Adolescent PR3-ANCA-positive hypertrophic pachymeningitis

Abstract

Rationale:Hypertrophic pachymeningitis (HP) is an uncommon, life-threatening disease that is seen in elderly patients with antineutrophil cytoplasmic antibody (ANCA) positivity. Proteinase-3 (PR3)-ANCA-positive HP has not been reported in adolescents. Here, we report the first case of adolescent PR3-ANCA-positive HP successfully treated with immunosuppressive therapy.Patient concerns:A 14-year-old female presented with fullness and pain in her right ear unresponsive to antibiotics. Laboratory tests showed an elevated C-reactive protein and PR3-ANCA positivity. Computed tomography and magnetic resonance imaging revealed mastoiditis in the right temporal bone. Surgical biopsy revealed severe fibrosis and prominent inflammatory-cell infiltration. She received prednisolone and methotrexate therapy, and then underwent a right mastoidectomy. Five months later, she developed headache, dysarthria, and multiple cranial nerve palsies. Further imaging revealed enhancement and thickening of the right hemispheric dura.Diagnosis:PR3-ANCA-positive HP.Interventions:She was successfully treated with steroid pulse therapy for 3 days, followed by high doses of prednisolone and intravenous cyclophosphamide.Outcome:The treatment resulted in significant improvement of her symptoms, laboratory data, and radiologic findings.Lessons:PR3-ANCA-positive HP can present not only in the elderly, but also in adolescence, and prompt diagnosis and treatment with immunosuppressive therapy is vital.

Longitudinal immune cell monitoring identified CD14++ CD16+ intermediate monocyte as a marker of relapse in patients with ANCA-associated vasculitis

“…We showed that changes in plasma cells were strongly correlated with the reactivation of CNS disease. This hypothesis is supported by our recent finding of a significant correlation between circulating B cells and the CNS component of BVAS [21,38]. CNS lesion may be a good indicator of the need for B cell depletion therapy in AAV.…”

Idiopathic Hypertrophic Pachymeningitis: Does Earlier Treatment Improve Outcome?

“…We performed a review of the literature (Medline 1990–present) using the search terms (hypertrophic pachymeningitis) AND (paediatric OR pediatric OR children OR childhood). Twelve cases referring to children (<18 years old) were identified and the full manuscripts were available for review [ 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 ].…”

“…Among the twelve pediatric cases of hypertrophic pachymeningitis reported in the literature, three cases developed in association with tuberculosis [ 10 , 11 , 17 ], one was PR3-ANCA-associated [ 13 ], two developed in association with IgG4-RD [ 16 , 18 ] and six were regarded as idiopathic [ 7 , 8 , 9 , 12 , 14 , 15 ]. The clinical features, MRI findings, biopsy results, treatment and outcomes are summarized in Table 2 .…”

Recognition of Rare, Atypical Manifestations Is Important for Diagnosis and Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Comment on the Article by Delaval et al

“…To the Editor: I read with great interest the article by Delaval et al reporting on their study of the largest case series of temporal arteritis (TA) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), including clinical, serologic, and histologic manifestations (1). Recent progress in the understanding of AAV has revealed that it can present rare, atypical manifestations, such as isolated dacryosialadenitis, isolated retroperitoneal fibrosis, and isolated hypertrophic pachymeningitis (2)(3)(4). Therefore, in such cases, it is important to recognize AAV as one of the differential diagnoses and to conduct histologic examination and ANCA testing for appropriate disease management.…”