GPA Relapses Tend to Affect the Same Organs as at Diagnosis

TOPLINE:

Relapses in patients with granulomatosis with polyangiitis (GPA) largely mirrored the initial disease pattern, most often affecting the ENT, lungs, or kidneys, with serious features recurring. Involvement of new organs was uncommon and usually occurred alongside previously affected organs.

METHODOLOGY:

Researchers analyzed data from a French national registry to assess whether organs affected at GPA diagnosis predicted involvement of the same organ(s) at the first relapse and to identify factors linked to new organ involvement.
They included 795 patients newly diagnosed with GPA between May 1983 and April 2018; the mean age was 53.2 years, and 56% were male.
Clinical features were grouped by organs and organ systems — the ENT, lungs, kidneys, nervous system, skin or mucosa, cardiovascular system, and gastrointestinal system — and by general systemic signs and symptoms.
The main outcome was concordance between the presenting features at baseline and those at the first relapse. Relapse was defined as the reappearance or new onset of GPA symptoms due to active vasculitis.
Patients were followed up for a median of 3.6 years.

TAKEAWAY:

Overall, 50% of patients experienced at least one relapse. The first relapse occurred after a median of 18 months and most often involved the ENT, kidneys, lungs, and nervous system.
Relapses tended to occur in the same organs affected at diagnosis, with the strongest concordance observed for the eyes (odds ratio [OR], 6.69), kidneys (OR, 3.58), lungs (OR, 3.35), nervous system (OR, 2.9), and skin or mucosa (OR, 4.53; P < .05 for all after false discovery rate correction). Additionally, serious features such as scleritis, alveolar hemorrhage, and worsening kidney function recurred.
First relapses were limited to initial organs in 56.1% of patients who experienced a relapse and involved new organs in 43.9%, usually alongside previously affected organs. Isolated involvement of new organs during relapses was uncommon.
The presence of systemic symptoms and the involvement of the ENT and lungs at diagnosis were independently associated with a lower risk for new organ disease at the first relapse (P < .0001 for all).

IN PRACTICE:

“Patients should be informed of the increased risk of recurrence in the organs initially involved at diagnosis, while also remaining vigilant for warning signs of new organ involvement,” the authors of the study wrote.

SOURCE:

The study was led by Michele Iudici, MD, PhD, MPH, Geneva University Hospitals and University of Geneva, Geneva, Switzerland. It was published online on December 31, 2025, in RMD Open.

LIMITATIONS:

Patients with more severe disease may have been overrepresented because the recruitment occurred at tertiary referral centers. Not all patients met the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria due to missing data. Changes in treatment across decades may have influenced relapse patterns and limited generalizability over time.

DISCLOSURES:

The authors did not receive any specific grant or funding for this study. The authors acknowledged receiving support in part from the Directorate of Health Care Supply of the French Ministry of Health. One author reported receiving consulting fees, speaking fees, honoraria, and funding for congress registration, travel, and accommodation from Boehringer Ingelheim, Sanofi, AstraZeneca, and GlaxoSmithKline. Two other authors reported having similar financial ties with several pharmaceutical and healthcare companies. All authors reported being co-investigators in academic studies for which rituximab was provided by Roche Pharma.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.