Table 1. Clinically most relevant ANA specificities in inflammatory connective tissue diseases.

Autoantibody	Frequency of detection in different CTDs	Clinical significance
anti-dsDNA	> 95% in active SLE with renal involvement 50–70% in active SLE without renal involvement < 40% in inactive SLE	ACR and SLICC classification criterion for SLE Prognostic marker for SLE (a marker of renal involvement, disease activity, use in therapy monitoring)
anti-SS-A (Ro60)	60–96% in primary SjS 40–60% in secondary SjS 25–60% SLE 60–100% SCLE 90% NLE	ACR/EULAR classification criterion for primary SjS Associated with extraglandular manifestations Found in mothers of children with NLE
anti-Ro52/TRIM21	17–63% SjS 23% SLE 20% SSc 30% of patients with the antisynthetase syndrome (in up to 72% of patients with positive anti-Jo1) in non-CTD diseases (28% PBC, 17% AIH)	Found in various autoimmune diseases
anti-SS-B (La)	40–70% in primary SjS 5–50% in secondary SjS 19–30% in SLE 25–80% in SCLE 70% NLE	Usually occur with SS-A antibodies Co-occurrence with SS-A antibodies usually correlates with fewer renal manifestations
anti-Sm	5–10% SLE	ACR and SLICC classification criterion for SLE High specificity for SLE
anti-RNP	100% MCTD, 13–32% SLE	Serological hallmark of MCTD (when present in high titer)
anti-Topo I/Scl70	65% in diffuse SSc	ACR/EULAR classification criterion for SSc Related to the more rapidly progressive systemic form of SSc
anti-CENP B	57–82% of patients with CREST syndrome 3–12% of patients with diffuse cutaneous Sc	ACR/EULAR classification criterion for SSc Associated with slowly developing limited cutaneous form of SSc (CREST syndrome)
anti-RNA-Pol III	3–19% in SSc	ACR/EULAR classification criterion for SSc Associated with diffuse skin manifestations and renal crisis
anti-Jo-1	24–30% IIM	ACR/EULAR classification criterion for adult and juvenile IIM Associated with interstitial pulmonary fibrosis
anti-PM/Scl	24–55% polymyositis/scleroderma overlap syndrome, 8–12% IIM, 1–16% SSc	Diagnostic marker for polymyositis/scleroderma overlap syndrome
anti-PCNA	3% SLE	Previously considered to be very specific for SLE
anti-ribosomal P	10–35% SLE	High specificity for SLE
anti-histones	92–95% drug-induced lupus, 50–80% SLE	High specificity for drug-induced lupus
anti-nucleosomes	56–90% SLE	High specificity for SLE
dsDNA – double stranded DNA. CTDs – Connective tissue diseases. SLE – Systemic lupus erythematosus. ACR – American College of Rheumatology. SLICC – Systemic Lupus International. Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus. SS-A (Ro60) – antigen A associated with Sjögren syndrome (60 kDa ribonucleoprotein). SjS – Sjögren syndrome. EULAR – European League Against Rheumatism. SCLE – Subacute cutaneous lupus erythematosus. NLE – Neonatal lupus erythematosus. Ro52/TRIM21 – 52 kDa ribonucleoprotein/ Tripartite motif-containing protein 21. SSc – Systemic sclerosis. PBC – Primary biliary cholangitis. AIH – Autoimmune hepatitis. CHB – Congenital heart block. SS-B (La) – antigen B associated with Sjögren syndrome. Sm – Smith antigen. RNP – ribonucleoprotein complex. MCTD – Mixed connective tissue disease. Scl-70/Topo-I – 70kDa antigen associated with scleroderma/Topoisomerase I. CENP-B – centromere protein B. CREST – Calcinosis, Raynaud’s syndrome, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. RNA-Pol-III – RNA polymerase III. Jo-1 – histidyl-tRNA synthetase. IIM – Idiopathic inflammatory myopathies. PM/Scl – antigen associated with Polymyositis / Scleroderma overlap syndrome. PCNA –proliferating cell nuclear antigen. ribosomal P – ribosomal P protein.