Vasculitis & polymyalgia rheumatica

Granulomatosis with polyangiitis with and without antineutrophil cytoplasmic antibodies: a case-control study

Rheumatology (Oxford)Published Case-control study

Authors' conclusion

Patients with ANCA-negative GPA have milder disease and a lower frequency of relapse than those with ANCA-positive GPA.

Study context

Population
Patients with granulomatosis with polyangiitis (GPA), single centre, 1 January 1996 to 31 December 2015
Comparison
ANCA-negative vs ANCA-positive GPA
  • ANCA-negative GPAn = 110
Outcome
Disease severity (BVAS/WG)
Methodological detailssingle-centre, sex and age matched, case-crossover study of seroconverters
  • Single-centre
  • Sex and age matched
  • Case-crossover study of seroconverters

Main finding

As reported in the abstract

ANCA-negative GPA

2

ANCA-positive GPA

6

P < 0.001

Observational data: associations, not effects.

Further findings

Relapse over 60 months

P = 0.009

OutcomeP value
Relapse over 60 monthsShorter time to event in ANCA-positive GPAP = 0.043

Organ manifestations

Mucous membranous/eye manifestations more frequent in ANCA-negative GPA
General symptoms, pulmonary and renal involvement more frequent in ANCA-positive GPA

Seroconversion to ANCA-positive during follow-up (case-crossover)

Seroconverters (n = 16): higher mean BVAS/WG at diagnosis
P < 0.001
Seroconverters: increased incidence of relapses after seroconversion
P = 0.004
Necrotizing granulomatous inflammation on biopsy: risk factor for seroconversion

Relapse risk factors

General manifestations, BMI > 30kg/m² and necrotizing granulomatous inflammation: more likely to relapse

Original publication

Granulomatosis with polyangiitis with and without antineutrophil cytoplasmic antibodies: a case-control study. Rheumatology (Oxford). Published 27 July 2024. doi:10.1093/rheumatology/keae379

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