Skip to main content

Advertisement

Log in

Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

The objective of this study is to test the association of the severity of nailfold capillaroscopy (NFC) abnormalities with mortality in systemic sclerosis (SSc). One hundred and seventy SSc patients underwent an extensive evaluation (including high-resolution computed tomography, pulmonary function tests, and Doppler echocardiography) at baseline following a standard protocol. Capillary loss on NFC was evaluated using the avascular score (AS, ranging from 0 to 3), and the mean number of ectasias, megacapillaries, and hemorrhages per finger was also recorded. After a mean period of 10.1 ± 4.9 years, the life status of the patients was ascertained. Univariate and multivariate Cox proportional hazards models were used for statistical analysis. Overall, 73 patients died. By univariate Cox analysis, the AS was significantly associated with mortality (hazard ratio [HR] = 1.64, 95% CI 1.22 to 2.19, p = 0.001). In our study, this association was stronger than that of race, gender, anticentromere antibodies, anti-topoisomerase I antibodies, and form of disease and had similar strength to that of skin score in univariate analyses. However, after controlling for a combination of variables (age, skin score, gender, race, signs of peripheral ischemia, and extent of interstitial lung disease, all independently associated with mortality), the association of AS with mortality was blunted (HR = 1.15, 95% CI 0.80 to 1.65, p = 0.445). Other NFC variables were not related to mortality. AS was associated with higher risk of death and, despite not having an independent association with mortality after controlling for a set of demographic and clinical variables, may be a useful tool in prognostic evaluation of SSc.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205

    CAS  PubMed  Google Scholar 

  2. Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore) 81:154–167

    Article  Google Scholar 

  3. Simeon CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M et al (2003) Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 42:71–75

    Article  CAS  Google Scholar 

  4. Altman RD, Medsger TA Jr, Bloch DA, Michel BA (1991) Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 34:403–413

    Article  CAS  PubMed  Google Scholar 

  5. Sampaio-Barros PD, Bortoluzzo AB, Marangoni RG, Rocha LF, Del Rio AP, Samara AM et al (2012) Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients. J Rheumatol 39:1971–1978

    Article  PubMed  Google Scholar 

  6. Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69:1809–1815

    Article  PubMed  Google Scholar 

  7. Maricq HR, LeRoy EC (1973) Patterns of finger capillary abnormalities in connective tissue disease by “wide-field” microscopy. Arthritis Rheum 16:619–628

    Article  CAS  PubMed  Google Scholar 

  8. Lee P, Leung FY, Alderdice C, Armstrong SK (1983) Nailfold capillary microscopy in the connective tissue diseases: a semiquantitative assessment. J Rheumatol 10:930–938

    CAS  PubMed  Google Scholar 

  9. Bredemeier M, Xavier RM, Capobianco KG, Restelli VG, Rohde LE, Pinotti AF et al (2004) Nailfold capillary microscopy can suggest pulmonary disease activity in systemic sclerosis. J Rheumatol 31:286–294

    PubMed  Google Scholar 

  10. Lee P (1993) Clinical aspects of systemic and localized sclerosis. Curr Opin Rheumatol 5:785–791

    Article  CAS  PubMed  Google Scholar 

  11. Smith V, Riccieri V, Pizzorni C, Decuman S, Deschepper E, Bonroy C et al (2013) Nailfold capillaroscopy for prediction of novel future severe organ involvement in systemic sclerosis. J Rheumatol 40:2023–2028

    Article  PubMed  Google Scholar 

  12. Sato LT, Kayser C, Andrade LE (2009) Nailfold capillaroscopy abnormalities correlate with cutaneous and visceral involvement in systemic sclerosis patients. Acta Reumatol Port 34:219–227

    PubMed  Google Scholar 

  13. Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C et al (2011) Survival in scleroderma: results from the population-based South Australian Register. Intern Med J 41:381–390

    Article  CAS  PubMed  Google Scholar 

  14. Kayser C, Sekiyama JY, Prospero LC, Camargo CZ, Andrade LE (2013) Nailfold capillaroscopy abnormalities as predictors of mortality in patients with systemic sclerosis. Clin Exp Rheumatol 31(Suppl 76):103–108

    PubMed  Google Scholar 

  15. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590

    Article  Google Scholar 

  16. LeRoy EC, Medsger TA (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28:1573–1576

    CAS  PubMed  Google Scholar 

  17. Brennan P, Silman A, Black C, Bernstein R, Coppock J, Maddison P et al (1992) Reliability of skin involvement measures in scleroderma. The UK Scleroderma Study Group. Br J Rheumatol 31:457–460

    Article  CAS  PubMed  Google Scholar 

  18. Bredemeier M, Chies JA, Wieck A, Capobianco KG, Pitrez EH, Rohde LE et al (2008) TCRBV20S1 and TCRBV3S1 gene segment polymorphisms in systemic sclerosis. J Rheumatol 35:1058–1063

    CAS  PubMed  Google Scholar 

  19. Andrade LE, Gabriel Júnior A, Assad RL, Ferrari AJ, Atra E (1990) Panoramic nailfold capillaroscopy: a new reading method and normal range. Semin Arthritis Rheum 20:21–31

    Article  CAS  PubMed  Google Scholar 

  20. SIM/NIS/DGTI/SES/RS. Sistema de Informações sobre Mortalidade do Núcleo de Informações em Saúde/RS.

  21. Pavan TR. Capilaroscopia periungueal em pacientes com esclerose sistêmica como preditor de mortalidade em um estudo de coorte [Dissertation]. Porto Alegre, Rio Grande do Sul, Brazil, Universidade Federal do Rio Grande do Sul, 2015. Submitted to LUME/UFRGS. http://www.lume.ufrgs.br/handle/10183/1

  22. Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al (2017) Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 1–9. doi:https://doi.org/10.1136/annrheumdis-2017-211448

  23. Hax V, Bredemeier M, Moro ALD, Pavan TR, Vieira MV, Pitrez EH et al (2017) Clinical algorithms for the diagnosis and prognosis of interstitial lung disease in systemic sclerosis. Semin Arthritis Rheum. https://doi.org/10.1016/j.semarthrit.2017.03.01923.

  24. Mazzotti NG, Bredemeier M, Brenol CV, Xavier RM, Cestari TF (2014) Assessment of nailfold capillaroscopy in systemic sclerosis by different optical magnification methods. Clin Exp Dermatol 39:135–141

    Article  CAS  PubMed  Google Scholar 

  25. Sekiyama JY, Camargo CZ, Andrade LE, Kayser C (2013) Reliability of widefield nailfold capillaroscopy and videocapillaroscopy in the assessment of patients with Raynaud’s phenomenon. Arthritis Care Res 65:1853–1861

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Vanessa Hax.

Ethics declarations

All patients signed an informed consent before entering the study. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation and with the Helsinki Declaration of 1975, as revised in 1983.

Conflicts of interest

MB and KGC are professionally involved in performing nailfold capillaroscopy. Other authors declare no conflict of interest.

Electronic supplementary material

ESM 1

(DOCX 45 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pavan, T.R., Bredemeier, M., Hax, V. et al. Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis. Clin Rheumatol 37, 475–481 (2018). https://doi.org/10.1007/s10067-017-3869-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-017-3869-1

Keywords

Navigation