Subacute skin lesions begin as erythematous papules or plaques and can evolve either into annular lesions similar to erythema annular centrifugum or into scaling that resembles psoriasis or lichen planus. They tend to occur on sun-exposed areas of the body but can have a generalized distribution, they are nonfixed and nonscarring, and they follow a waxing and waning course. This lupus subset was first described by Sontheimer et al. (Sontheimer et al. 1979) in 1979. Two thirds of these patients show antibodies to Ro/SSA, mostly as anti-60-kDa Ro/SSA and annular lesions, with an HLA-A1, -B8, -DR3 association (Bielsa et al. 1991). Seven percent to 21% of patients with lupus present these cutaneous eruptions (McCauliffe 1997).
Comparing anti-Ro/SSA-positive with anti-Ro/SSA-negative patients with SLE, there is a correlation between anti-Ro/SSA-positive and photosensitivity and anti-
Ro/SSA-negative and nephritis (Mond et al. 1989). The typical presentation of a patient with anti-Ro/SSA antibodies covers features of Sjogren's syndrome with xerostomia, xerophthalmia, a history of parotid swelling, and arthralgias. Beyond ANAs (sometimes only weakly positive) and anti-Ro/SSA antibodies (often together with anti-La/SSB antibodies), many of these patients prove to have high titers of rheumatoid factors (>80%), elevated erythrocyte sedimentation rates, and hypergammaglobulinemia, which may be associated with purpura. Clinically, a polyadenopathy of lymph nodes, subfebrile states, and a tendency toward leukopenia marks the transition to SLE (also classified as Sjogren's/lupus overlap syndrome). When those patients develop additional features of systemic disease (i. e., arthritis, serositis, hemolytic anemia, and renal or cerebral involvement), anti-ds-DNA antibodies are usually present. Other typical associations with anti-Ro/SSA antibodies are C2 or C4 complement deficiency, interstitial pneumonitis, and late-onset lupus.
Anti-Ro/SSA antibodies are also related to congenital heart block and neonatal lupus, which has to be kept in mind in patients with SCLE. Half of these patients fulfill the ACR criteria for SLE (Callen and Klein 1988) but in general have a more favorable long-term prognosis.
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