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06-11-2021 | ACR 2021 | Conference coverage | News

Observational data show patterns of neurologic, ocular manifestations in ANCA-associated vasculitis

Author: Laura Cowen


medwireNews: Neurologic and ocular manifestations are common among people with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) but vary by disease type, show data from the Vasculitis Clinical Research Consortium reported at the ACR Convergence 2021 virtual meeting.

The findings were presented as posters outlining two separate analyses, the first of which looked at neurologic involvement in 1368 patients with AAV who took part in the multicenter Vasculitis Clinical Research Consortium observational studies between 2006 and 2021.

Rula Hajj-Ali (Cleveland Clinic, Ohio, USA) reported that, overall, 34% of participants had neurologic involvement. The prevalence was 66.7% in the 326 people with eosinophilic granulomatosis with polyangiitis (EGPA), 24.9% in the 869 with granulomatosis with polyangiitis (GPA), and 21.8% in the 170 participants with microscopic polyangiitis (MPA).

The peripheral nervous system (PNS) was more commonly affected than the central nervous system (CNS), at rates of 27.3% and 3.4%, respectively, but this varied by AAV type.

In people with GPA, 69.0% of neurologic manifestations involved the PNS, 17.1% involved the CNS, and 13.9% were unclassified. PNS involvement was more heavily weighted in people with MPA, accounting for 89.2% of all neurologic manifestations, while CNS involvement accounted for 2.7% with 8.1% of cases unclassified.

For EGPA, the corresponding proportions were 88.5%, 4.1%, and 5.1% with an additional 2.3% having both PNS and CNS involvement.

When looking at other organ involvement, Hajj-Ali and team found that skin and kidney manifestations were associated with neurologic involvement in both GPA and EGPA, along with musculoskeletal and venous thrombosis manifestations in GPA only. In MPA, constitutional and musculoskeletal symptoms were more common in people with versus without neurologic involvement.

Hajj-Ali concluded that the data “should raise the awareness of this common manifestation of vasculitis,” adding that “[t]he relationships of neurologic involvement with other specific manifestations of disease warrants additional study.”

In the second poster, Mats Junek (McMaster University, Hamilton, Ontario, Canada) reported the rates of ocular manifestations among 1389 Vasculitis Clinical Research Consortium study participants with AAV.

Junek and team found that ocular involvement at diagnosis was most common in people with GPA, occurring in 30.3% of 852 participants. The rates were much lower in the 165 people with MPA and the 372 with EGPA, at 4.2% and 3.5%, respectively.

The incidence was lower still during a median follow-up of 4.0 years, occurring in a corresponding 6.6%, 0.6%, and 0.5%.

At diagnosis, the most common ocular manifestation was conjunctivitis/episcleritis in people with GPA and MPA, accounting for 43.0% and 42.8% of all ocular involvement in these groups, respectively. In EGPA, the most common ocular manifestation was retinal vasculitis, occurring in 38.5% of cases.

The most common ocular complication during follow-up was cataracts, with incidence rates of 13.1%, 9.1%, and 15.3% among the people with GPA, MPA, and EGPA, respectively.

Visual impairment, diplopia, or blindness were most common in people with GPA, affecting 3.6% compared with 1.8% of those with MPA and just 0.3% of those with EGPA.

Junek concluded: “These data are informative for clinicians caring for patients with AAV and investigators studying this spectrum of vasculitis.”

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ACR Convergence 2021; 3–9 November  (abstract 0417)
ACR Convergence 2021; 3–9 November (abstract 0426)