Skip to main content
Log in

Disease activity score for still’s disease

  • ORIGINAL ARTICLE
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Objective

To develop the Still’s Disease Activity Score (SDAS).

Methods

We used data from the prospective adult-onset Still’s disease cohort study and evaluated the disease activity. An expert group selected the most frequent, reproducible, and objective variables significantly modified in statistical analysis when comparing patients in the active group and in the remission group. These criteria were weighted to design the Still’s Disease Activity Score (SDAS). The Delphi method was used to appreciate the level of disease activity. Total SDAS was calculated for each patient and compared to final consensus experts.

Results

At the diagnosis, all patients had an active disease (n = 80), while 48 patients were in remission at 6 months. The SDAS criteria were weighted as follows: fever ≥ 38.5 °C (1 point), rash (1 point), joint involvement (arthralgia: 1 point, swollen joints count “SJC”: 1–3 SJC: 2 points, ≥ 4 SJC: 3 points), physician global assessment VAS ≥ 5/10 or a raise in physician VAS ≥ 2/10 (3 points), patient VAS ≥ 5 or a raise in patient VAS ≥ 2/10 (1 point), and CRP (> 10 mg/l: 1 point, ≥ 100 mg/l: 2 points). At 6 months, the consensus was achieved for 76 (95%) patients with 40 in remission (0–1 point), 8 in low disease activity (2–3 points), 16 in moderate disease activity (4–7 points), and 12 in severe disease activity (≥ 8 points).

Conclusion

The Still’s Disease Activity Score is a valid and sensitive assessment of the disease activity and the therapeutic response in Still’s disease, despite its heterogeneous manifestations and patterns with systemic and articular forms.

Key Points

• The Still’s Disease Activity Score (SDAS) is a good simple tool to assess the activity of the disease in a stable state for a week.

• The SDAS is developed specifically for Still’s disease without the need for an application or a calculator to calculate SDAS in routine clinical practice.

• SDAS is a composite score classifying the disease activity in remission, low disease activity, moderate disease activity, and severe disease activity despite its heterogeneous patterns (systemic and articular forms).

• The SDAS is a valid, reliable, and sensitive score and can be useful to guide the therapeutic strategy in clinical practice and in research.

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

Access this article

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

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. Ruscitti P et al (2024) Recent advances and evolving concepts in Still’s disease. Nat Rev Rheumatol 20:116–132

    Article  PubMed  Google Scholar 

  2. Bindoli S et al.(2023) Efficacy and safety of therapeutic interventions for the treatment of Still’s disease: a systematic review and meta-analysis informing the EULAR/PReS recommendations for the diagnosis and management of systemic juvenile idiopathic arthritis and adult-onset Still’s disease. Arthritis Rheumatol 75 (Suppl. 9), Abstr. 2569

  3. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–430

    CAS  PubMed  Google Scholar 

  4. Fautrel B, Zing E, Golmard JL, LeMoel G, Bissery A, Rioux C et al (2002) Proposal for a newset of classification criteria for adult-onset still disease. Med (Baltimore) 81:194–200

    Article  Google Scholar 

  5. Daghor-Abbaci et al (2023) Proposal of a new diagnostic algorithm for adult onset Still’s disease. Clin Rheumatol 42:1125–1135

    Article  PubMed  Google Scholar 

  6. Gerfaud-Valentin M, Jamilloux I, Iwaz J, Sève P (2014) Adult onset Still’s disease. Autoimmun Rev 13:708–712

    Article  CAS  PubMed  Google Scholar 

  7. Colina M, Zucchini W, Ciancio G, Orzincolo C, Trotta F, Govoni M (2011) The evolution of adult-onset Still’s disease: an observational and comparative study in a cohort of 76 Italian patients. Semin Arthritis Rheum 41:279–285

    Article  PubMed  Google Scholar 

  8. Abbaci-Daghor K, Berrah A (2019) Severe destructive arthritis in adult onset Still’s disease. Rheumatol Adv Pract 3(1):rky052. https://doi.org/10.1093/rap/rky052

  9. Munagala R, Misra R, Agarwal V, Lawrence A, Aggarwal A (2012) Adult onset Still’s disease: experience from a tertiary care rheumatology unit. Int J Rheum Dis 15(6):136–141

    Google Scholar 

  10. Hot A, Toh ML, Coppéré B, Perard L, Girard-Madoux MH, Mausservey C et al (2010) Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features and long-term outcome: a case-control study of 8 patients. Medicine (Baltimore) 89:37–46

    Article  PubMed  Google Scholar 

  11. Abbaci Daghor K, Otmane A, Makrelouf M et al (2021) Prognostic factors in adult onset Still’s disease. Arch Rheum & Arthritis Res. 1(5) ARAR.MS.ID.000523. https://doi.org/10.33552/ARAR.2021.01.000523

  12. -Bindoli S et al. Adult‑onset Still’s disease (AOSD): advances in understanding pathophysiology, genetics and emerging treatment options. Drugs. https://doi.org/10.1007/s40265-024-01993

  13. Gabay C, Fautrel B, Rech B, Spertini F, Feist E, Kötter I (2018) Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of Tadekinig alfa (IL-18BP) in adult-onset Still’s disease. Ann Rheum Dis 77:840–847

    CAS  PubMed  Google Scholar 

  14. Ruscitti P, Cipriani P, Masedu F, Lacono D, Ciccia F, Liakouli V (2016) Adult onset Still’s disease : evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three center. Medicine 14:194

    PubMed  PubMed Central  Google Scholar 

  15. Rau M, Schiller M, Krienke S, Heyder P, Lorenz H, Blank N (2010) Clinical manifestations but not cytokine profiles differentiate adult-onset Still’s disease and sepsis. J Rheumatol 37(11):2369–2376

    Article  CAS  PubMed  Google Scholar 

  16. Tibaldi J, Pistzerio A, Aldera E (2020) Development and initial validation of a composite DAS for systemic JIA. Rheumatology 59:3505–3514. https://doi.org/10.1093/rheumatology/keaa240

    Article  CAS  PubMed  Google Scholar 

  17. Macovei LA et al (2022) Adult-onset Still’s disease—a complex disease, a challenging treatment. Int J Mol Sci 23:12810. https://doi.org/10.3390/ijms232112810

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M et al (1991) Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70:118–136

    Article  CAS  PubMed  Google Scholar 

  19. Feist E, Mitrovic S, Fautrel B (2018) Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol 14:603–618. https://doi.org/10.1038/s41584-018-0081

    Article  PubMed  PubMed Central  Google Scholar 

  20. Daghor-Abbaci K, Ait Hamadouche N, Otmani F et al (2022) Validation of the neutrophil-to-lymphocyte ratio as a new simple biomarker of adult onset Still’s disease. Medicine 101:32

    Article  Google Scholar 

  21. Leavis HL et al (2023) Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts. Rheumatology 00:1–8. https://doi.org/10.1093/rheumatology/kead461

    Article  Google Scholar 

  22. Schoels et al (2017) Evaluation of newly proposed remission cut-points for disease activity score in 28 joints (DAS28) in rheumatoid arthritis patients upon IL-6 pathway inhibition. Arthritis Res Ther 19:155

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Maranini B, Ciancio G, Govoni M (2021) Adult-onset Still’s disease: novel biomarkers of specific subsets, disease activity, and relapsing forms. Int J Mol Sci 22:13320. https://doi.org/10.3390/ijms222413320

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Puéchal X, DeBandt M, Berthelot J-M, Breban M, Dubost J-J, Fain O et al (2011) Toci-lizumab in refractory adult Still’s disease. Arthritis Care Res 63:155–159

    Article  Google Scholar 

  25. Vitale A, Cavalli G, Colafrancesco S, Priori R, Valesini G, Argolini LM (2019) Long-term retention rate of anakinra in adult onset Still’s disease and predictive factors for treatment response. Front Pharmacol 10:296

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Lequerré T, Quartier P, Rosellini D, Alaoui F, De Bandt M, Mejjad O et al (2008) Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. Ann Rheum Dis 67:302–308

    Article  PubMed  Google Scholar 

  27. Nordström D, Knight A, Luukkainen R, van Vollenhoven R, Rantalaiho V, Kaja-lainen A et al (2012) Beneficial effect of interleukin 1 inhibition with anakinra inadult-onset Still’s disease. An open, randomized, multicenter study. J Rheumatol 39:2008–11

    Article  PubMed  Google Scholar 

  28. Piram M, Koné-Paut I, Lachmann HJ et al (2014) Validation of the auto-inflammatory diseases activity index (AIDAI) for hereditary recurrent fever syndromes. Ann Rheum Dis 73:2168–2173

    Article  PubMed  Google Scholar 

  29. Wallace CA, Giannini EH, Huang B et al (2011) American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 63:92936

    Article  Google Scholar 

  30. Kalyoncu U, Kasifoglu T, Omma A et al (2023) Derivation and validation of adult Still Activity Score (SAS). Joint Bone Spine 90:105499

    Article  PubMed  Google Scholar 

  31. Kalyoncu U, Solwaz D, Emmungil H, Yazici A, Kasifoglu T, Kimyon G et al (2016) Response rate of initial conventional treatments, disease course and related factors of patients with adult onset Still disease: data from a large multicenter cohort. J Autoimmun 16:S0896-8411

    Google Scholar 

  32. Vercruysse F, Barnetche T, Lazaro E, Shipley E, Lifermann F, Balageas A (2019) Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy. Arthritis Res Ther 21:53

    Article  PubMed  PubMed Central  Google Scholar 

  33. Cavalli G, Tomelleri A, De Luca G, Campochiaro C, Dinarello C, Baldissera E, Dagna L (2019) Efficacy of canakinumab as first-line biologic agent in adult-onset Still’s disease. Arthritis Res Ther 21:54

    Article  PubMed  PubMed Central  Google Scholar 

  34. Tomaras S, Goetzke CC, Kallinich T, Feist E (2021) Adult-onset Still’s disease: clinical aspects and therapeutic approach. J Clin Med 10:733. https://doi.org/10.3390/jcm10040733

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

We thank all internists, rheumatologists, and all physicians who collaborated in the realization of this study.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Karima Daghor-Abbaci.

Ethics declarations

Disclosures

None.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Daghor-Abbaci, K., Laraba, N., Dahou-Makhloufi, C. et al. Disease activity score for still’s disease. Clin Rheumatol (2024). https://doi.org/10.1007/s10067-024-07127-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s10067-024-07127-8

Keywords

Navigation