European researchers publish recommendations for child-onset lupus

European researchers recently published recommendations for child-onset lupus, as well as neuropsychiatric child-onset lupus.

“It must be noted that good quality evidence regarding diagnosis and treatment of [child-onset systemic lupus erythematosus] cSLE is limited,” the researchers wrote. “Due to lack of robust evidence underpinning some statements, the expert panel refrained from being too specific regarding diagnostic procedures, monitoring intervals or specific drug treatments.”

Recommendations were kept if they had at least 80% agreement among the panel. In total, the researchers made 35 recommendations. They made the following 11 recommendations for diagnosis of cSLE:

systemic lupus international collaborating clinics (SLICC) criteria can be used for the classification of cSLE;
in the presence of positive antinuclear antibodies (ANA) combined with at least two clinical SLICC criteria or in the presence of positive ANA combined with at least on clinical and one immunological SLICC criterion, physicians should refer to a pediatric rheumatologist;
anti-Sm, anti-RNA-a anti-Ro/SS-A and anti-La/SS-B should be included in the consideration of cSLE diagnosis;
when a patient is ANA positive, but anti-double stranded DNA (dsDNA) and negative for extractable nuclear antigens, a diagnosis of cSLE can still be made;
hereditary complement deficiencies should be considered in all patients with cSLE;
patients should have a chest X-ray at diagnosis;
patients should be screened for cardiac abnormalities with an electrocardiogram (ECG) and echocardiography at diagnosis;
patients with cSLE and respiratory symptoms or signs — but without acute infection — should receive a pulmonary function test that includes CO diffusion;
physicians should investigate exertional intolerance and initial investigations should include chest X-ray, pulmonary function test with CO diffusion, echocardiography and an ECG;
unexplained fever should trigger a search for infection and macrophage activation syndrome (MAS); and
if MAS is suspected, physicians should consider a bone marrow aspirate and if MAS is suspected and the patient is clinically unstable, treatment should not be delayed if a bone marrow aspirate is not possible;

They made the following nine recommendations for management of the disease:

active disease should be continually monitored with a full clinical evaluation that includes body weight, height and blood pressure; urine dipstick testing; proteinuria estimation; blood tests including albumin, creatinine, estimated glomerular filtration rate (eGFR), erythrocyte sedimentation rate, C3, C4 and anti-dsDNA; and complete blood cell count;
clinical evaluation should occur between every 2 and 4 weeks for the first 2 months to 4 months after diagnosis or flare and then according to treatment response;
children who receive systemic corticosteroids should be checked for linear growth;
patients should have their disease activity accessed regularly with a standard measure;
patients should have their disease damage checked yearly with a standard measure;
patients should have access to an ophthalmologist;
annual eye screening should be considered for those taking hydroxychloroquine;
sun protection can be considered in patients with skin manifestations; and
there should be a coordinated transition program with pediatric and adult specialists;

They made the following five recommendations for treatment of the disease:

patients should receive hydroxychloroquine regularly;
compliance should be checked in all treatment change decisions;
when it is not possible to taper prednisone, a disease-modifying antirheumatic drug (DMARD) should be added to therapy;
when hemolysis is present and hemoglobin is lower than normal, a DMARD should be added to therapy; and
if rituximab is required, the recommended dose is either 750 mg/m² per dose up to a maximum of 1 g at day 1 and day 15 or 375 mg/m² per dose once a week for four doses.

In addition, they made the following six recommendations for diagnosis of neuro-psychiatric cSLE (NP-cSLE):

nomenclature and case definitions by the American College of Rheumatology should be used in classification of NP-SLE syndromes;
in patients with new or unexplained symptoms or signs suggestive of neuropsychiatric disease, initial diagnostic work-up should include the same work-up as performed in patients without SLE;
in patients with a suspected diagnosis of NP-cSLE or worsening symptoms of NP-cSLE, infections, hypertension, metabolic abnormalities or adverse events of medication should be excluded;
diagnostic work-up may include lumbar puncture and analysis of cerebrospinal fluid, electroencephalogram (EEG), neuropsychological assessment of cognitive function, ophthalmologist consultation, nerve conductional studies and neuroimaging;
a typical MRI of the central nervous system does not exclude NP-cSLE; and
cognitive impairment should be assessed either in collaboration with a neuropsychologist or with validated tests for cognitive impairment of cSLE.

They also made the following four recommendations for treatment of NP-cSLE:

when neuropsychiatric manifestations are caused by immune or inflammatory processes and non-SLE-related causes are excluded, then corticosteroids and immunosuppressive therapy are recommended;
antiepileptic drugs are not necessary after a single seizure in the absence of MRI lesions and definite epileptic abnormalities on EEG after seizure recovery;
long-term antiepileptic treatment should be considered for seizure recurrence; and
there is a need for pediatric NP-cSLE research on treatment.

“These recommendations should facilitate the optimization of the management of this rare disease,” the researchers wrote. – by Will A. Offit

Disclosure: The researchers report no relevant financial disclosures.

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Source:

Groot N, et al. Ann Rheum Dis. 2017;doi:10.1136/annrheumdis-2016-210960.