Childhood SLE
Childhood SLE
Childhood SLE
STANDARD
TREATMENT
GUIDELINES 2022
Childhood
Systemic Lupus
Erythematosus
Lead Author
Nutan Kamath
Co-Authors
Amit Rawat, Aaradhana Singh
Chairperson
Remesh Kumar R
IAP Coordinator
Vineet Saxena
National Coordinators
SS Kamath, Vinod H Ratageri
Member Secretaries
Krishna Mohan R, Vishnu Mohan PT
Members
Santanu Deb, Surender Singh Bisht, Prashant Kariya,
Narmada Ashok, Pawan Kalyan
Childhood Systemic
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Lupus Erythematosus
Definition
Laboratory investigations form integral part of diagnosis and management of patients with SLE.
Investigations
Antinuclear antibodies Indirect immunofluorescence Present in more than 90% of
(ANA) on HEp2 cells remains the gold children with lupus. Patterns of
standard for detection staining may reflect antigenic
specificity of the antibody
Anti-double stranded Enzyme immunoassay or ;; High specificity for SLE
antibodies (anti-dsDNA) immunofluorescence ;; Marker for active disease
especially lupus nephritis
Complement estimation Nephelometry or enzyme Complement C3 and C4 are
immunoassay commonly measured. Low levels
detected in active disease
Extractable nuclear Can be detected by Provide useful information on
antigens immunoblotting or by antigenic specificity of ANA
counterimmunoelectrophoresis
Antiphospholipid (aPL) ;; ELISA for anticardiolipin and Positive in significant proportion
antibodies anti-β2-glycoprotein I antibodies of pediatric SLE patients.
;; Lupus anticoagulant assay by Anticoagulants (heparin and oral
activated partial thromboplastin anticoagulants) may interfere with
time (aPTT) and dilute Russell’s detection of lupus anticoagulant
viper venom time (dRVVT)
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Childhood Systemic Lupus Erythematosus
Treatment
Childhood systemic lupus erythematosus ranges in severity from mild disease with arthritis
and rash to devastating nephritis with renal failure or profound neurological disturbances.
Approach to therapy is individualized for each patient and based on the clinical features.
Nevertheless, treatment is unified based on a few guiding principles.
General Treatment
Immunize for age with Recognise “Flare of disease
non-live vaccines in cSLE Calcium and vitamin D activity” (development of
(influenza, hepatitis supplementation if on steroids new symptoms and signs
A and B, human folic acid supplementation if which require a change in
papilloma, meningococcal, on methotrexate therapy)
pneumococcal, COVID-19)
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Childhood Systemic Lupus Erythematosus
Organ Specific Treatment
Assess Disease
Damage Index
Activity and
Assessment of disease activity and damage is best done by pediatric rheumatologist
using SLE Disease Activity Index (SLEDAI)/British Isles Lupus Assessment Group
(BILAG) index and paediatric version of American College of Rheumatology/Systemic
Lupus International Collaborating Clinics (ACR/SLICC) damage score index.
Counseling and Education
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Childhood Systemic Lupus Erythematosus
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Childhood Systemic Lupus Erythematosus
Contd...
Name Dosage Indications Important side Monitoring
effects
Nonsteroidal GI intolerance/ bleed, AST/ALT, serum
anti- renal impairment, creatinine at
inflammatory hypersensitivity baseline and 6
drugs (NSAIDs) month intervals
Naproxen 15–20 mg/kg/day Myalgia, arthralgia, Pseudoporphyria-
in two divided arthritis pruritis, urticarial,
doses (maximum morbiliform rash,
dose 1 g) erythema multiforme
Drugs used in Management of SLE
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Childhood Systemic Lupus Erythematosus
Contd...
Name Dosage Indications Important side Monitoring
effects
Cyclosporine 2.5–5 mg/kg/day Membranous nephrotoxic Blood urea
A nephropathy nitrogen,
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Childhood Systemic Lupus Erythematosus
Role of General Pediatrician (GP)
Maintain immunization
Never stop
schedule. Administer
immunosuppression
non-live vaccines. Discuss Counsel parents and child
without discussion with the
about live vaccines with
rheumatologist
the rheumatologist
Prognosis
Early diagnosis and appropriate therapy ensures good prognosis. Intercurrent infections, flares,
renal and cardiovascular disease, neurologic manifestations and poor adherence to treatment
affects outcome. Morbidity of the disease and adverse effects of the medications must be
minimized to achieve satisfactory long-term outcome.
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Childhood Systemic Lupus Erythematosus
;; Procainamide ;; Phenytoin
;; Hydralazine ;; Carbamazepine
Drug-induced Lupus
;; Minocycline ;; Valproate
;; Isoniazid, Rifampicin ;; Penicillamine
;; Amiodarone
Infections
Lupus Mimics
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Childhood Systemic Lupus Erythematosus
Summary
Diagnosis and management of cSLE is a challenge due to its myriad clinical presentations.
Shared care pathways between specialists from different disciplines and different levels of care
(primary, secondary, and tertiary) are of paramount importance in management of cSLE.
Further Reading
Rheumatol. 2019;71(9):1400-12.
;; Gergianaki I, Bertsias G. Systemic lupus erythematosus in primary care: an update and practical
messages for the general practitioner. Front Med (Lausanne). 2018;5:161.
;; Groot N, de Graeff N, Marks SD, Brogan P, Avcin T, Bader-Meunier B, et al. European evidence-based
recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE
initiative. Ann Rheum Dis. 2017;76(12):1965-73.
;; Massias JS, Smith EMD, Al-Abadi E, et al. Clinical and laboratory characteristics in juvenile-onset
systemic lupus erythematosus across age groups. Lupus. 2020;29(5):474-81.
;; Trindade VC, Carneiro-Sampaio M, Bonfa E, Silva CA. An update on the management of childhood-
onset systemic lupus erythematosus. Paediatr Drugs. 2021;23(4):331-47.
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