Clinical setting of patients with systemic sclerosis by serum autoantibodies

Abstract

Associations of antinuclear (ANA) and anticardiolipin (aCL) antibodies with clinical manifestations were analyzed in patients with systemic sclerosis (SSc). We studied 105 SSc patients: 28 had limited cutaneous SSc (lcSSc) involving fingers; 36 had intermediate cutaneous SSc involving limbs and face; 33 had diffuse cutaneous SSc (dcSSc) involving the trunk; 8 had a sclerosis sine scleroderma. Clinical manifestations and instrumental and laboratory findings were considered to calculate a disease score. Serum anticentromere (ACA), anti-topoisomerase I (anti-topo I) antibodies, and aCL (of IgG/IgA/IgM classes) were investigated by conventional methods. ACA positive patients (n=18), compared to ACA negative, showed higher prevalence of IcSSc (p < 0.001), lower prevalence of restrictive ventilatory defect (p=0.006), and lower disease score (p=0.008). Anti-topo I positive patients (n= 70) showed lower prevalence of lcSSc (p =0.001) compared to anti-topo I negative. In aCL positive patients (n=27) widespread skin and visceral involvement occurred more frequently than in aCL negative. The association with myocardial ischemia or necrosis (p=0.010) was significant. Occurrence of ACA excluded the coexistence of anti-topo I (p < 0.001), and aCL (p=0.037). aCL positive patients showed higher disease score in comparison with ACA positive patients (p=0.003). In conclusion ACA recognize patients with a mild disease. aCL in contrast to ACA are better than anti-topo I in recognizing the most severe pictures of SSc.

Therapeutic plasma exchange for the acute management of the catastrophic antiphospholipid syndrome: ?2-glycoprotein I antibodies as a marker of response to therapy

“…However, in this patient the potential benefit of TPE may have been overshadowed by extensive vasculopathy secondary to her underlying disease [23] and the added hypercoagulable risk associated with coexisting protein S deficiency. Antiphospholipid antibodies have been reported infrequently in patients with scleroderma [24][25][26]. Associated clinical events have included large vessel arterial thrombosis (bilateral superficial femoral artery with above-knee amputations in a patient with IgG aCL [24]), multiorgan dysfunction typical of APS in a patient with IgG (strong) and IgM (weak) aCL [25], and myocardial ischemia or necrosis in ten patients [26].…”

“…Antiphospholipid antibodies have been reported infrequently in patients with scleroderma [24][25][26]. Associated clinical events have included large vessel arterial thrombosis (bilateral superficial femoral artery with above-knee amputations in a patient with IgG aCL [24]), multiorgan dysfunction typical of APS in a patient with IgG (strong) and IgM (weak) aCL [25], and myocardial ischemia or necrosis in ten patients [26]. We have not found prior reports of detected ␤ 2 GPI antibodies in patients with scleroderma.…”

Update on autoantibodies in systemic sclerosis

“…Some authors reported an association of anticardiolipin antibodies with increased disease severity, particularly as manifested by pulmonary hypertension and endothelial injury [146][147][148][149], but these findings were not corroborated by all studies [150,151]. Antiphospholipid syndrome in association with SSc has been reported rarely, most recently in association with pregnancy loss [152][153][154].…”

Evidence‐based guidelines for the use of immunologic tests: Anticentromere, Scl‐70, and nucleolar antibodies

“…From the literature search, 59 articles assessing the correlation between SSc‐associated autoantibodies and specific aspects of prognosis met the criteria for further review. Of these, 37 unduplicated series were chosen, graded “A” (8–12), and form the basis of the recommendations (Tables 4–7 (1–3, 7, 34, 36, 41, 42, 51, 53, 59, 63, 64, 70, 80–82, 85–102). It would have been desirable to include other outcomes in this analysis, such as death, disability, or renal failure.…”

“…Type II disease includes arm, leg, and face involvement, and type III disease includes truncal involvement. Twenty‐one studies were reviewed examining ACA for defining the extent of cutaneous disease (34, 41, 42, 51, 53, 59, 63, 82, 85, 87, 89–96, 98, 99, 104) (Table 4). The sensitivity of ACA in predicting the presence of lcSSC using ACR criteria (96) was 44%; specificity 93%; and positive LR of 6.1.…”