title: Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review.
abstract: The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35.5 ± 20.4 years (median 48, range 8-71 years). Mean time ± SD between GPA first symptom and PM diagnosis was 59.8 ± 70.1 months (median 48, range 2-252 months). An important difference between children and adults was the median time elapsed between first GPA symptoms and PM diagnosis (range 2-4 months vs 5-252 months, respectively). Chronic headache was present in all, followed by intracranial hypertension (n = 5), single cranial nerve palsy and orbital mass (n = 4), seizures (n = 3), cavernous sinus syndrome and multiple cranial nerve palsies (n = 2), and meningism and cerebellar syndrome (n = 1 each). At time of PM diagnosis, mean BVAS/WG (Birmingham Vasculitis Activity Score for Wegener's granulomatosis) was 4 ± 2.4 and mean VDI of 2 ± 1.6, mostly due to ENT damage. Gadolinium-enhanced brain MRI showed dural thickening in 12 patients and leptomeningeal enhancement in one. All received a combination of glucocorticoids plus immunosuppressants, rituximab being used favourably in one refractory case. Improvement was observed in 12 patients. Chronic headache should lead to suspect PM. PM predominates in localised GPA. Children may present it earlier in the disease course than adults. Treatment is non-standardised and remains difficult.
authors.affiliation: Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502, Col. Sección XVI, Tlalpan, C.P, 14080, Mexico City, Mexico.
authors.forename: Violeta
authors.initials: V
authors.lastname: Higuera-Ortiz
authors.affiliation: Department of Radiology and Molecular Imaging, Instituto Nacional de Ciencias Médicas y Nutrición, Mexico City, Mexico.
authors.forename: Abraham
authors.initials: A
authors.lastname: Reynoso
authors.affiliation: Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502, Col. Sección XVI, Tlalpan, C.P, 14080, Mexico City, Mexico.
authors.forename: Natllely
authors.initials: N
authors.lastname: Ruiz
authors.affiliation: Department of Radiology and Molecular Imaging, Instituto Nacional de Ciencias Médicas y Nutrición, Mexico City, Mexico.
authors.forename: Rosa Delia
authors.initials: RD
authors.lastname: Delgado-Hernández
authors.affiliation: Department of Radiology, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: Gilberto
authors.initials: G
authors.lastname: Gómez-Garza
authors.affiliation: Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502, Col. Sección XVI, Tlalpan, C.P, 14080, Mexico City, Mexico. felipe98@prodigy.net.mx.
authors.forename: Luis Felipe
authors.initials: LF
authors.lastname: Flores-Suárez
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D002648:Child; D003389:Cranial Nerve Diseases / Q000209:etiology; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006261:Headache / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D019586:Intracranial Hypertension / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000175:diagnosis / Q000188:drug therapy*; D008800:Mexico; D008875:Middle Aged; D012189:Retrospective Studies; D012196:Review Literature as Topic; D000069283:Rituximab / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: Central nervous system; Granulomatosis with polyangiitis; MRI; Pachymeningits; Vasculitis