Nerve ultrasound findings in Sjögren's syndrome-associated neuropathy.
Sjögren's syndrome
Sjögren's syndrome-associated neuropathy
nerve sonography
nerve ultrasound
neuro-Sjögren
Journal
Journal of neuroimaging : official journal of the American Society of Neuroimaging
ISSN: 1552-6569
Titre abrégé: J Neuroimaging
Pays: United States
ID NLM: 9102705
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
revised:
30
06
2021
received:
08
06
2021
accepted:
30
06
2021
pubmed:
17
7
2021
medline:
19
3
2022
entrez:
16
7
2021
Statut:
ppublish
Résumé
The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy. Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve. Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
Sections du résumé
BACKGROUND AND PURPOSE
OBJECTIVE
The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy.
METHODS
METHODS
Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve.
RESULTS
RESULTS
Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound.
CONCLUSIONS
CONCLUSIONS
In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1156-1165Informations de copyright
© 2021 American Society of Neuroimaging.
Références
Sjögren H. Zur Kenntnis der Keratoconjunctivitis Sicca. Acta Ophthalmol 1933;13:1-39.
Fox RI. Sjögren's syndrome. Lancet 2005;366:321-31.
Seeliger T, Prenzler NK, Gingele S, et al. Neuro-Sjögren: peripheral neuropathy with limb weakness in Sjögren's syndrome. Front Immunol 2019;10:1600.
Seeliger T, Gingele S, Bönig L, et al. CIDP associated with Sjögren's syndrome. J Neurol 2021 (Epub ahead of print)
Pars K, Pul R, Schwenkenbecher P, et al. Cerebrospinal fluid findings in neurological diseases associated with Sjögren's syndrome. Eur Neurol 2017;77:91-102.
Jaskólska M, Chylińska M, Masiak A, et al. Neuro-Sjögren: uncommon or underestimated problem? Brain Behav 2020;10:e01665.
Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. Brain 2005;128:2518-34.
Seeliger T, Bönig L, Witte T, et al. Hearing dysfunction in patients with neuro-Sjögren: a cross-sectional study. Ann Transl Med 2020;8:1069.
Böhm J, Schelle T. Stellenwert der hochauflösenden sonografie bei der diagnostik peripherer nervenerkrankungen. Aktuelle Neurol 2013;40:258-68.
Imamura K, Tajiri Y, Kowa H, Nakashima K. Peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy detected by ultrasonography. Intern Med 2009;48:581-2.
Beekman R, Van Den Berg LH, Franssen H, Visser LH, Van Asseldonk JTH, Wokke JHJ. Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy. Neurology 2005;65:305-7.
Scheidl E, Böhm J, Simó M, et al. Ultrasonography of MADSAM neuropathy: focal nerve enlargements at sites of existing and resolved conduction blocks. Neuromuscul Disord 2012;22:627-31.
Taniguchi N, Itoh K, Wang Y, et al. Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Ultrasound 2000;28:488-91.
Matsuoka N, Kohriyama T, Ochi K, et al. Detection of cervical nerve root hypertrophy by ultrasonography in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci 2004;219:15-21.
Herraets IJT, Goedee HS, Telleman JA, et al. Nerve ultrasound for diagnosing chronic inflammatory neuropathy: a multicenter validation study. Neurology 2020;95:e1745-e53.
Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome. Ann Rheum Dis 2017;76:9-16.
Chisholm DM, Waterhouse JP, Mason DK. Lymphocytic sialadenitis in the major and minor glands: a correlation in postmortem subjects. J Clin Pathol 1970;23:690-4.
Grimm A, Axer H, Heiling B, Winter N. Nerve ultrasound normal values - readjustment of the ultrasound pattern sum score UPSS. Clin Neurophysiol 2018;129:1403-9.
Grimm A, Winter N, Rattay TW, et al. A look inside the nerve - morphology of nerve fascicles in healthy controls and patients with polyneuropathy. Clin Neurophysiol 2017;128:2521-26.
Stålberg E, Falck B, Gilai A, Jabre J, Sonoo M, Todnem K. Standards for quantification of EMG and neurography. The International Federation of Clinical Neurophysiology. Electroencephalogr Clin Neurophysiol Suppl 1999;52:213-20.
Hughes R, Bensa S, Willison H, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 2001;50:195-201.
Seror R, Theander E, Brun JG, et al. Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis 2015;74:859-66.
Fisse AL, Katsanos AH, Gold R, Pitarokoili K, Krogias C. Cross-sectional area reference values for peripheral nerve ultrasound in adults: a systematic review and meta-analysis-Part III: Cervical nerve roots and vagal nerve. Eur J Neurol 2021;28:2319-26.
Telleman JA, Grimm A, Goedee S, Visser LH, Zaidman CM. Nerve ultrasound in polyneuropathies. Muscle Nerve 2018;57:716-28.
Zaidman CM, Al-Lozi M, Pestronk A. Peripheral nerve size in normals and patients with polyneuropathy: an ultrasound study. Muscle Nerve 2009;40:960-6.
Kramer M, Grimm A, Winter N, et al. Nerve ultrasound as helpful tool in polyneuropathies. Diagnostics (Basel) 2021;11:211.
Beekman R, Van Den Berg LH, Franssen H, Visser LH, Van Asseldonk JTH, Wokke JHJ. Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy. Neurology 2005;65:305-7.
Kerasnoudis A, Pitarokoili K, Gold R, Yoon MS. Nerve ultrasound and electrophysiology for therapy monitoring in chronic inflammatory demyelinating polyneuropathy. J Neuroimaging 2015;25:931-9.
Padua L, Granata G, Sabatelli M, et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol 2014;125:160-5.
Di Pasquale A, Morino S, Loreti S, Bucci E, Vanacore N, Antonini G. Peripheral nerve ultrasound changes in CIDP and correlations with nerve conduction velocity. Neurology 2015;84:803-9.
Scheidl E, Böhm J, Simó M, Bereznai B, Bereczki D, Arányi Z. Different patterns of nerve enlargement in polyneuropathy subtypes as detected by ultrasonography. Ultrasound Med Biol 2014;40:1138-45.
Grant IA, Hunder GG, Homburger HA, Dyck PJ. Peripheral neuropathy associated with sicca complex. Neurology 1997;48:855-62.
Bellander BM, Singhrao SK, Ohlsson M, Mattsson P, Svensson M. Complement activation in the human brain after traumatic head injury. J Neurotrauma 2001;18:1295-1311.
Liang DY, Li X, Shi X, et al. The complement component C5a receptor mediates pain and inflammation in a postsurgical pain model. Pain 2012;153:366-72.
Stahel PF, Morganti-Kossmann MC, Kossmann T. The role of the complement system in traumatic brain injury. Brain Res Rev 1998;27:243-56.