Clinical Rheumatology

, Volume 24, Issue 6, pp 652–654 | Cite as

Unusual association of systemic sclerosis and ankylosing spondylitis

  • Cinira S. Soledade
  • Percival D. Sampaio-Barros
  • Adil M. Samara
  • João Francisco Marques-Neto

Systemic sclerosis (SSc) and ankylosing spondylitis (AS) are diseases that present distinct epidemiologic patterns [1, 2]. Since apparently no physiopathologic link exists between them, the chances of these two infrequent diseases occurring in the same patient are remote. In a review of the literature only four reports were found. The first one reported a patient with a long-standing untreated AS who presented with a malignant lymphoma and simultaneously developed SSc [3]. The second was a report of a patient with a combination of scleroderma and AS, whose presenting complaint was transfer dysphagia due to impaired relaxation of the upper esophageal sphincter as a result of tight overlying cervical skin or sclerodermatous involvement of the sphincter itself [4]. The third described a 40-year-old patient with a diagnosis of spondyloarthropathy associated with the CREST (calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia) syndrome [5]. A description...


Ankylose Spondylitis Interstitial Lung Disease Force Vital Capacity Pulmonary Involvement Idiopathic Inflammatory Myopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Braun J, Bollow M, Remlinger G et al (1998) Prevalence of spondyloarthropathies in HLA-B27 positive and negative blood donors. Arthritis Rheum 41:58–67CrossRefPubMedGoogle Scholar
  2. 2.
    Mayes M (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29:239–254PubMedGoogle Scholar
  3. 3.
    O’Hare JA, Murnaghan D (1982) Ankylosing spondylitis, lymphoma and scleroderma—a unique conjunction. Ir J Med Sci 151:351–352PubMedGoogle Scholar
  4. 4.
    Witt P, Thomas E (1987) Transfer dysphagia in a patient with the rare combination of scleroderma and ankylosing spondylitis. J Natl Med Assoc 79:993–996PubMedGoogle Scholar
  5. 5.
    Pham T, Daumen-Legre V, Lafforgue P (1999) Concomitant spondyloarthropathy and CREST syndrome. Clin Exp Rheum 17:754Google Scholar
  6. 6.
    Benedek TG, Rodnan GP (1982) The early history and nomenclature of scleroderma and of its differentiation from sclerema neonatorum and scleroedema. Semin Arthritis Rheum 12:52–67CrossRefPubMedGoogle Scholar
  7. 7.
    Steen VD, Medsger TA Jr, Rodnan GP (1982) D-penicillamine therapy in progressive systemic sclerosis (scleroderma). Ann Intern Med 97:652–659PubMedGoogle Scholar
  8. 8.
    Van der Linden S, Valkenburg HA, Cats A (1984) Evaluation of diagnostic criteria for ankylosing spondylitis: a proposal for modification of the New York criteria. Arthritis Rheum 27:361–368PubMedGoogle Scholar
  9. 9.
    Masi AT, Rodnan GP, Medsger TA Jr et al (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590PubMedGoogle Scholar
  10. 10.
    Rabin BS, Rodnan GP, Bassion S, Gill TJ (1975) HbA antigens in progressive systemic sclerosis (scleroderma). Arthritis Rheum 18:381–382PubMedGoogle Scholar
  11. 11.
    Langevitz P, Buskila D, Gladman DD, Darlington GA, Farewell VT, Lee P (1992) HLA alleles in systemic sclerosis: association with pulmonary hypertension and outcome. Br J Rheumatol 31:609–613PubMedGoogle Scholar
  12. 12.
    Reveille JD, Fischback M, McNearney T et al (2001) Systemic sclerosis in 3 US ethnic groups: a comparison of clinical, sociodemographic, serologic, and immunogenetic determinants. Semin Arthritis Rheum 30:332–346CrossRefPubMedGoogle Scholar
  13. 13.
    Sampaio-Barros PD, Bértolo MB, Kraemer MHS, Marques Neto JF, Samara AM (2001) Primary ankylosing spondylitis: patterns of disease in 147 Brazilian patients. J Rheumatol 28:560–565PubMedGoogle Scholar
  14. 14.
    Conde RA, Sampaio-Barros PD, Donadi EA et al (2003) Frequency of HLA-B27 alleles in Brazilian AS patients. J Rheumatol 30:2511–2512PubMedGoogle Scholar
  15. 15.
    Olsen NJ, King LE Jr, Park JH (1996) Muscle abnormalities in scleroderma. Rheum Dis Clin North Am 22:783–796PubMedGoogle Scholar
  16. 16.
    Faus-Riera S, Martínez-Pardo S, Blanch-Rubió J, Benito-Ruiz P, Duró-Pujol JC, Corominas-Torres JM (1991) Muscle pathology in ankylosing spondylitis: clinical, enzymatic, electromyographic and histologic correlation. J Rheumatol 18:1368–1371PubMedGoogle Scholar
  17. 17.
    Silver RM (1996) Clinical problems: the lungs. Rheum Dis Clin North Am 22:825–840PubMedGoogle Scholar
  18. 18.
    Turetschek K, Ebner W, Fleischmann D et al (2000) Early pulmonary involvement in ankylosing spondylitis: assessment with thin-section CT. Clin Radiol 53:632–636CrossRefGoogle Scholar
  19. 19.
    White B, Moore W, Wigley FM, Xiao HQ, Wise RA (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132:947–954PubMedGoogle Scholar

Copyright information

© Clinical Rheumatology 2004

Authors and Affiliations

  • Cinira S. Soledade
    • 1
  • Percival D. Sampaio-Barros
    • 1
  • Adil M. Samara
    • 1
  • João Francisco Marques-Neto
    • 1
  1. 1.Rheumatology Unit, Department of Internal MedicineState University of Campinas Faculty of Medical Sciences (UNICAMP)CampinasBrazil

Personalised recommendations