Background/Purpose
Granulomatosis with polyangiitis (Wegener’s) (GPA) is an ANCA-associated vasculitis (AAV) predominantly affecting small-sized vessels, involving mainly the upper and lower respiratory tracts and kidneys. Cytoplasmic labeling by ANCA directed against proteinase-3 (PR3) is associated with >80% of the systemic GPA forms. GPA mainly affects white Europeans (Caucasians/Hispanic), with mean age at diagnosis of 52 years. Recent data revealed an AAV genetic component in European patients. Rarely, GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans.
Methods Among 915 GPA patients included in the FVSG database, geographic origin and ethnicity were known for 761. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans were analyzed.
Results
Among the 761 patients with available geographic origin and ethnicity, 690 (91%) were white Europeans and 22 (2.9%) were sub-Saharans (n=8) or Afro-Caribbeans (French West Indies, n=14). While sex ratios were similar for the 2 populations, sub-Saharans and Afro-Caribbeans, compared to white Europeans, were younger at GPA diagnosis (41.7±14.7 vs 52.1±16.0; P<0.001), had more frequent chondritis (19 vs 6%; P<0.05), central nervous system involvement (18 vs 4.3%; P<0.05), severe granulomatous manifestations [eg retroorbital tumor (9.1 vs 0.9%; P<0.05), subglottic stenosis (19 vs 2.2; P<0.01) and pachymeningitis (13.6 vs 1.6%; P<0.01)]. In contrast, sub-Saharans and Afro-Caribbeans had less frequent fever (20 vs 51%; P<0.05), weight loss (25 vs 51%; P<0.05), kidney involvement (38 vs 59%; P=0.07), cardiovascular involvement (0 vs 15%; P=0.06) and peripheral neuropathy (4.5 vs 22%; P=0.06), and their serum creatinine levels (70 vs. 175 µmol/L; P<0.01) and BVAS (12.3±7.2 vs 19.6±9.1; P<0.01) were significantly lower. Finally, relapse-free survival tended to be shorter for sub-Saharans and Afro-Caribbeans (median survival 44.8 vs 59.8 months), without reaching the significance [HR 1.75 (0.92–4.80); P=0.08]. Overall survival was similar for the 2 populations.
Conclusion
Our findings indicated different GPA clinical presentations in white Europeans vs sub-Saharans and Afro-Caribbeans, with blacks having more frequent severe granulomatous manifestations and less frequent constitutional symptoms, renal involvement and peripheral neuropathy. Prognoses did not differ according to geographic origin.
Disclosure:
B. Terrier,
None;
C. Deligny,
None;
X. Puéchal,
None;
P. Godmer,
None;
P. Charles,
None;
G. Hayem,
None;
B. Dunogué,
None;
P. Cohen,
None;
S. Arfi,
None;
L. Mouthon,
None;
L. Guillevin for the French Vasculitis Study Group,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-wegeners-according-to-geographic-origin-and-ethnicity-clinical-biological-presentation-and-outcome/