Pediatric emergency medicine trisk 733

having SLE The proposed scoring system is currently being studied to see in what way the sensitivity and specificity compares to the ACR and SLICC classification systems More research is needed to determine if the EULAR/ACR classification system is applicable for diagnosing SLE in children It is probable that in the future, this new classification will replace the 1997 ACR and 2012 SLICC classification systems FIGURE 101.2 Mucosal lesions (macules and ulcers) of the palate in an adolescent girl with active lupus Goals of Treatment SLE is often more severe in children than in adults Although adult lupus patients are more likely to die of complications, children and adolescents with lupus are more likely to succumb earlier, during the acute stages of the disease Common causes of death within the first years of diagnosis are pancreatitis, pulmonary hemorrhage, infection, thromboembolic disease, and active neuropsychiatric disease Delayed diagnosis and treatment are strong risk factors for morbidity and mortality in pediatric lupus In view of the fact that cumulative disease activity over time correlates with damage from the disease, expedient diagnosis and appropriately aggressive treatment is particularly critical for children Thus, pediatricians need to maintain a high index of suspicion for lupus, and physicians experienced in the care of children with SLE should participate in the diagnosis and management of all pediatric lupus patients Clinical Considerations Clinical Recognition Although SLE is often considered a disease of adulthood, up to 20% of lupus patients are diagnosed during the first two decades of life Childhood SLE affects girls more often than boys but this gender difference occurs to a lesser extent than in adults Incidence and prevalence rates vary by ethnicity and are higher in Hispanic, Asian, Native American, and African populations The mean age of diagnosis in children is approximately 12 to 13 years The onset of SLE may be insidious or acute The initial presentation usually includes constitutional features, such as fever, malaise, and weight loss, in addition to manifestations of specific organ involvement such as rash, pericarditis, arthritis, or seizures Because virtually any part of the body may be affected by SLE, patients may present with a bewildering variety of signs and symptoms Although many of these are nonspecific, the examiner’s level of suspicion for possible SLE should increase as the number of involved organ systems increases Further, although SLE presents with a wide array of symptoms, the majority of pediatric cases present with a recognizable constellation of complaints related to musculoskeletal, cutaneous, renal, and hematologic involvement In French and Canadian studies, the most common presenting manifestations in children are hematologic (anemia, lymphopenia, leukopenia, and/or thrombocytopenia); mucocutaneous (malar rash and/or ulcers); musculoskeletal (arthritis or arthralgia); presence of fever; and renal abnormalities (nephritis or nephritic syndrome) (Please refer to the SLICC criteria discussed above for specific details about making the diagnosis.) Triage Considerations Fever in a child with SLE represents a potential emergency Children with SLE are at increased risk of infections from their disease activity and also from the immunosuppressive therapies that they receive to control their illness Patients with fever should be evaluated rapidly and thoroughly, and often require empiric broadspectrum antibiotics while awaiting the results of the diagnostic evaluation Patients taking corticosteroids may require stress doses during acute febrile illness Children with SLE are also at increased risk for a wide variety of cardiac, pulmonary, and gastrointestinal (GI) complications, many of which are life-threatening See Table 101.3 , which describes the complications of SLE Clinical Assessment Arthritis in SLE is usually symmetric, involving both large and small joints Swollen joints may be quite painful, but they are usually not erythematous Cutaneous lesions are present in more than 85% of patients with SLE The typical malar rash with butterfly distribution is present at diagnosis about half the time Features that help to distinguish it from other rashes are sparing of the nasolabial folds, extension onto the nose, and extension of the rash onto the chin Painless oral or nasal ulcerations, alopecia, and photosensitivity are common Discoid lesions are less frequent in children but when seen are characteristic ( Fig 101.1 ) Evidence of renal disease is present in approximately 50% of children with SLE at the time of presentation, with nearly 90% developing some degree of renal involvement during the course of their disease This is significantly higher than in adult patients, in whom renal disease develops in about half Lupus nephritis is usually asymptomatic, although close questioning often reveals nocturia due to impaired renal concentrating mechanisms Edema or hypertension may be clues to involvement of the kidney Despite significant improvements in treatment, the extent of renal involvement remains the single most important determinant of prognosis in SLE, and therefore will highly influence choice of immunosuppressive therapy Thus, most children with evidence of kidney disease undergo renal biopsy to more precisely characterize the pathology and help optimize the therapeutic regimen Clinical evidence of CNS involvement may occur at disease onset or later in the course Symptoms and signs referable to the CNS include headache, seizures, polyneuropathy, hemiparesis/hemiplegia, and ophthalmoplegia Particularly in the ED setting, the clinician should be aware of the risk of stroke (both thrombotic and hemorrhagic) and of sinus vein thrombosis Chorea is the most common movement disorder and may be a presenting sign; Lyme disease (LD) and rheumatic fever must also be considered in such cases Cranial nerve palsies most commonly involve the optic nerve, trigeminal nerve, and nerves controlling the extraocular muscles Myasthenia gravis should be excluded if any extraocular muscles are involved Neuropsychiatric manifestations include mood disorders, hallucinations, memory alterations, and psychosis; rarely, psychiatric symptoms may be the first clinical manifestation of childhood lupus Pericarditis is the most prevalent form of cardiopulmonary involvement in SLE Myocarditis occurs less frequently Heart murmurs caused by valvular lesions are not common, but asymptomatic vegetations on valve leaflets are seen at autopsy in most patients (Libman–Sacks endocarditis), which is why patients with SLE are at increased risk for subacute bacterial endocarditis Myocardial infarctions have been reported in children with lupus, and the possibility of myocardial ischemia should be kept in mind if a child with lupus develops acute chest pain Pleuropulmonary involvement occurs in more than 50% of cases of SLE Unilateral or bilateral pleural effusions may occur, and pulmonary hemorrhage, although uncommon, also occurs in children with SLE Pulmonary function testing (PFT) demonstrating an elevated DLCO offers a readily available, noninvasive technique for identifying blood in the lungs Pulmonary embolus, particularly in children with antiphospholipid antibodies, also must be considered in children with the acute onset of chest pain For any SLE patient with pleuropulmonary manifestations, disease-related involvement must be distinguished from intercurrent infection, CHF, aspiration pneumonia, and renal failure Common GI manifestations include nausea, vomiting, and anorexia Persistent localized abdominal pain should suggest specific organ involvement, such as pancreatitis or gastric ulcer, both of which may occur from the disease or secondary to medical therapy Malabsorption syndrome may be a manifestation of SLE When accompanied by melena, it suggests poorly controlled disease complicated by GI vasculitis This is associated with a 50% mortality rate without expeditious evaluation and treatment Of course, abdominal pain in SLE is not always related to the underlying disease but may stem from other causes, including appendicitis, ruptured ovarian cyst, or pelvic inflammatory disease Further complicating evaluation is the fact that manifestations of any of these conditions may be masked or altered by the corticosteroids and immunosuppressive agents most patients receive Mild to moderate anemia is common in SLE Hemolytic anemia associated with a positive Coombs test is most characteristic An acute decrease in the hemoglobin or hematocrit should alert the physician to the possibility of internal hemorrhage or massive hemolysis Autoimmune thrombocytopenia, even in the absence of offending drugs, is commonly seen in SLE; up to 20% of adults initially diagnosed with idiopathic thrombocytopenic purpura (ITP) progress to full-blown lupus over the ensuing years Leukopenia and lymphopenia are additional hematologic abnormalities characteristically seen in SLE; apart from viral infections and drug toxicity, few other conditions cause children’s lymphocyte counts to fall to