Validation of the ACR EULAR Provisional 2017 Classification Criteria of Granulomatosis with Polyangiitis (GPA) Amongst Patients with ANCA Associated Vasculitis

Aman Sharma¹, Adarsh MB², Shankar Naidu³, Manish Rathi⁴, Benzeeta Pinto⁵, Varun Dhir⁶, Roshan Verma⁷, Kusum Sharma⁸, Ritambhra Nada⁹, Sanjay Jain⁵ and Ranjana Minz¹⁰, ¹Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ²Internal Medicine, PGIMER, Chandigarh, India, ³PGIMER, cHANDIGARH, India, ⁴Department of Nephrology,, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁵PGIMER, CHANDIGARH, India, ⁶Internal Medicine (Rheumatology Unit), Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁷PGIMER, Chandigarh, India, ⁸Department of Medical Microbiology,, PGIMER,, Chandigarh, India, ⁹Histopathology, Professor, Chandigarh, India, ¹⁰Department of Immunopathology,, PGIMER,, Chandigarh, India

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: classification criteria and vasculitis, Wegener's granulomatosis

Session Information

Date: Monday, November 6, 2017

Title: Vasculitis Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: The purpose of this study was to validate the recently proposed ACR EULAR 2017 classification criteria of GPA in a real life cohort of ANCA associated vasculitis (AAV).

Methods: The proposed ACR EULAR 2017 criteria of GPA were applied to patients diagnosed to have AAV according to European Medicines Agency (EMA) algorithm. The level of agreement between the two criteria was assessed using CohenÕs kappa, and positive and negative percent agreement (PPA and NPA).

Results: 187 patients with mean age of 41.5±15.0 years were included. Female: male ratio was 1:0.8. ANCA by IIF was done in all patients and was positive in 142(cANCA -97, pANCA -45) . PR3/MPO ELISA was available in 118 patients(PR3-71, MPO- 31). Using EMA algorithm EGPA was diagnosed in 6, GPA in 148 and MPA in 33. With ACR 1990 criteria, GPA was diagnosed in 118 patients while with ACR/EULAR 2017 criteria 115 were classified as GPA. ACR/EULAR 2017 had better agreement with EMA (kappa-0.35, PPA-91.3 and NPA-40.2) than ACR criteria (kappa 0.15, PPA -68.6 and NPA -45.8). The sensitivity and specificity of ACR/EULAR 2017 against EMA GPA was 70.9% and 74.3% respectively. Ten out of thirty three MPA patients were reclassified as GPA by ACR/EULAR 2017 criteria. Out of 43 patients who were GPA by EMA algorithm but not by ACR EULAR 2017 criteria, 37 had lung and renal involvement (DCVAS score 2), three had sinus and renal involvement (DCVAS score 3) and three had lung involvement with MPO positivity (DCVAS score 2). This resulted in low NPA of ACR EULAR 2017 criteria with EMA algorithm.

Conclusion: Within the limits of a retrospective design, the study showed fair agreement with high PPA but low NPA of ACR/EULAR 2017 criteria with EMA algorithm.

Table 1. Reclassification of various AAV patients (EMA and ACR 1990) by ACR EULAR 2017 provisional criteria of GPA

ANCA associated vasculitis	No. of patients (n=187, %,)	Reclassified as GPA by ACR/EULAR 2017 criteria
ACR 1990 GPA	118(63.1)	79
EMA GPA	148(79.1)	105
EMA MPA	33(17.6)	10
EMA EGPA	6(3.2)	0

Figure 1 – Showing different AAV according to various classification criteria

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Disclosure: A. Sharma, None; A. MB, None; S. Naidu, None; M. Rathi, None; B. Pinto, None; V. Dhir, None; R. Verma, None; K. Sharma, None; R. Nada, None; S. Jain, None; R. Minz, None.

To cite this abstract in AMA style:

Sharma A, MB A, Naidu S, Rathi M, Pinto B, Dhir V, Verma R, Sharma K, Nada R, Jain S, Minz R. Validation of the ACR EULAR Provisional 2017 Classification Criteria of Granulomatosis with Polyangiitis (GPA) Amongst Patients with ANCA Associated Vasculitis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/validation-of-the-acr-eular-provisional-2017-classification-criteria-of-granulomatosis-with-polyangiitis-gpa-amongst-patients-with-anca-associated-vasculitis/. Accessed .

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