Summary
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 (IcSSc) 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 IcSSc (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.
Similar content being viewed by others
References
Medsger TA Jr. Systemic sclerosis (scleroderma), eosinophilic fasciitis, and calcinosis. In: Mc Carty DJ, Philadelphia, Lea & Febiger, editors, Arthritis and Allied Conditions, 1993: 1253–1292.
White B. Immune abnormalities in systemic sclerosis. Clin Dermatol 1994;12:349–359.
Giordano M, Valentini G, Migliaresi S, Picillo U, Vatti M. Different antibody patterns and different prognoses in patients with scleroderma with various extent of skin sclerosis. J Rheumatol 1986; 13: 911–916.
Steen VD, Powell DL, Medsger TA Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum 1988; 31: 196–203.
Hughes GRV, Harris EN, Gharavi AE. The anticardiolipin syndrome. J Rheumatol 1986; 13: 486–489.
Alarçon-Segovia D, Deleze M, Oria CV, Sanchez-Guerrero J, Gomez-Pacheco L, Cabiedes J, Fernandez L, Ponce de Leon S. Antiphospholipid antibodies and antiphospholipid syndrome in systemic lupus erythematosus. A prospective analysis of 500 consecutive patients. Medicine 1989; 68: 353–365.
Picillo U, Migliaresi S, Marcialis MR, Longobardo A, La Palombara F, Tirri G. Longitudinal survey of anticardiolipin antibodies in systemic lupus erythematosus. Relationships with clinical manifestations and disease activity in an Italian series. Scand J Rheumatol 1992; 21: 271–276.
Asherson RA, Khamashta MA, Ordi-Ros J, Derksen RHWM, Machin SJ, Path FRC, Barquinero J, Outt HH, Harris EN, Phil M., Vilardell-Torres M, Hughes GRV. The “primary” antiphospholipid lipid syndrome: major clinical and serological features. Medicine (Baltimore) 1989; 68: 366–73.
Seibold JR, Knight PJ, Peter JB. Anticardiolipin antibodies in systemic sclerosis. Arthritis Rheum 1986; 29: 1052–3.
Bamberga P, Asero R, Vismara A, Brucato A, Riboldi P, Meroni PL. Anticardiolipin antibodies in progressive systemic sclerosis (PSS). Clin Exp Rheumatol 1987; 5: 387–8.
Malia RG, Greaves M, Rowlands LM, Lawrence ACK, Hume A, Rowell NR, Moult J, Holt CM, Lindsey N, Hughes P. Anticardiolipin antibodies in systemic sclerosis: immunological and clinical associations. Clin Exp Immunol 1988; 73: 456–60.
Buchanan RRC, Wardlaw JR, Riglar, AG, Littlejohn, GO, Miller MH. Antiphospholipid antibodies in the connective tissue diseases: their relation to the antiphospholipid syndrome and forme fruste disease. J Rheumatol 1989; 16: 757–61.
Picillo U, Migliaresi S, Marcialis MR, Ferruzzi AM, Tirri G. Clinical significance of anticardiolipin antibodies in patients with systemic sclerosis. Autoimmunity 1995; 20: 1–7.
Masi AT, Rodnan GP, Medsger TA Jr, Altman RD, D'Angelo WA, Fries JF, LeRoy EC, Kirsner AB, MacKenzie AH, McShane DJ, Myers AR, Sharp GC. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581–90.
Rodnan GP, Jablonska S, Medsger TA Jr. Classification and nomenclature of progressive systemic sclerosis (scleroderma). Clin Rheum Dis 1979; 5: 5–14.
Bernstein RM, Steigerwald JC, Tan EM. Association of antinuclear and antinucleolar antibodies in progressive systemic sclerosis. Clin Exp Immunol 1982; 48: 43–51.
Lopez LR, Santos ME, Espinoza LR, La Rosa FG. Clinical significance of immunoglobulin A versus immunoglobulins G and M anticardiolipin antibodies in patients with systemic lupus erythematosus. Correlation with thrombosis, thrombocytopenia and recurrent abortion. Am J Clin Pathol 1992; 98: 449–54.
Jones JV, Eastwood BJ, Jones E, James H, Mansour M. Antiphospholipid antibodies in a healthy population: methods for estimating the distribution. J Rheumatol 1995; 22: 55–61.
Glantz SA. Primer of biostatistics. New York: McGraw-Hill, 1987.
Rothfield NF. Autoantibodies in scleroderma. Rheum Dis Clin North Am 1992; 18: 483–98.
Weiner ES, Earnshaw WC, Senécal J-L, Bordwell B, Johnson P, Rothfield NF. Clinical associations of anticentromere antibodies and antibodies to topoisomerase I: a study of 355 patients. Arthritis Rheum 1988; 31: 378–85.
Reveille JD, Durban E, Goldstein R, Moreda R, Arnett FC. Racial differences in the frequencies of scleroderma-related autoantibodies. Arthritis Rheum 1992; 35: 216–18.
McNeilage, L.J., Youngchaiyud, U., Whittingham, S. Racial differences in antinuclear antibody patterns and clinical manifestations of scleroderma. Arthritis Rheum 1989; 32: 54–60.
Barnett AJ, Miller MH, Littlejohn GO. A survival study of patients with scleroderma diagnosed over 30 years (1953–1983): the value of a simple cutaneous classification in early stages of the disease. J Rheumatol 1988; 15: 276–83.
Kuwana M, Kaburaki J, Okano Y, Tojo T, Homma M. Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis. Arthritis Rheum 1994; 37: 75–83.
Picillo U, Migliaresi S, Vatti M, Marcialis MR, Ferruzzi AM, Tirri G. Demographic differences in the frequencies of scleroderma-related autoantibodies. Arthritis Rheum 1993; 36: 1332–3.
Kuwana M, Okano Y, Kaburaki J, Tojo T, Medsger TA Jr. Racial differences in the distribution of systemic sclerosis-related serum antinuclear antibodies. Arthritis Rheum 1994; 37: 902–6.
Steen VD, Conte C, Owens GR, Medsger TA Jr. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994; 37: 1283–9.
Kallenberg CGM, Pastoor GW, Wouda AA, The TH Antinuclear antibodies in patients with Raynaud's phenomenon: clinical significance of anticentromere antibodies. Ann Rheum Dis 1982; 41: 382–7.
Vlachoyiannopoulos, P.G., Drosos, A.A., Wiik, A., Moutsopoulos, H.M. Patients with anticentromere antibodies, clinical features, diagnoses and evolution. Br J Rheumatol 1993; 32: 297–301.
Migliaresi S, Picillo U, Tirri G, Improta RDG, La Palombara F, Giordano M. Infrequency of anticentromere antibody in patients without systemic sclerosis and without Raynaud's phenomenon. Arthritis Rheum 1987; 30: 358–9.
Wigley FM, Wise RA, Miller R, Needleman BW, Spence RJ. Anticentromere antibody as a predictor of digital ischemic loss in patients with systemic sclerosis. Arthritis Rheum 1992; 35: 688–93.
Herrick AL, Heaney M, Hollis S, Jayson MIV. Anticardiolipin, anticentromere and anti-Scl-70 antibodies in patient with systemic sclerosis and severe digital ischaemia. Ann Rheum Dis 1994; 53: 540–2.
Ruffatti A, Calligaro A, Bombardieri S, Gambari PF, Todesco S. Association of anti-centromere and anti-Scl 70 antibodies in scleroderma: report of two cases. J Clin Lab Immunol 1985; 16: 227–9.
Catoggio LJ, Bernstein RM, Black CM, Hughes GRV, Maddison PJ. Serological markers in progressive systemic sclerosis: clinical correlations. Ann Rheum Dis 1983; 42: 23–7.
Reimer G, Rose KM, Scheer U, Tan EM. Autoantibody to RNA polymerase I in scleroderma sera. J Clin Invest 1987; 79: 65–72.
Reimer G, Steen VD, Penning CA, Medsger TA Jr, Tan EM. Correlates between autoantibodies to nucleolar antigens and clinical features in patients with systemic sclerosis (scleroderma). Arthritis Rheum 1988; 31: 525–32.
Oddis CV, Okano Y, Rudert WA, Trucco M, Duquesnoy RJ, Medsger TA Jr. Serum autoantibody to the nucleolar antigen PM-Scl. Clinical and immunogenetic associations. Arthritis Rheum 1992; 35: 1211–7.
Okano Y, Steen VD, Medsger TA Jr. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992; 35: 95–100.
Guldner H-H, Szosteki C, Vosberg H-P, Lakomek HJ, Penner E, Bautz FA. Scl-70 autoantibodies from scleroderma patients recognize a 95 kDa protein identified as topoisomerase I. Chromosoma 1986; 94: 132–8.
Carreras LO, Vermylen JG. "Lupus" anticoagulant and thrombosis: possible role of inhibition of prostacyclin formation. Thromb Haemost 1982; 48: 38–40.
Tsakiris DA, Marbet GA, Makris PE, Settas L, Duckert F. Impaired fibrinolysis as an essential contribution to thrombosis in patients with lupus anticoagulant. Thromb Haemost 1989; 61: 175–7.
Del Papa N, Meroni PL, Tincani A, Harris EN, Pierangeli SS, Barcellini W, Borghi MO, Balestrieri G, Zanussi C. Relationship between anti-phospholipid and anti-endothelial cell antibodies: further characterization of the reactivity on resting and cytokine-activated endothelial cells. Clin Exp Rheumatol 1992; 10: 37–42.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202–5.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Picillo, U., Migliaresi, S., Marcialis, M.R. et al. Clinical setting of patients with systemic sclerosis by serum autoantibodies. Clin Rheumatol 16, 378–383 (1997). https://doi.org/10.1007/BF02242455
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02242455