A European initiative called SHARE has issued new international guidelines for the diagnosis, management, and treatment of childhood-onset systemic lupus erythematosus (cSLE).
The recommendations are expected to support uniform, high-quality care for children with the disease.
The study, “European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative,” appeared in the journal Annals of Rheumatic Diseases.
The research was led by Noortje Groot, MD, from the Department of Pediatric Immunology at the University Medical Centre Utrecht in the Netherlands.
Childhood-onset SLE can be life-threatening and presents more severe clinical manifestations than its adult-onset counterpart. Its low prevalence ranges from 1.89 to 25.7 per 100,000 children worldwide, which complicates clinical research. Guidelines based on evidence are sparse. Disease management and treatment strategies vary widely across different countries and clinicians.
As a result, the SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, a European Union-funded project, was launched to develop evidence-based and international guidelines for the diagnosis, treatment, and management of pediatric rheumatic diseases, including cSLE.
The initiative included 16 pediatric rheumatology experts who conducted a literature review focused only on children and adolescents.
The study resulted in 25 recommendations toward the diagnosis, management, and treatment of cSLE, and 10 for neuropsychiatric cSLE (NP-cSLE). The authors advise that when multiple symptoms are involved, the most severe should guide treatment decisions.
Recommendations for the diagnosis of cSLE include:
- Criteria from the systemic lupus international collaborating clinics (SLICC) can be used to classify cSLE;
- In the presence of positive antinuclear antibodies (ANA) with at least two clinical SLICC criteria, or a positive ANA with at least one clinical and one immunological SLICC criteria, the patient should be referred to a pediatric rheumatologist;
- Antibodies associated with autoimmune diseases, such as anti-Sm, anti-RNP-a, anti-Ro/SS-A, and anti-La/SS-B should be screened when diagnosing cSLE;
- A diagnosis of cSLE is still valid in patients who are ANA-positive, but anti-dsDNA and ENA negative;
- Hereditary complement deficiencies (a subgroup of immunodeficiencies) should be considered in cSLE patients, especially in young patients;
- A chest X-ray should be conducted in all cSLE patients;
- Cardiac abnormalities should be screened;
- Pulmonary function, including CO diffusion, should be tested in cSLE patients with respiratory symptoms (and in the absence of acute infection);
- Exertional intolerance (chronic fatigue) should be investigated, following the above recommendations for diagnosis;
- In case of unexplained fever, infection and macrophage activation syndrome (MAS, a severe complication of chronic rheumatic diseases) should be investigated;
- In case MAS is suspected, a bone marrow aspirate should be done to facilitate diagnosis. If MAS is suspected and the patient is clinically unstable, not enabling the bone marrow aspirate, treatment should proceed anyway.
Regarding guidelines for disease management, the experts included:
- Regular disease monitoring should include a full clinical evaluation, with determination of body weight, height, and blood pressure; proteinuria (loss of proteins in urine) assessment; urine dipstick testing; blood tests measuring albumin, creatinine, and anti-dsDNA; and a complete blood cell count;
- Initial clinical evaluation should be done every two to four weeks for the first two to four months after diagnosis, and then depending on the response to therapy;
- Children with cSLE receiving systemic corticosteroids should be monitored regularly for linear growth;
- Disease activity in children with cSLE should be assessed regularly using a standardized measure;
- Likewise, disease damage in children with cSLE should be assessed annually using a standardized measure;
- All cSLE patients should have access to an ophthalmologist;
- Children with cSLE taking hydroxychloroquine should have annual eye screening;
- Sun protection should be recommended for patients with skin manifestations;
- A coordinated transition program involving pediatric and adult specialists is key for the best long-term outcomes in cSLE.
Recommendations for treatment of cSLE:
- All children with cSLE should be on routine hydroxychloroquine treatment;
- Patients’ compliance should be actively monitored, especially in situations of treatment modification;
- In cases when the prednisone dose cannot be tapered, a disease-modifying anti-rheumatic drug (DMARD) should be added to the regimen;
- A DMARD should also be included in the treatment if hemolysis is present and hemoglobin is low;
- If Rituxan (rituximab) is needed, the recommended dose is either 750 mg/m2 (up to a maximum of 1 g) at day 1 and day 15; or 375 mg/m2 once a week for four doses.
Diagnostic guidelines were also made for NP-cSLE:
- The nomenclature and case definitions by the American College of Rheumatology should be used for NP-cSLE;
- Patients with cSLE symptoms suggestive of neuropsychiatric disease should be evaluated with the same initial diagnostic workup as patients without SLE;
- In patients with suspected or worsened NP-cSLE, underlying factors such as hypertension, infections, metabolic abnormalities, or adverse effects of therapy should be excluded;
- A diagnostic workup may include a variety of tests, including lumbar puncture, electroencephalogram (EEG), neuropsychological tests of cognition, consultation with an ophthalmologist, and/or neuroimaging (MRI);
- Absence of CNS alterations as detected by an MRI does not exclude NP-cSLE;
- Cognitive dysfunction should be determined either in collaboration with a neuropsychologist or through validated tests in cSLE.
Finally, regarding treatment of NP-cSLE, the experts recommended:
- Corticosteroids and immunosuppressive therapy in cases of neuropsychiatric manifestations caused by an immune or inflammatory process, with non-SLE-related causes excluded;
- In the absence of MRI lesions and definite epileptic abnormalities on EEG, anti-epileptic drugs are normally not required;
- However, long-term anti-epileptic treatment should be followed for recurrent seizures;
- Additional research regarding treatment of pediatric NP-cSLE patients is necessary.
Overall, “the SHARE project has resulted in recommendations on diagnosis, management and treatment of cSLE and NP-cSLE, based on best available evidence and expert opinion. These recommendations should facilitate the optimization of the management of this rare disease,” the authors said in the report.
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