Atypical Autoantibodies in Patients with Primary Sjögren Syndrome: Clinical Characteristics and Follow-Up of 82 Cases
Section snippets
Patients
We studied 402 patients diagnosed with primary SS seen consecutively in our Department since 1994. All patients fulfilled 4 or more of the preliminary diagnostic criteria for SS proposed by the European Community Study Group in 1993 (6) (including as mandatory criterion either positive immunological markers or salivary lip biopsy) and underwent a complete history and physical examination. Diagnostic tests for SS (rose bengal staining, Schirmer test, parotid scintigraphy, and salivary gland
General Description
Eighty-two (20%) patients had atypical autoantibodies (Table 1): 36 had antiphospholipid antibodies (aPL) (19 positive IgG-aCL, 19 LA, and 6 IgM-aCL), 21 positive anti-DNA antibodies, 13 ANCA, 10 anti-RNP, 8 ACA, 6 anti-Sm, 2 anti-Scl70, and 1 anti-Jo-1 antibodies. In 15 patients, more than 1 of these autoantibodies were detected. There were 77 (94%) women and 5 (6%) men (female:male ratio, 15:1), with a mean age at protocol inclusion of 57.3 ± 1.77 years (range, 20 to 87). A retrospective
Discussion
The diagnosis of SAD is a clinical challenge based on the application of internationally agreed sets of classification criteria that often combine diverse organ-specific manifestations with findings of circulating autoantibodies. In primary SS, the clinical significance of autoantibodies considered typical of other SAD is controversial. We have identified 224 previously reported cases with atypical autoantibodies (Table 2) (8, 10, 11, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36,
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2022, Seminars in Arthritis and RheumatismCitation Excerpt :Other autoantibodies previously found to be detected in a subgroup of SS patients include those directed against centromere antigens (ACA), namely centromere proteins (CENP). Though they are classically detected in limited forms of systemic sclerosis (SSc), earlier reports revealed their presence in a subset of SS [6] sharing features with limited SSc (lcSSc) [7–13] in association with characteristic ultra-sonographic salivary gland findings such as hyperechoic bands [14]. Beyond anticentromere antibodies, many SSc-specific reactivities directed against nuclear and nucleolar antigens have been identified in recent years and included among others, antibodies against topoisomerase I (Scl-70), RNA-polymerase III (RP11, RP155), fibrillarin, nucleolar organizing region 90 (NOR90), Th/To ribonucleoprotein (Th/To) and polymyositis/scleroderma (PM/Scl) autoantigens.
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2022, Physician Assistant Clinics