Springer Seminars in Immunopathology

, Volume 4, Issue 3, pp 253–273 | Cite as

Ro (SSA) and La (SSB) antibodies

  • Elaine L. Alexander
  • Thomas T. Provost
Article

Summary

This review traces the historical development of information regarding the Ro (SSA) and La (SSB) autoantibody systems over the past twenty years. Clinical and serologic findings are integrated with fundamental observations in this rapidly expanding area of research. Retrospective analysis of the physicochemical properties of the antigens and the cellular staining characteristics of antibodies to these antigens suggest that SjD and Ro and SSA, as well as SjT and La, SSB, and Ha antigens probably are similar macromolecules. The immunologic identity of Ro with SSA and La with SSB and Ha has been established previously. Antibodies to these antigens are directed against macromolecules containing small RNA nucleotides.

Antibodies to the Ro (SSA)-La(SSB) antigen system commonly are detected in the sera of patients with systemic lupus erythematosus and Sjögren's syndrome and appear to be of diagnostic significance. These antibodies occur in up to one quarter of patients with systemic lupus erythematosus (SLE) without the sicca complex, but also in patients with ANA negative SLE who have a prominent photosensitive dermatitis and may have serious renal disease, subacute cutaneous SLE, and in infants and mothers of infants with neonatal SLE. Thus, these antibody systems form a serologic link between many unusual connective tissue diseases and systemic SLE.

Antibodies to Ro (SSA)-La(SSB) are associated not only with Sjögren's syndrome occurring alone, but also with Sjögren's syndrome occurring in the setting of other connective tissue diseases including SLE and rheumatoid arthritis. Anemia, leukopenia, and thrombocytopenia, as well as hyperglobulinemia and the presence of rheumatoid factor, cryoglobulins, and antibodies to nuclear antigens are associated significantly with Ro positivity in Sjögren's syndrome patients. There is a striking association of vasculitis in the clinical setting of Sjögren's syndrome with the presence of antibodies to Ro (SSA). In addition to peripheral nerve involvement, unusual central nervous system manifestations as well as myositis occur in these Ro(SSA) positive Sjögren's syndrome patients. Deposition of immunoglobulin and complement within vessel walls of kidney and muscle from Ro positive patients with Sjögren's syndrome suggests a possible role for immune complex deposition in the pathogenesis of the vasculitis.

Keywords

Systemic Lupus Erythematosus Vasculitis Myositis Connective Tissue Disease Immune Complex Deposition 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Akizuki M, Powers R, Hohnan HR (1977) A soluble acidic protein of the cell nucleus which reacts with serum from patients with systemic lupus erythematosus and Sjögren's syndrome. J Clin Invest 59: 264PubMedGoogle Scholar
  2. 2.
    Alexander EL, Hirsch TJ, Arnett FC (1981) The Ro(SSA) and La(SSB) antibody system and Sjögren's syndrome. Rheumatol, in pressGoogle Scholar
  3. 3.
    Alexander EL, Hirsch T, Arnett FC (1980) The clinical spectrum of anti-Ro antibody. Arthritis Rheum 23: 648Google Scholar
  4. 4.
    Alexander EL, Alexander GE, Provost TT (1981) Central nervous system vasculitis associated with Sjögren's syndrome and anti-Ro(SSA) and La(SSB) antibodies. (Neurology, in press)Google Scholar
  5. 5.
    Alspaugh MA, Tan EM (1975) Antibodies to cellular antigens in Sjögren's syndrome. J Clin Invest 55: 1067PubMedGoogle Scholar
  6. 6.
    Alspaugh MA, Talal N, Tan EM (1976) Differentiation and characterization of autoantibodies and their antigens in Sjögren's syndrome. Arthritis Rheum 19: 216PubMedGoogle Scholar
  7. 7.
    Alspaugh M, Maddison P (1979) Resolution of the identity of certain antigen antibody systems in systemic lupus erythematosus and Sjögren's syndrome: An interlaboratory collaboration. Arthritis Rheum 22: 796PubMedGoogle Scholar
  8. 8.
    Asheron GL (1959) Antibodies against nuclear and cytoplasmic cell constituents in systemic lupus erythematosus and other diseases. Br J Exp Pathol 40: 209PubMedGoogle Scholar
  9. 9.
    Anderson JR, Gray KG, Beck JS (1961) Precipitating autoantibodies in Sjögren's disease. Lancet ii: 456Google Scholar
  10. 10.
    Anderson JR, Gray KG, Beck JS (1962) Precipitating autoantibodies in the connective tissue diseases. Ann Rheum Dis 21: 360PubMedGoogle Scholar
  11. 11.
    Beck JS (1962) Partial identification of the “speckled” nuclear antigen. Lancet 1: 241PubMedGoogle Scholar
  12. 12.
    Beck JS, Anderson JR, Block KJ (1965) Antinuclear and precipitating autoantibodies in Sjögren's syndrome. Ann Rheum Dis 24: 16PubMedGoogle Scholar
  13. 13.
    Bell DA, Maddison P (1980) HLA antigens in relationship to serologic subsets of systemic lupus erythematosus (SLE). Clin Res 28: 604 (A)Google Scholar
  14. 14.
    Bloch KH, Buchanan WW, Wohl MJ (1965) Sjögren's syndrome: A clinical, pathological, and serological study of sixty-two cases. Medicine 44: 187PubMedGoogle Scholar
  15. 15.
    Bunim JJ (1961) A broader spectrum of Sjögren's syndrome and its pathogenetic implications. Ann Rheum Dis 20: 1PubMedGoogle Scholar
  16. 16.
    Chameides L, Truex RC, Vetter V (1977) Association of maternal systemic lupus erythematosus with congenital complete heart block. N Engl J Med 297: 1204PubMedGoogle Scholar
  17. 17.
    Clark G, Reichlin M, Tomasi TB (1969) Characterization of a soluble cytoplasmic antigen reactive with sera from patients with systemic lupus erythematosus. J Immunol 102: 117PubMedGoogle Scholar
  18. 18.
    Draznin TH, Esterly NB, Furey NL (1979) Neonatal lupus erythematosus. J Am Acad Dermatol 1: 437PubMedGoogle Scholar
  19. 19.
    Franco HL, Weston WL, Peebles C (1981) Autoantibodies directed against sicca syndrome antigens in the neonatal lupus syndrome. J Am Acad Dermatol 4: 67PubMedGoogle Scholar
  20. 20.
    Gajdusek DC (1958) An “autoimmune” reaction against human tissue antigens in certain acute and chronic diseases -I Serological investigations. Arch Intern Med 101: 9Google Scholar
  21. 20a.
    Gladman DA, Chalmers A, Urowitz MB (1978) Systemic lupus erythematosus with negative LE cells and antinuclear factor. J Rheumatol 5: 142PubMedGoogle Scholar
  22. 21.
    Jones BR (1958) Lacrimal and salivary precipitating antibodies in Sjögren's syndrome. Lancet ii: 773Google Scholar
  23. 22.
    Jordon RE, Provost TT (1976) The complement system and the skin. In Malkinson FD, Pearson RW (eds) Year Book of Dermatology. Year Book Medical Publishers, p 7Google Scholar
  24. 23.
    Kassan SS, Gardy M (1978) Sjögren's syndrome: An update and overview. Am J Med 64: 1037PubMedGoogle Scholar
  25. 24.
    Kassan SS, Akizuki M, Steinberg AD (1977) Antibody to a soluble acidic nuclear antigen in Sjögren's syndrome. Am J Med 63: 328PubMedGoogle Scholar
  26. 25.
    Kephardt D, Hood A, Provost TT (1981) Neonatal lupus: Serologic findings. J Invest Dermatol, in pressGoogle Scholar
  27. 26.
    Koffler D, Schur PM, Kunkel HG (1967) Immunological studies concerning the nephritis of systemic lupus erythematosus. J Exp Med 126: 607PubMedGoogle Scholar
  28. 27.
    Koffler D, Agnello V, Kunkel HG (1974) Polynucleotide immune complexes in serum and glomeruli of patients with systemic lupus erythematosus. Am J Pathol 74: 109PubMedGoogle Scholar
  29. 28.
    Lerner MR, Steitz JA (1979) Antibodies to small nuclear RNA's complexed with proteins are produced by patients with systemic lupus erythematosus. Proc Natl Acad Sci USA 76: 5495PubMedGoogle Scholar
  30. 29.
    Mackay IR, Gajdusek DC (1958) An “autoimmune” reaction against human tissue antigens in certain acute and chronic diseases. II Clinical correlations 101: 30Google Scholar
  31. 30.
    Maddison PJ, Provost TT, Reichlin M (1981) Serological findings in patients with “ANA-negative” systemic lupus erythematosus. Medicine 60: 87PubMedGoogle Scholar
  32. 31.
    Maddison PJ, Reichlin M (1979) Deposition of antibodies to a soluble cytoplasmic antigen in the kidneys of patients with systemic lupus erythematosus. Arthritis Rheum 22: 858PubMedGoogle Scholar
  33. 32.
    Maddison P, Mogavero H, Provost TT (1979) The clinical significance of autoantibodies to a soluble cytoplasmic antigen in systemic lupus erythematosus and other connective tissue diseases. J Rheumatol 6: 189PubMedGoogle Scholar
  34. 33.
    Martinez-Lavin M, Vaughan JH, Tan EM (1979) Autoantibodies and the spectrum of Sjögren's syndrome. Ann Intern Med 91: 185PubMedGoogle Scholar
  35. 34.
    Mattioli M, Reichlin M (1974) Heterogeneity of RNA protein antigens reactive with sera of patients with systemic lupus erythematosus: Description of a cytoplasmic nonribosomal antigen. Arthritis Rheum 17: 421PubMedGoogle Scholar
  36. 35.
    Moutsopoulos HM, Klippel JH, Pavlidis N (1980) Correlative histologic and serologic findings of sicca syndrome in patients with systemic lupus erythematosus. Arthritis Rheum 23: 36PubMedGoogle Scholar
  37. 36.
    Pittsley RA, Talal N (1980) Neuromuscular complications of Sjögren's syndrome. in Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology. Vol 39. North Holland Publishing Co, Amsterdam, p 419Google Scholar
  38. 37.
    Provost TT, Ahmed AR, Maddison PJ (1977) Antibodies to cytoplasmic antigens in lupus erythematosus- Serologic marker for systemic disease. Arthritis Rheum 20: 1457PubMedGoogle Scholar
  39. 38.
    Provost TT, Andres G, Maddison PJ (1980) Lupus band test in untreated SLE patients: Correlation of immunoglobulin deposition in the skin of the extensor forearm with clinical renal disease and serological abnormalities. J Invest Derm 74: 407PubMedGoogle Scholar
  40. 39.
    Reichlin M, Mattioli M (1974) Antigens and antibodies characteristic of systemic lupus erythematosus. Bull Rheum Dis 24: 756Google Scholar
  41. 40.
    Ringel SP, Forstot JZ, Butcher D (1980) Sjögren's syndrome and polymyositis. Neurology (NY) 30: 424Google Scholar
  42. 41.
    Silberberg OH, Drachman DA (1962) Late-life myopathy occurring with Sjögren's syndrome. Arch Neurol 6: 428PubMedGoogle Scholar
  43. 42.
    (1980) Sjögren's syndrome (sicca syndrome): Current issues, NIH Conference, Moderator: HM Moutsopoulos. Ann Intern Med 92: 212Google Scholar
  44. 43.
    Sontheimer RD, Thomas JR, Gilliam JN (1979) Subacute cutaneous lupus erythematosus. Arch Dermatol 115: 1409PubMedGoogle Scholar
  45. 44.
    Sontheimer RD, Stastny P, Maddison P (1980) Immunological and HLA associations in subacute lupus erythematosus (SOLE). Clin Res 28: 582(A)Google Scholar
  46. 45.
    Synkowski DR, Provost TT (1980) Correlation of cutaneous and systemic features with nuclear and cytoplasmic antibodies in systemic lupus erythematosus. Clin Res 28: 583(A)Google Scholar
  47. 46.
    Wasicek CA, Maddison PJ, Reichlin M (1979) Occurrence of antibodies to single stranded DNA in ANA negative patients. Clin Exp Immunol 37: 190PubMedGoogle Scholar
  48. 47.
    Weston WL, Franco HL, Tan E (1980) Association of antibodies to sicca syndrome antigens in newborns with lupus erythematosus and their mothers. Clin Res 28: 582(A)Google Scholar

Copyright information

© Springer-Verlag 1981

Authors and Affiliations

  • Elaine L. Alexander
    • 1
  • Thomas T. Provost
    • 1
  1. 1.Departments of Medicine and DermatologyJohns Hopkins School of MedicineBaltimoreUSA

Personalised recommendations