Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes

Rheumatology (Oxford). 2015 Mar;54(3):424-32. doi: 10.1093/rheumatology/keu336. Epub 2014 Sep 3.

Abstract

Objective: The aim of this study was to describe the presentation and outcomes of patients with granulomatosis with polyangiitis (GPA) presenting with CNS involvement.

Methods: Patients were included in this nationwide retrospective study if they had GPA according to ACR criteria and/or the European Medicines Agency algorithm and CNS involvement.

Results: Thirty-five patients were included in the study. CNS involvement was observed in 51% of patients at GPA diagnosis. Headache (66%) was the main symptom, followed by sensory (43%) and motor impairment (31%). CNS involvement was characterized by pachymeningitis in 20, cerebral ischaemic lesions in 15 and haemorrhagic lesions in 2, with hypophyseal involvement in 2 patients. According to the clinical-radiological presentation, we distinguished granulomatous (G-CNS) and vasculitic (V-CNS) phenotypes. G-CNS patients more frequently had headaches, while V-CNS patients more frequently had motor impairment and renal involvement. Induction therapy produced clinical responses in 86% of patients. Baseline modified Rankin scale was higher for V-CNS than G-CNS patients (3 vs 2, P = 0.002). Initial spinal cord pachymeningitis was significantly associated with the need for a new induction regimen for relapsing/refractory disease (P = 0.01). Long-term neurological sequelae were noted in 51% of patients, including 35% with G-CNS and 69% with V-CNS (P = 0.08). Neurological sequelae were mainly noted in cases of spinal cord pachymeningitis (100%) and ischaemic or haemorrhagic lesions (73%).

Conclusion: The clinical-radiological phenotype distinguished different long-term outcomes in patients with GPA and CNS involvement. Long-term neurological sequelae persisted in half of patients, mainly those with spinal cord pachymeningitis and vasculitic lesions.

Keywords: ANCA; central nervous system; cerebral vasculitis; granulomatosis with polyangiitis; pachymeningitis.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Brain Ischemia / diagnosis
  • Brain Ischemia / diagnostic imaging
  • Brain Ischemia / drug therapy
  • Central Nervous System Diseases / diagnosis*
  • Central Nervous System Diseases / diagnostic imaging*
  • Central Nervous System Diseases / drug therapy
  • Child
  • Child, Preschool
  • Female
  • France
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / epidemiology
  • Headache / diagnosis
  • Headache / diagnostic imaging
  • Headache / drug therapy
  • Humans
  • Male
  • Meningitis / diagnosis
  • Meningitis / diagnostic imaging
  • Meningitis / drug therapy
  • Middle Aged
  • Phenotype*
  • Prevalence
  • Prognosis
  • Radiography
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult

Substances

  • Adrenal Cortex Hormones