Meiqi Wu1,2, Jingyun Ren1,2, Yaping Luo1,2. 1. Department of Nuclear Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital. 2. Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine; Beijing, PR China.
Abstract
RATIONALE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT. PATIENT CONCERNS: A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago. DIAGNOSIS: Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis. INTERVENTIONS: The patient was treated with a higher dose of prednisone and cyclophosphamide. OUTCOMES: After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord. LESSONS: 18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.
RATIONALE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT. PATIENT CONCERNS: A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago. DIAGNOSIS: Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis. INTERVENTIONS: The patient was treated with a higher dose of prednisone and cyclophosphamide. OUTCOMES: After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord. LESSONS: 18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.
Authors: T Sakairi; N Sakurai; M Nakasatomi; H Ikeuchi; Y Kaneko; A Maeshima; Y Nojima; K Hiromura Journal: Scand J Rheumatol Date: 2018-11-05 Impact factor: 3.641
Authors: Rosa Fonti; Barbara Salvatore; Amalia De Renzo; Emanuele Nicolai; Silvana Del Vecchio Journal: Clin Nucl Med Date: 2016-02 Impact factor: 7.794
Authors: J C Jennette; R J Falk; P A Bacon; N Basu; M C Cid; F Ferrario; L F Flores-Suarez; W L Gross; L Guillevin; E C Hagen; G S Hoffman; D R Jayne; C G M Kallenberg; P Lamprecht; C A Langford; R A Luqmani; A D Mahr; E L Matteson; P A Merkel; S Ozen; C D Pusey; N Rasmussen; A J Rees; D G I Scott; U Specks; J H Stone; K Takahashi; R A Watts Journal: Arthritis Rheum Date: 2013-01