Background/Purpose: The frequency of CNS involvement in granulomatosis with polyangiitis (GPA) is uncommon (4-18%). Among them, pachymeningitis (PM) has been reported in 0.6-8%. Improvement of these complications has been reported with conventional therapy (CS + CYC), and recently with rituximab, but many patients have recurrences. Data on related factors, outcome and quality of life is scarce. We preliminarily describe CNS involvement as investigated with MRI, PM presence in symptomatic and asymptomatic patients with GPA, and its implications for quality of life.

Methods: Forty consecutive GPA patients fullfiling the ACR criteria and/or the 2012 Chapel Hill Consensus Conference definition were studied. After informed consent, gadolinium-enhanced brain MRI in search of PM, irrespective of CNS symptoms, was done. The presence of diffuse or focal meningeal enhancement was necessary for PM diagnosis. Main subgroups for analysis were formed according to two main criteria: PM on MRI, and CNS symptoms presence or absence. They were compared regarding clinical, serological, neuroimaging and treatment-related findings. Functional status was assessed with the Short Form 36 (SF-36) health survey and Karnofsky Performance Scale Index. Univariate analysis was used to establish proportions with means ± SD and medians calculated; bivariate analysis was used to compare groups, Student’s t test for continuous variables and X² test with Yates correction for categorical variables. Significance was established when p<0.05.

Results: Ten patients had generalised disease, the rest localised. Age range was 8-72 years old. Main differences regarding PM and CNS symptomatology as related to MRI findings are shown in the table.

* Defined as that compromising life or vital organ function; ♯ mean values ± SD; § median values (intervals)

Conclusion: We found PM in 19/40 cases (47.5%) and irrespective of symptoms it was present in 1/3 of cases. Its cause (active disease or chronic inflammation, possibly due to fibrosis) is difficult to discern. Therefore, optimal therapeutic measures are unknown. Although the majority of patients had localised -predominant upper airway and ocular- disease, SGS had developed in a minority of patients with PM. This may suggest that operative mechanisms differ in both locations, and although scarring has been considered to play a role in PM in GPA, it might not be exclusive. Certain features relate straightforwardly to better health status, but in those asymptomatic PM patients, we found lower SF-36 scores, implying impaired quality of life. That PM occurs frequently in localised disease does not mean less severity than generalised malady. Although limited by the small sample size, CNS involvement detection in GPA, even in absence of symptoms, could be useful as impairment in quality of life may occur. Further research on this issue is warranted, with proper placement of early neuroimaging.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mri-findings-in-granulomatosis-with-polyangiitis-pachymeningitis-and-implications-in-quality-of-life/