Abstract | OBJECTIVE: METHODS: We retrospectively analysed 15 patients (11 men and four women). HPM was diagnosed based on thickening and enhancing of the brain and/or spinal dura mater on gadolinium-enhanced magnetic resonance imaging (MRI) T1 sequence. RESULTS: The median age at HPM onset was 60 years. Headache and cranial nerve impairment were observed in 14 and 10 patients, respectively. Otitis media and/or mastoiditis were found as complications of AAV in 11 patients. Fourteen patients were classified as having granulomatosis with polyangiitis (GPA). Single-positive myeloperoxidase- ANCA, single-positive proteinase 3-ANCA, and double-positive ANCA were identified in seven patients, five patients, and one patient, respectively. With MRI, thickening of the dura mater in the cranial fossa and tentorium cerebelli was found in 10 and eight patients, respectively. For remission induction, all patients were treated with corticosteroids, and immunosuppressants were added in 10 patients. Dura mater thickening partially improved in all patients, and cranial neuropathy completely remitted in eight patients. In a median follow-up of 43 months, four patients had HPM relapse and underwent reinduction therapy. All six patients treated with cyclophosphamide at initial therapy did not relapse. CONCLUSIONS:
|
Authors | T Sakairi, N Sakurai, M Nakasatomi, H Ikeuchi, Y Kaneko, A Maeshima, Y Nojima, K Hiromura |
Journal | Scandinavian journal of rheumatology
(Scand J Rheumatol)
Vol. 48
Issue 3
Pg. 218-224
(May 2019)
ISSN: 1502-7732 [Electronic] England |
PMID | 30394816
(Publication Type: Journal Article)
|
Chemical References |
- Antibodies, Antineutrophil Cytoplasmic
- Immunosuppressive Agents
|
Topics |
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
(complications, diagnosis)
- Antibodies, Antineutrophil Cytoplasmic
(blood)
- Brain
(diagnostic imaging)
- Cranial Nerve Diseases
(diagnosis, etiology)
- Dura Mater
(diagnostic imaging, pathology)
- Female
- Granulomatosis with Polyangiitis
(complications, diagnosis)
- Humans
- Hypertrophy
- Immunosuppressive Agents
(therapeutic use)
- Japan
- Magnetic Resonance Imaging
(methods)
- Male
- Meningitis
(diagnosis, drug therapy, immunology, physiopathology)
- Middle Aged
- Remission Induction
|