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Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry.

Michele Iudici1,2, Christian Pagnoux1, Delphine S Courvoisier2

  • 1National Referral Center for Rare Systemic Autoimmune Diseases, Université de Paris, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.

Rheumatology (Oxford, England)
|September 20, 2021
PubMed
Summary

Localized granulomatosis with polyangiitis (L-GPA) patients are younger and have distinct features from systemic GPA (S-GPA). While relapse risks are similar, L-GPA shows higher survival rates and more ENT involvement, with some progressing to S-GPA.

Keywords:
ANCA-associated vasculitisgranulomatosis with polyangiitisvasculitis

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Area of Science:

  • Rheumatology
  • Immunology
  • Vasculitis Research

Background:

  • Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease affecting small blood vessels.
  • GPA presents in localized (L-GPA) and systemic (S-GPA) forms, with distinct clinical characteristics and progression patterns.
  • Understanding the differences between L-GPA and S-GPA is crucial for effective patient management and treatment strategies.

Purpose of the Study:

  • To compare the diagnostic features and long-term evolution of localized GPA (L-GPA) versus systemic GPA (S-GPA).
  • To analyze treatment responses and outcomes, including relapse rates and survival, in L-GPA and S-GPA patients.
  • To identify the rate and characteristics of L-GPA progression to S-GPA.

Main Methods:

  • Utilized the French Vasculitis Study Group Registry, applying EULAR definitions for L-GPA and S-GPA.
  • Analyzed and compared patient characteristics at diagnosis, including demographics and clinical manifestations.
  • Compared long-term outcomes such as relapse-free survival, relapse rates, refractory disease, and overall survival between L-GPA and S-GPA cohorts.

Main Results:

  • L-GPA patients (10% of 795) were younger, more frequently presented with saddle nose or subglottic stenosis, and were less often PR3-ANCA-positive than S-GPA patients.
  • Induction therapy for L-GPA less commonly included cyclophosphamide (CYC) but more often involved methotrexate (MTX) and glucocorticoids; 64% of MTX-treated L-GPA patients progressed.
  • L-GPA and S-GPA had comparable relapse-free survival and refractory disease rates, but L-GPA exhibited more frequent ENT/lung relapses and higher overall survival. 22.8% of L-GPA progressed to S-GPA over 3.5 years.

Conclusions:

  • L-GPA and S-GPA share similar relapse risks but differ in relapse patterns and overall survival, with L-GPA patients having better survival rates.
  • Approximately one-quarter of L-GPA patients may develop S-GPA over time, typically without severe end-stage organ damage.
  • L-GPA patients experienced more ENT-related damage, highlighting the need for targeted management of this manifestation.