Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis

Abstract

Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature

“…A more recent study confirmed the above observations, reporting a 36% incidence of HP in patients with GPA and CNS involvement (9 of 25 patients). Headache was the dominant initial symptom (8 of 9, 88.9%).…”

“…In conclusion, HP is a rare complication of GPA that is increasingly being reported lately. It is proposed that it may constitute a different clinical spectrum of GPA, especially in the granulomatous, localized p‐ANCA (MPO‐ANCA) positive type . In our case, the patient developed a chronic, atypical headache due to HP, as the first and dominant symptom of GPA, which is the norm for this specific manifestation.…”

“…7 Following similar observations, the diagnostic criteria for GPA have been revised to include a limited form of GPA. 2,15 A more recent study 15 confirmed the above observations, reporting a 36% incidence of HP in patients with GPA and CNS involvement (9 of 25 patients). Headache was the dominant initial symptom (8 of 9, 88.9%).…”

“…Additionally, a similar incidence of HP (44%) was reported in a series of patients who developed such severe CNS complications that a hospitalization in a neurological department was eventually required . Following similar observations, the diagnostic criteria for GPA have been revised to include a limited form of GPA …”

“…[10][11][12][13] However, in the modern era of neuroimaging, thanks to the increased availability of MRI scans, reports are focusing more and more on granulomatous manifestations of GPA and the impact of their CNS sequelae, 14 especially on the increased frequency of patients with hypertrophic pachymeningitis (HP) (as high as 36% of patients with GPA and CNS involvement or even 44% of more severe cases). 7,15 In light of the above development, we report the case of a patient with headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of GPA and we review the recent literature.…”