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Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa

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Abstract

The 1990 American College of Rheumatology (ACR) criteria for the classification of polyarteritis nodosa (PAN) have many pitfalls and performed poorly when used for diagnostic purposes. Recently, a provisional seven-item diagnostic criteria for PAN was proposed. To validate the provisional seven-item diagnostic criteria for PAN in a cohort of PAN patients from a tertiary care centre in India. Clinical details of patients diagnosed as PAN as per the European Medicines Agency algorithm between 2005 and 2020 were collected retrospectively. Age and sex-matched anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) patients were included in the non-PAN group. Patients with a deficiency of adenosine deaminase 2 (DADA2) were included as a separate group. The sensitivity, specificity, positive and negative predictive values (PPV and NPV) for ACR criteria, the Ministry of Health, Labour and Welfare (MHLW) in Japan diagnostic criteria and the seven-item diagnostic criteria were calculated. Thirty-seven PAN, 14 DADA2 and 37 AAV patients were included in the analysis. The sensitivity, specificity, PPV and NPV of the seven-item criteria were 83.7%, 96.8%, 97.3% and 81.1% respectively. For the ACR criteria, sensitivity was 82.9% and specificity was 79.5%. The sensitivity, specificity for MHLW criteria were 77.3% and 90% respectively. The sensitivity and specificity of seven-item criteria for DADA2 patients were 58.8% and 88.2% respectively. There was very poor agreement between the ACR criteria and the seven-item and MHLW criteria and fair agreement between seven-item and MHLW criteria (κ = 0.279). The provisional seven-item criteria for PAN performed well with high specificity and PPV.

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Authors and Affiliations

  1. Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    GSRSNK Naidu, Chirag Rajkumar Kopp, Varun Dhir, Sanjay Jain & Aman Sharma

  2. Department of Internal Medicine, Indira Gandhi Medical College, Shimla, India

    Vikas Sharma

  3. Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Manphool Singhal

  4. Department of Clinical Immunology and Rheumatology, St. John’s Medical College, Bangalore, India

    Benzeeta Pinto

  5. Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Ritambhra Nada

  6. Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Ranjana W. Minz

Authors
  1. GSRSNK Naidu
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  2. Chirag Rajkumar Kopp
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  3. Vikas Sharma
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  4. Manphool Singhal
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  5. Benzeeta Pinto
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  6. Varun Dhir
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  7. Ritambhra Nada
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  8. Ranjana W. Minz
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  9. Sanjay Jain
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  10. Aman Sharma
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Contributions

GN and AS contributed to the conception and design of the study. GN, CRK, VS, MS, BP, VD, RN, RWM, SJ and AS contributed to the acquisition of clinical, radiological, histopathological and immunological data. GN and AS analysed and interpreted the data. GN and AS drafted the first manuscript and all the other authors have done a critical review of the content and approved the final version submitted. All authors agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Aman Sharma.

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None for all the authors.

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Study approved by Institute Ethics Committee.

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Waived by the Institute Ethics Committee.

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Naidu, G., Kopp, C.R., Sharma, V. et al. Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa. Rheumatol Int 41, 1651–1655 (2021). https://doi.org/10.1007/s00296-021-04867-7

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  • DOI: https://doi.org/10.1007/s00296-021-04867-7

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