diagnosed in the presence of evidence of multiple organ system involvement, so no
laboratory study is pathognomonic.
TABLE 101.2
IMMUNOMODULATORY AGENTS FOR THE TREATMENT OF SLE IN
CHILDREN
Biologic
effects
Principal toxicities
Hydroxychloroquine Blocks
Retinopathy, nausea, rash,
lysosome
agranulocytosis
processing
of
autoantigens
Azathioprine
Precursor of 6- Bone marrow suppression,
MP; blocks
infection (especially
purine
zoster), nausea, hepatitis,
synthesis
rash
Mycophenolate
Blocks purine Bone marrow suppression,
mofetil
synthesis
infections, nausea,
diarrhea
Cyclophosphamide Alkylates
Bone marrow suppression,
DNA
opportunistic infections,
leading to
nausea, alopecia, bladder
cytotoxicity
toxicity, infertility,
cardiotoxicity
Rituximab
Chimeric anti- Tumor lysis syndrome,
CD20
anaphylaxis,
monoclonal
hypogammaglobulinemia,
antibody
opportunistic infections
that depletes
B-cells
Belimumab
Monoclonal
Opportunistic infections,
antibody
infusion reactions,
directed
headache, nausea, fatigue,
against
depression including
BLyS
suicidal ideation
Monitor
Ophthalmology
evaluation
every 6 mo,
CBC, LFTs
every 3–6 mo
CBC, lymphocyte
count, LFTs
CBC, lymphocyte
count
WBC, UA,
BUN/Cr
IgG level,
lymphocyte
count
Monitor for
worsening
depression
SLE, systemic lupus erythematosus; CBC, complete blood cell count; LFT, liver function test; 6-MP, 6mercaptopurine; DNA, deoxyribonucleic acid; WBC, white blood cells; UA, urinalysis; BUN/Cr, blood urea
nitrogen/creatinine; CD20, B lymphocyte surface marker; IgG, immunoglobulin G; BLyS, B lymphocyte stimulator.