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Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome

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Abstract

The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud’s phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma.

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References

  1. Wilson WA, Gharavi AE, Koike T et al (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42:1309–1311

    Article  PubMed  CAS  Google Scholar 

  2. Zandman-Goddard G, Tweezer-Zaks N, Shalev T et al (2007) A novel overlap syndrome: systemic sclerosis associated with antiphospholipid syndrome: a case series. Ann N Y Acad Sci 1108:497–504

    Article  PubMed  CAS  Google Scholar 

  3. Caldas CA, de Carvalho JF (2009) Primary antiphospholipid syndrome with and without Sneddon’s syndrome. Rheumatol Int [Epub ahead of print]

  4. Wielosz E, Majdan M, Zychowska I, Jeleniewicz R (2008) Coexistence of five autoimmune diseases: diagnostic and therapeutic difficulties. Rheumatol Int 28:919–923

    Article  PubMed  Google Scholar 

  5. Turkcapar N, Olmez U, Ozer D, Duzgun N, Duman M (2006) A case of overlap syndrome with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and secondary Sjögren’s syndrome. Rheumatol Int 26:841–845

    Article  PubMed  Google Scholar 

  6. Assous N, Allanore Y, Batteux F et al (2005) Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. Clin Exp Rheumatol 23:199–204

    PubMed  CAS  Google Scholar 

  7. Toya SP, Tzelepis GE (2009) The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications. Rheumatol Int 29:861–868

    Article  PubMed  Google Scholar 

  8. Slobodin G, Rosner I, Rozenbaum M, Naschitz JE, Zuckerman E, Yeshurun D (2002) Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion. Rheumatol Int 22:170–172

    Article  PubMed  Google Scholar 

  9. Malia RG, Greaves M, Rowlands LM et al (1988) Anticardiolipin antibodies in systemic sclerosis: immunological and clinical associations. Clin Exp Immunol 73:456–460

    PubMed  CAS  Google Scholar 

  10. Katayama I, Otoyama K, Kondo S et al (1990) Clinical manifestations in anticardiolipin antibody-positive patients with progressive systemic sclerosis. J Am Acad Dermatol 23:198–201

    Article  PubMed  CAS  Google Scholar 

  11. Fonollosa V, Selva A, Lima J et al (1991) Anticardiolipin antibodies in systemic sclerosis. J Am Acad Dermatol 25:133–134

    Article  PubMed  CAS  Google Scholar 

  12. Herrick AL, Heaney M, Hollis S, Jayson MIV (1994) Anticardiolipin, anticentromere and anti-Scl-70 antibodies in patients with systemic sclerosis and severe digital ischaemia. Ann Rheum Dis 53:540–542

    Article  PubMed  CAS  Google Scholar 

  13. Picillo U, Migliaresi S, Marcialis MR et al (1995) Clinical significance of anticardiolipina antibodies in patients with systemic sclerosis. Autoimmunity 20:1–7

    Article  PubMed  CAS  Google Scholar 

  14. Ihn H, Sato S, Fujimoto M et al (1996) Measurement of anticardiolipin antibodies by ELISA using β2-glycoprotein I (β2-GPI) in systemic sclerosis. Clin Exp Immunol 105:475–479

    Article  PubMed  CAS  Google Scholar 

  15. Pope JE, Thompson A (2007) The frequency and significance of anticardiolipin antibodies in scleroderma. J Rheumatol 27:1450–1452

    Google Scholar 

  16. Shapiro LS (1990) Large vessel arterial thrombosis in systemic sclerosis associated with antiphospholipid antibodies. J Rheumatol 17:685–688

    PubMed  CAS  Google Scholar 

  17. Whittaker R, Barnett A, Ryan P (1993) Antiphospholipid syndrome in scleroderma. J Rheumatol 20:1598–1600

    PubMed  CAS  Google Scholar 

  18. Antonioli CM, Danieli E, Airò P, Cattaneo R, Tincani A (2003) More on anticardiolipin and anti-beta2 glycoprotein I in systemic sclerosis. Ann Rheum Dis 62:589–590

    Article  PubMed  CAS  Google Scholar 

  19. Gupta R, Thabah MM, Gupta S, Shankar S, Kumar A (2009) Clinical significance of antiphospholipid antibodies in Indian scleroderma patients. Rheumatol Int [Epub ahead of print]

  20. Poormoghim H, Lucas M, Fertig N, Medsger TA Jr (2000) Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum 43:444–451

    Article  PubMed  CAS  Google Scholar 

  21. Minatani M, Takasaki J, Iwata H, Kinoshita M, Aotsuka S, Sumiya M (2000) Acute interstitial pneumonia associated with antiphospholipid syndrome in a patient with systemic sclerosis. Clin Exp Rheumatol 18:786

    PubMed  CAS  Google Scholar 

  22. Marie I, Jouen F, Hellot M-F, Levesque H (2008) Anticardiolipin and anti-b2 glycoprotein I antibodies and lupus-like anticoagulant: prevalence and significance in systemic sclerosis. Br J Dermatol 158:141–144

    PubMed  CAS  Google Scholar 

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Acknowledgments

JF Carvalho received grants from the Federico Foundation and CNPq (300665/2009-1).

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Correspondence to Jozélio Freire de Carvalho.

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Leite, P.d., de Carvalho, J.F. Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome. Rheumatol Int 32, 3265–3268 (2012). https://doi.org/10.1007/s00296-010-1482-6

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  • DOI: https://doi.org/10.1007/s00296-010-1482-6

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