The Co-existence of Rheumatoid Arthritis and Granulomatosis With Polyangiitis: Two Cases and Review of the Literature

Abstract

Granulomatosis with polyangiitis (GPA) and rheumatoid arthritis (RA) have shared features including vasculitis, ocular inflammation, interstitial lung disease, and arthritis but existing evidence indicates they are distinct conditions. Interestingly, the co-existence of GPA and RA has been described in the literature. Herein, we report two cases of GPA developing in patients with underlying RA and examine the relationship between the two conditions. Two cases of GPA that developed in patients with preexisting RA are described in detail. Additionally, PubMed was searched for articles in English showing an association of RA and GPA using keywords “rheumatoid arthritis, and vasculitis, and Wegener’s, and ANCA, and granulomatosis polyangiitis.” In addition to our two cases of RA and GPA overlap, 14 reports were identified in PubMed library from 1970 to 2020. Most of the cases were females (14/16, 88%), and had RA as the initial diagnosis (15/16, 94%). The mean age of RA diagnosis was 45.5 years, the mean age of GPA diagnosis was 52 years and the mean interval between both diagnoses was 101 months. Cyclophosphamide and steroid therapy were used in most of the cases. There are numerous reports of GPA and RA overlap in the literature. GPA should be considered in the differential diagnosis when vasculitis develops in patients with RA.

Meta‐analysis confirmed genetic susceptibility conferred by multiple risk variants from CTLA4 and SERPINA1 in granulomatosis with polyangiitis

“…9 Recent studies on GPA revealed co-existence of GPA and rheumatoid arthritis (RA) due to the increased frequency of 620 W functional variant of PTPN22. 10 Currently 4 genome-wide association studies (GWAS) have been reported on AAV where 3/4 studies provided substantial evidence for the association of HLA DP with GPA, and non-HLA susceptibility genes including SERPINA1, PRTN3 and PTPN22 with AAV. 11 The other 3 GWAS performed by Lyons et al, included 1683 GPA patients and 6858 controls and found association of SERPINA1, PRTN3, ARHGAP18, MOSPD2 genes with GPA 3 ; Xie et al reported SEMA6A as significant associated with GPA when compared with 987 GPA patients and 2731 controls, 12 and Merkel et al compared 1556 GPA patients and 4723 controls and identified SERPINA1 and PRTN3 as susceptibility genes.…”

“…Recent studies on GPA revealed co‐existence of GPA and rheumatoid arthritis (RA) due to the increased frequency of 620 W functional variant of PTPN22 10 . Currently 4 genome‐wide association studies (GWAS) have been reported on AAV where 3/4 studies provided substantial evidence for the association of HLA DP with GPA, and non‐HLA susceptibility genes including SERPINA1 , PRTN3 and PTPN22 with AAV 11 .…”