Cerebrospinal fluid levels of BAFF and APRIL as direct indicators of disease activity in anti-neutrophil cytoplasmic ant
- PDF / 1,246,306 Bytes
- 4 Pages / 595.276 x 790.866 pts Page_size
- 76 Downloads / 157 Views
LETTERS OF BIOMEDICAL AND CLINICAL RESEARCH
Cerebrospinal fluid levels of BAFF and APRIL as direct indicators of disease activity in anti-neutrophil cytoplasmic antibody–related hypertrophic pachymeningitis Yasuhiro Shimojima 1
&
Dai Kishida 1 & Shun Nomura 1 & Yoshiki Sekijima 1
Received: 29 May 2020 / Revised: 23 June 2020 / Accepted: 26 June 2020 # International League of Associations for Rheumatology (ILAR) 2020
One of the major causes of immune-mediated hypertrophic pachymeningitis (HP) is anti-neutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) [1–3]. The characteristic brain magnetic resonance imaging (MRI) findings, including gadolinium (Gd)–enhanced thickened dura mater, elevated levels of protein in the cerebrospinal fluid (CSF), and IgG index, are the common indicators of HP. Moreover, we have previously demonstrated that the levels of B cell activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand (APRIL) are significantly increased in the CSF during the active phase of HP; furthermore, they are correlated with IgG index in ANCA-associated HP (ANCA-HP), suggesting that BAFF and APRIL may potentially impact the development of ANCA-HP through promoting autoreactive B cells in the central nervous system (CNS) [3, 4]. Even though other CSF biomarkers of HP have also been identified previously [1, 5], it was not possible to evaluate the quantitative alteration of biomarkers after the remission, because all the investigations were based on the comparisons with other neurological diseases [1, 3–5]. Moreover, it is required to clarify their practical usefulness in evaluating therapeutic efficacy. Here, we evaluated CSF levels of BAFF and APRIL, using the enzyme-linked immunosorbent assay [3, 4], by reviewing two cases before and after the treatment. Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10067-020-05270-6) contains supplementary material, which is available to authorized users. * Yasuhiro Shimojima [email protected] 1
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi Matsumoto 390-8621 Japan
Case 1 A 49-year-old woman suffering from granulomatosis with polyangiitis (GPA), who was treated with prednisolone (PSL) at 20 mg/day and methotrexate at 8 mg/week, was admitted to our hospital because she had high-grade fever and severe headache. She had a 13-year history of GPA with scleritis, sinusitis, and pulmonary nodules, and was previously treated with cyclophosphamide, tacrolimus, or cyclosporin. Brain MRI scans revealed thickened dura mater on the right parietotemporal lobe (Fig. 1a). Increased serum levels of Creactive protein (CRP; 14.57 mg/dL; normal, < 0.1 mg/dL) and proteinase 3-ANCA (65.3 U/mL; normal, < 3.4 U/mL) were identified. Routine CSF examinations revealed increased protein levels (56 mg/dL; normal, 10–40 mg/dL) and IgG index (0.92), while indicating the normal number of cells (4 cells/μL; normal, < 5 cells/μL). Intravenous
Data Loading...
