For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
8
views
5
references
 Top references
cited by
2
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      447
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy

      letter

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.

          Related collections

          Most cited references5

          • Record: found
          • Abstract: found
          • Article: not found

          Standardized neurologic evaluations of 128 patients with Wegener granulomatosis.

          Krista K Bartz, E Reinhold, Friederike A Arlt … (2001)
          To assess the frequency and type of neurologic involvement in a cohort of patients with generalized Wegener granulomatosis (WG). In a prospective analysis the clinical, electrophysiologic, radiological, and serologic data of 128 patients have been studied over a median observation period of 19 months (range, 1-60 months). Sixty-four patients (50%) revealed central or peripheral nervous system involvement. Peripheral neuropathy (PN) affected 56 patients, in 9 cases the central nervous system was involved, and in 6 cases the cranial nerves were involved. Thirty-one patients showed a distal symmetrical polyneuropathy, 25 a mononeuritis multiplex. Within the first 2 years of the disease course 47 of the 56 patients had developed their PN, sometimes as the initial symptom of WG. Patients with PN were significantly more often male (34 of 65 patients) than female (22 of 63 patients, P =.04), were significantly older at the onset of WG (median age, 53 vs 44 years; P =.001), had a significantly larger disease extent (P =.001), and had higher classic antineutrophil cytoplasmic antibody titers (P =.002) than neurologically unaffected patients. Response to immunosuppression was moderate concerning peripheral nervous system manifestations. Peripheral neuropathy is frequent in generalized WG, occurring early in the disease course. As PN can be the first and sole symptom of a beginning systemic vasculitis, it is important that in cases of PN of an unclear origin, interdisciplinary investigations are initiated to detect, treat, and closely follow-up a possible underlying WG, especially as these patients seem to have a more severe disease course.
          Article 0 comments Cited 29 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA.

            P Lamprecht, U Schönermarck, E Csernok … (2001)
            To evaluate the prevalence and association of antineutrophil cytoplasmic antibodies (ANCA) and their subtypes [proteinase 3 (PR3)-ANCA, myeloperoxidase (MPO)-ANCA] with distinct clinical features in various clinicopathological syndromes. All consecutive ANCA-positive patients seen at the combined unit for rheumatology for Bad Bramstedt and the University of Lübeck between 1989 and 1999 were analysed. ANCA were detected by an immunofluorescence technique and ANCA subspecificities were determined by ELISA. Clinical features at presentation and diagnoses were recorded according to standardized procedures. Among 4620 patients tested, 333 were cytoplasmic ANCA-positive and 291 were perinuclear ANCA-positive. cANCA/PR3-ANCA were strongly associated with Wegener's granulomatosis (WG), whereas pANCA/MPO-ANCA were associated with a diverse disease spectrum. Further investigation of PR3-ANCA-positive (n=80) and MPO-ANCA-positive patients (n=40) revealed a greater extent of disease [disease extent index (DEI); median 8 vs 5, P<0.01] and more frequent involvement of the upper/lower respiratory tract and the eyes in PR3-ANCA-positive than in MPO-ANCA-positive patients. Fewer than 5% of WG patients were MPO-ANCA-positive. Compared with matched PR3-ANCA-positive WG patients, the MPO-ANCA-positive WG patients had a lower DEI (median 5 vs 8) and had a lower frequency of peripheral neuropathy. ANCA testing is useful due to its high sensitivity and specificity, especially for cANCA/PR3-ANCA in WG. We found a divergence in the disease spectrum between PR3- and MPO-ANCA-positive patients, characterized by higher DEI and extrarenal manifestations in the PR3-ANCA group. MPO-ANCA was rarely found in WG and was associated with less organ involvement.
            Article 0 comments Cited 22 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: not found
              • Article: not found

              What is the significance in routine care of c-ANCA/PR3-ANCA in the absence of systemic vasculitis? A case series

              A. KNIGHT, A Ekbom, L. BRANDT … (2008)
              Article 0 comments Cited 5 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Contributors
                Masanori Kurihara
                Journal
                Journal ID (nlm-ta): eNeurologicalSci
                Journal ID (iso-abbrev): eNeurologicalSci
                Title: eNeurologicalSci
                Publisher: Elsevier
                ISSN (Electronic): 2405-6502
                Publication date PMC-release: 05 October 2016
                Publication date Collection: March 2017
                Publication date (Electronic): 05 October 2016
                Volume: 6
                Pages: 4-5
                Affiliations
                [a ]Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan
                [b ]Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
                [c ]Department of Nephrology, Mitsui Memorial Hospital, Tokyo, Japan
                [d ]Department of Neurology, Teikyo University School of Medicine, Tokyo, Japan
                Author notes
                [* ]Corresponding author at: Department of Neurology, Graduate School of Medicine, University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.Department of NeurologyGraduate School of MedicineUniversity of Tokyo7-3-1, Hongo, Bunkyo-kuTokyo113-8655Japan mkurihara-tky@ 123456umin.ac.jp
                Article
                Publisher ID: S2405-6502(16)30031-4
                DOI: 10.1016/j.ensci.2016.10.001
                PMC ID: 5721574
                PubMed ID: 29260006
                SO-VID: 50e48668-0aac-4e77-b760-319b1a837bde
                Copyright © © 2016 The Author(s)
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 29 September 2016
                Date accepted : 4 October 2016
                Categories
                Subject: Letters to the Editor

                Keywords: pr3-anti-neutrophil cytoplasmic antibody (pr3-anca),chronic inflammatory demyelinating polyneuropathy (cidp),vasculitic neuropathy
                Data availability:
                Keywords: pr3-anti-neutrophil cytoplasmic antibody (pr3-anca), chronic inflammatory demyelinating polyneuropathy (cidp), vasculitic neuropathy

                Comments

                Comment on this article

                scite_

                Similar content447

                See all similar

                Cited by2

                See all cited by

                Most referenced authors30

                See all reference authors