How does the ANA profile aid in the diagnosis and management of SLE and related juvenile systemic CTDs

How does the ANA profile aid in the diagnosis and management of SLE and related juvenile systemic CTDs?

Although a negative ANA profile does not rule out any of the juvenile systemic CTDs, a positive ANA profile can be extremely useful in making a specific diagnosis. In particular, anti-dsDNA and anti-Smith (anti-Sm) antibodies are specific for SLE; high titers of anti-ribonucleoprotein antibody are suggestive of MCTD; and anti-Ro (SS-A) and/or anti-La (SS-B) antibodies are found in Sjögren syndrome, although this syndrome and antibodies against Ro (SS-A) are most commonly seen as a part of SLE. In addition, anti-histone antibodies may be seen in SLE and in drug-induced lupus. These two diagnoses may be distinguished by the presence of antibodies to specific histones. Finally, a positive ANA is essentially never found in systemic-onset juvenile idiopathic arthritis (Still disease).

Antinuclear Antibody Subtypes in Juvenile Systemic Connective Tissue Disease

Active SLEMCTDSScCRESTPrimary SjögrenJIA (Poly)
ANA99%100%70%–90%60%–90%>70%40%–50%
Anti-native DNA60%NegNegNegNegNeg
Anti-Sm30%NegNegNegNegNeg
Anti-RNP30%>95% titer >1:10,000Common (low titer)Neg (low titer)RareRare
Anti-centromereRareRare10%–15%60%–90%NegNeg
Anti-Ro (SS-A)30%RareRareNeg70%Rare
Anti-La (SS-B)15%RareRareNeg60%Rare

ANA, antinuclear antibody; anti-RNP, anti-ribonucleoprotein; anti-Sm, anti-Smith antibody; CREST, calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias; JIA, juvenile idiopathic arthritis; MCTD, mixed connective tissue disease; SLE, systemic lupus erythematosus; SSc, systemic sclerosis/scleroderma.

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