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Proposal of a new diagnostic algorithm for adult-onset Still’s disease

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A LETTER TO THE EDITOR to this article was published on 20 February 2023

Abstract

Objective

This study was performed to develop a new diagnostic algorithm for adult-onset Still’s disease (AOSD).

Methods

We conducted a multicenter prospective nationwide case–control study in tertiary Internal Medicine, Rheumatology, and Infectious Diseases departments, to include successively patients with suspected AOSD based on the presence of two or more major criteria of Yamaguchi and/or Fautrel classifications. Patients were classified as AOSD or controls according to a predefined procedure. A receiving operating characteristic curve was used to determine the best cutoff value of the points-based score for disease classification. A diagnostic algorithm was developed to help the physician in the diagnostic approach.

Results

A total of 160 patients were included, 80 patients with AOSD and 60 controls with different diagnoses. Twenty patients with incomplete data were excluded. In the multivariate analysis, 6 items remained independently associated with AOSD diagnosis: typical rash (OR: 24.01, 3 points), fever ≥ 39 °C (OR: 17.34, 3 points), pharyngitis (OR: 10.23, 2 points), arthritis (OR: 9.01, 2 points), NLR ≥ 4 (OR: 11.10, 2 points), and glycosylated ferritin ≤ 20% (OR: 1.59, 1 point).

AOSD should be considered if the patient satisfies 7 points with a sensitivity of 92.5%, specificity of 93.3%, and accuracy of 92.8% (area under the curve (AUC): 0.97 [95% CI: 0.94–0.99]). The present points-based score was more accurate and sensitive than the Yamaguchi classification (78.8%, 92.5%, p = 0.01) and Fautrel classification (76.3%, 92.5%, p = 0.004). A typical rash associated with a points-based score ≥ 7 points leads to a very likely disease.

Conclusion

The proposed new algorithm could be a good diagnostic tool for adult-onset Still’s disease in clinical practice and research.

Key Points

• A diagnostic algorithm was performed to help the physician in the diagnostic approach of AOSD.

• The points-based score included in this algorithm had a high sensitivity and accuracy.

• This diagnostic algorithm can be useful in the clinical research.

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Data Availability

The data that support the findings of this study are available from the corresponding author [K.D.A], upon reasonable request.

Abbreviations

ANA:

Antinuclear antibodies

Anti-CCP:

Anti-cyclic citrullinated peptide

AOSD:

Adult-onset Still’s disease

AUC:

Area under the curve

CI:

Confidence interval

CRP:

C-reactive protein

GF:

Glycosylated ferritin

HIV:

Human immunodeficiency virus

NLR:

Neutrophil-to-lymphocyte ratio

NPV:

Negative predictive value

OR:

Odds ratio

PPV:

Positive predictive value

RHL:

Reactive hemophagocytic lymphohistiocytosis

RF:

Rheumatoid factor

ROC:

Receiver operating characteristic

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Acknowledgements

We wish to thank all internist, rheumatologist, infectious disease specialist, and all the physicians who collaborate in the realization of this study.

Author information

Authors and Affiliations

  1. Internal Medicine Department, University of Algiers 1, Faculty of Medical Sciences, Benyoucef Benkhedda, Bab El Oued University Hospital Center, 16000, Bab El Oued City, Algiers, Algeria

    Karima Daghor-Abbaci, Nazim Laraba & Abdelkrim Berrah

  2. Biochemistry, Hemotology and Genetics Laboratory of Research, Bab El Oued University Hospital Center, Bab El Oued City, Algiers, Algeria

    Karima Daghor-Abbaci, Mohamed Makrelouf & Amel Otmane

  3. Department of Epidemiology, Bab El Oued University Hospital Center, Bab El Oued City, Algiers, Algeria

    Nadia Ait Hamadouche

  4. Rheumatology Department, Bab El Oued University Hospital Center, Bab El Oued City, Algiers, Algeria

    Chafia Dahou Makhloufi & Farida Mechid

  5. Internal Medicine Department, Mustapha Bacha University Hospital Center, 1st May City, Algiers, Algeria

    Fifi Otmani

  6. Department of Epidemiology, Mustapha Bacha University Hospital Center, 1st May City, Algiers, Algeria

    Nourredine Smail

  7. Internal Medicine Department, Kouba University Hospital Center, Kouba City, Algiers, Algeria

    Malika Boucelma

  8. Infectious Diseases Department, El Hadi Flici University Hospital Center, Casbah City, Algiers, Algeria

    Fatma Zohra Aissat

  9. Rheumatology Department, Benimessous University Hospital Center, Algiers, Benimessous City, Algeria

    Salima Lefkir-Teffiani & Bilel Bengana

  10. Internal Medicine Department, Annaba University Hospital Center, Annaba City, Algeria

    Nadia Boukheris

  11. Internal Medicine Department, Birtraria University Hospital Center, El Biar City, Algiers, Algeria

    Amar Tebaibia

  12. Internal Medicine Department, Bouloughine University Hospital Center, Algiers, Bouloughine City, Algeria

    Baya Taharbouchet

  13. Internal Medicine Department, Benimessous University Hospital Center, Algiers, Benimessous City, Algeria

    Soraya Ayoub

  14. Internal Medicine Department, Laghouat University Hospital Center, Laghouat City, Algeria

    Brahim Benziane

  15. Internal Medicine Department, Zmirli University Hospital Center, El Harrache City, Algiers, Algeria

    Nadia Oumnia

  16. Rheumatology Department, Douera University Hospital Center, Blida City, Algeria

    Chafika Haouichet

  17. Rheumatology Department, Benaknoun University Hospital Center, Benaknoun City, Algiers, Algeria

    Fella Hanni

  18. Internal Medicine Department, Mostaghanem University Hospital Center, Mostaghanem City, Algeria

    Djennete Hakem

  19. Internal Medicine Department, Rouiba University Hospital Center, Rouiba City, Algiers, Algeria

    Nacera Benfenatki

Authors
  1. Karima Daghor-Abbaci
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  2. Nadia Ait Hamadouche
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  3. Chafia Dahou Makhloufi
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  4. Farida Mechid
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  5. Fifi Otmani
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  6. Mohamed Makrelouf
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  8. Nourredine Smail
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  10. Fatma Zohra Aissat
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  16. Soraya Ayoub
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  17. Brahim Benziane
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  18. Nadia Oumnia
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  19. Chafika Haouichet
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  20. Fella Hanni
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  21. Nazim Laraba
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  23. Nacera Benfenatki
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  24. Abdelkrim Berrah
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Corresponding author

Correspondence to Karima Daghor-Abbaci.

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Daghor-Abbaci, K., Ait Hamadouche, N., Makhloufi, C.D. et al. Proposal of a new diagnostic algorithm for adult-onset Still’s disease. Clin Rheumatol 42, 1125–1135 (2023). https://doi.org/10.1007/s10067-023-06509-8

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