 90% of children with


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• eFig. 106.4  ​Magnetic resonance image of the lungs of a 16-year-old girl

with systemic lupus erythematosus. This image was obtained after the

patient was stabilized. Multiple areas of increased signal are scattered
throughout both cerebral hemispheres, consistent with ischemic infarctions resulting from lupus cerebritis. Other views demonstrated similar infarcts in the brainstem and cerebellar hemisphere. Magnetic resonance
angiography obtained at the same time revealed normal vasculature, suggesting pathologic involvement of brain tissue but sparing of vessels. The
patient responded well to pulse intravenous methylprednisolone and​
cyclophosphamide, with no residual neurologic deficit.


CHAPTER 106  Pediatric Rheumatologic Disease

SLE at some point in the disease course. Anti-Smith (Sm) antibodies are also highly specific for SLE but are seen less commonly, in
approximately 20% of pediatric patients.49 Other autoantibodies—
including anti-SSA, anti-SSB, and anti-ribonucleoprotein (RNP),
commonly included in an extractable nuclear antigen (ENA) or
multiplex array panel, occur in varying frequency in SLE patients.
These antibodies may be helpful to monitor for specific patterns
of organ involvement and disease complications but are not
diagnostic of SLE.
High-titer ANA results (.1:640 dilution) are more concerning than lower-titer values for the presence of an underlying autoimmune disease. However, even high-titer ANAs are not specific
for SLE and may be seen in the setting of other rheumatologic
disorders, such as systemic sclerosis (scleroderma), mixed connective
tissue disease (MCTD), dermatomyositis, or Sjögren syndrome.
Scleroderma and MCTD are discussed further on ExpertConsult.
com.
In terms of SLE disease activity monitoring, complement
levels (C3 and C4) are typically low in active SLE, reflecting consumption in the setting of immune complex formation. Serial
measurements of C3, C4, and anti-dsDNA are often used in
monitoring response to therapy but may not reflect all aspects of
disease activity. Importantly, assessment of SLE disease activity
requires a holistic approach, including clinical parameters, and
individual patients may exhibit different patterns in disease activity markers.

Markers of systemic inflammation are elevated in active SLE.
ESR can be markedly elevated and is a strong indicator of active
disease. Interestingly, CRP levels are often normal or only modestly elevated in active lupus. Patients presenting to the ICU with
signs of persistent inflammation and a significant discordance
between ESR and CRP should be evaluated for SLE as an underlying diagnosis. Even modest elevations in CRP in known SLE
patients should raise suspicion for acute infection.

Management
Patients with SLE often require ICU-level care; they can be severely ill at diagnosis and with disease flares. Treatment is often
divided conceptually into initial induction therapy designed to
gain control of active disease followed by longer-term maintenance regimens. For hospitalized patients, induction treatment is
typically initiated with high-dose pulse IV methylprednisolone
(30 mg/kg per day, maximum dose 1000 mg), administered over
1 hour for 3 to 5 consecutive days. Subsequently, prednisone is
given IV or by mouth starting at 1 to 2 mg/kg per day divided
twice a day in tapering doses over the next several months. Steroidsparing agents, such as cyclophosphamide and rituximab (an
anti-CD20 antibody), are typically necessary in patients with
significant life-threatening or major organ (CNS, renal, pulmonary) involvement. Choice of specific agent is dependent on the
type of disease manifestation and patient-specific factors. Treatment of pulmonary hemorrhage typically involves a combination
of supportive therapies and aggressive immunosuppression. Despite the need for systemic heparinization, extracorporeal membrane oxygenation has been found to be safe and life-saving in
children with pulmonary hemorrhage who fail conventional
therapy.50,51 Plasmapheresis may be required as adjunctive therapy
in refractory cases.24 TTP should also be treated with corticosteroids and plasmapheresis followed by cyclophosphamide or rituximab. Nonsteroidal antiinflammatory drugs (NSAIDs), corticosteroids, and colchicine may be used acutely for symptom relief in
conjunction with traditional steroid-sparing medications to treat

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pericarditis. Severe renal involvement warrants treatment with
high-dose IV steroid therapy, cyclophosphamide, and/or mycophenolate mofetil. Treatment for CNS involvement includes
high-dose IV steroid therapy and cyclophosphamide. Plasmapheresis may be necessary in severe cases. Rituximab is also increasingly used but remains an experimental therapy.52

Cyclophosphamide dosing is typically based on body surface
area and dosing is lower than what is used in oncologic treatment.
Side effects of cyclophosphamide include alopecia, nausea, vomiting, cytopenias, and syndrome of inappropriate antidiuretic hormone secretion (SIADH). Patients must be adequately hydrated
to prevent development of hemorrhagic cystitis. Longer term,
there is a risk of subfertility or infertility related to cumulative
dose and increasing age.53 Although definitive evidence of efficacy
is lacking, postmenarchal female patients undergoing longer cyclophosphamide-based regimens may benefit from concomitant
ovarian suppression.54
Rituximab may cause a hypersensitivity reaction as well as
hypogammaglobulinemia and cytopenias, increasing risk of infection. A significant potential future side effect is the risk of progressive multifocal encephalopathy.55 For patients with less severe
disease manifestations, mycophenolate mofetil (MMF) or azathioprine are typically used to induce and maintain remission. Renal
failure, nephrotic syndrome, and dialysis can all affect drug metabolism and clearance; consultation with pediatric nephrology
and pharmacy services may assist with proper dosing.

Neonatal Systemic Lupus Erythematosus
Infants can develop antibody-mediated disease due to transmission of maternal antibodies in utero; mothers may or may not be
symptomatic from these autoantibodies. Symptoms can present
prenatally or, up to 6 months of age, maternal anti-SSA and antiSSB antibodies in the fetal circulation can result in rashes, transaminitis, cytopenias (typically, thrombocytopenia), and heart
block.56 The level of maternal antibodies in an infant’s circulation
will gradually decline over the first 9 months of life. Thus, most
manifestations other than heart block usually resolve without
treatment; however, exchange transfusion has been used as acute
therapy in severe cases. Unfortunately, heart block often develops
prenatally, can be severe, and is usually irreversible. Affected infants may require a pacemaker and ionotropic support.56

Raynaud Phenomenon
Asymmetric triphasic color changes presenting in prepubertal females or any male in childhood deserves further evaluation for
underlying systemic disease. Raynaud phenomenon is a triphasic
color change typically in the distal extremities (fingers and toes;
rarely, ears, nose). Transient vasoconstriction causes a white or

gray (depending on underlying skin pigmentation) numb phase,
often followed by a blue tinge due to vascular congestion and then
a red, painful reperfusion phase. This should be differentiated
from the more symmetric, cyanotic appearance of acrocyanosis.
Raynaud phenomenon can occur as a primary idiopathic process
in otherwise healthy individuals or can be secondary to an underlying rheumatologic disease, most commonly SLE, MCTD,
scleroderma, antiphospholipid syndrome (APS), or a primary
vasculitis. Idiopathic or primary Raynaud phenomenon often
presents in older adolescent females and is rarely associated with
morbidity. However, true Raynaud phenomenon presenting in a
prepubertal child or male of any age should raise suspicion for an
underlying rheumatologic disease. Raynaud phenomenon due to


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S E C T I O N X I   Pediatric Critical Care: Immunity and Infection

underlying rheumatologic disease may be difficult to control; vasospasm may be persistent due to the underlying vascular inflammation and irritability. If vasospasm persists, it may compromise
distal tissue integrity and require ICU-level intervention, including initiation of vasodilating agents such as iloprost, to prevent
digit loss.

Antiphospholipid Syndrome
Key Points
Children presenting with unexplained stroke or thrombosis
should be evaluated for APS as part of a hypercoagulability evaluation. Acute thrombotic microangiopathy affecting multiple organ systems should prompt consideration of catastrophic antiphospholipid syndrome (CAPS).
Clinical Presentation
APS is an autoimmune, prothrombotic state characterized by
arterial or venous thrombosis, pregnancy morbidity, and specific
laboratory features.57 It may occur as a primary disorder, secondary to other autoimmune diseases (typically SLE), or in the setting of infection or malignancy. Circulating autoantibodies to a

variety of cell membrane proteins result in inappropriate platelet
and/or endothelial activation and subsequent pathophysiology.
The clinical presentation depends on the site and type of vascular
involvement and accompanying nonthrombotic manifestations.
Venous thrombosis occurs most commonly in pediatric patients
and can present in various ways, including superficial thrombophlebitis, deep vein thrombosis, pulmonary thromboembolism,
and cerebral venous sinus thrombosis. Arterial occlusion may
also occur, though is less common and may also present in a variety of ways, including stroke, renal thrombotic microangiopathy, myocardial infarction, bone infarction, or mesenteric artery
thrombosis. Rarely, autoantibodies may target prothrombin itself
and lead to a combined bleeding diathesis as well as tendency
toward hypercoagulability with factor II replacement.58 Nonthrombotic clinical features are extremely variable but include
cytopenias; neurologic abnormalities (movement disorders, migraine, transverse myelitis); skin findings (livedo reticularis, cutaneous necrosis, skin ulcerations, Raynaud phenomenon); valvular heart disease; and renal involvement (hypertension,
hematuria). CAPS refers to the acute onset (,1 week) of smallvessel occlusion affecting three or more organ systems, leading to
multiorgan failure.59
Laboratory Studies
For any child presenting with a vascular thrombosis, a full hypercoagulability workup should be undertaken (see also Chapter 89).
To diagnose APS, children must have one or more of the characteristic laboratory features: positive anticardiolipin antibodies,
anti-b2 glycoprotein antibodies, or lupus anticoagulant. The activated partial thromboplastin time (aPPT) may be prolonged and
does not correct with addition of normal serum (mixing study),
indicating the presence of an interfering antibody. As these aPLs
can be transiently induced in the setting of infection, a definite
diagnosis of APS requires evidence of persistent elevation on two
or more occasions 12 weeks apart.
Management
For children with vascular thrombosis due to APS, anticoagulation is typically initiated with unfractionated heparin. Given that
lupus anticoagulant can artificially prolong the aPPT, monitoring

antifactor Xa levels may be a more reliable measure of therapeutic
anticoagulation. For acute, severe thrombotic events, thrombolytic therapy, such as tissue plasminogen activator, has been used
successfully in children.60

Given the high mortality rate in CAPS, aggressive therapy is
essential and should include anticoagulation, high-dose corticosteroids, and occasionally plasma exchange; IV immunoglobulin
(IVIG), cyclophosphamide, and rituximab may be of additional
benefit.58,59

Juvenile Dermatomyositis
Key Points
Juvenile dermatomyositis (JDM) is a systemic inflammatory vasculopathy that presents with rashes and weakness of proximal
muscle groups. Patients presenting with persistently elevated aspartate transaminase (AST) greater than alanine transaminase
(ALT) without liver pathology should be evaluated for myositis.
Levels of creatine kinase (CK), aldolase, and lactate dehydrogenase (LDH) levels may be helpful, along with MRI. Aspiration
due to pharyngeal muscle involvement in JDM patients may
be clinically silent. Patients with newly diagnosed or active JDM
presenting with acute abdominal pain should be evaluated
for intestinal perforation due to possible gastrointestinal (GI)
involvement.
Clinical Presentation
JDM is a vasculopathy that primarily targets the skin and muscles. It is characterized by weakness and skin findings: rash over
the eyelids (heliotrope rash); face (malar rash); neck and upper
back (shawl sign); extensor surfaces of the joints in the hands,
elbows, and knees (Gottron papules); and periungual telangiectasias.61 The inflammation primarily affects striated muscle but can
also involve smooth muscle. Typically, proximal rather than distal
muscle groups are affected, often leading to profound shoulder
and hip girdle muscle weakness. The muscles of the palate, pharynx, and upper third of the esophagus can also be involved, resulting in dysphagia, dysphonia, and risk of aspiration. In severe
cases, weakness may lead to respiratory failure. However, this may
not be apparent, as weakness can mask retractions and typical
signs of respiratory distress.
The vascular injury in JDM can also target the gastrointestinal (GI) tract and may cause bleeding, necrosis, and perforation. Cardiac muscle can also become inflamed and, rarely,
myocarditis and conduction defects have been reported.61 ILD
occurs secondary to disease activity and/or chronic aspiration

and may be difficult to detect early in disease. The kidneys are
not typically targeted by vasculitis in JDM, but rhabdomyolysis
with secondary renal impairment can rarely occur, especially
with initial presentation or acute disease flares. As with many of
the rheumatologic diseases, severely ill patients with JDM can
develop MAS. A more long-term complication of uncontrolled
JDM disease activity is calcinosis, with calcium precipitation in
the skin and underlying fascial tissues, particularly around the
joints and sites of repeated trauma. In severe cases, this can
impair mobility. As opposed to adult patients where dermatomyositis may be the presenting sign of an underlying malignancy, JDM is rarely a paraneoplastic syndrome in younger
pediatric patients. However, older adolescent patients who
present with JDM require a careful screen for teratoma or other
underlying cancer prior to initiation of immunosuppressive
treatment regimens.62