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IgG4-related disease of the central and peripheral nervous systems

Published on Feb 1, 2018in Lancet Neurology28.75
· DOI :10.1016/S1474-4422(17)30471-4
Mahmoud A. AbdelRazek1
Estimated H-index: 1
(Harvard University),
Nagagopal Venna16
Estimated H-index: 16
(Harvard University),
John H. Stone67
Estimated H-index: 67
(Harvard University)
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Abstract
Summary IgG4-related disease can involve nearly any organ system, including the central and peripheral nervous systems. The pathology findings are consistent from organ to organ, but careful clinicopathological correlation is necessary to establish the diagnosis. Many non-neurological and neurological inflammatory conditions, previously regarded as idiopathic in nature, are now recognised to fall within the spectrum of IgG4-related disease. The condition is highly treatable, but probably remains substantially under-recognised. In this Review, we offer an important and timely update on the current and emerging aspects of this neurological disease. Following a short overview of IgG4-related disease, we describe the current understanding of neurological findings, pathophysiology, approaches to diagnosis, and treatment of IgG4-related disease affecting the central and peripheral nervous systems.
  • References (76)
  • Citations (7)
Cite
References76
Newest
Published on Apr 1, 2017in European Radiology3.96
J. Ben Soussan1
Estimated H-index: 1
,
Romain Deschamps10
Estimated H-index: 10
+ 8 AuthorsH. Picard3
Estimated H-index: 3
Objectives To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD).
Published on Feb 1, 2017in Annals of the Rheumatic Diseases14.30
Takashi Maehara16
Estimated H-index: 16
(Kyushu University),
Hamid Mattoo16
Estimated H-index: 16
(Harvard University)
+ 7 AuthorsShiv Pillai45
Estimated H-index: 45
(Harvard University)
Objectives IgG4-related disease (IgG4-RD) is a chronic, systemic, inflammatory condition of unknown aetiology. We have recently described clonally expanded circulating CD4 + cytotoxic T lymphocytes (CTLs) in IgG4-RD that infiltrate affected tissues where they secrete interleukin (IL)-1β and transforming growth factor -β1 (TGF-β1). In this study, we sought to examine the role of CD4 + CTLs in the pathogenesis of IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) and to determine whether thes...
Published on Dec 1, 2016in Journal of Neurosurgery
Bishan Dass Radotra19
Estimated H-index: 19
,
Ashish Aggarwal20
Estimated H-index: 20
+ 2 AuthorsDebajyoti Chatterjee4
Estimated H-index: 4
IgG4-related disease is relatively new disease entity and a rare one, and our knowledge of this entity continues to evolve. It was first described in the pancreas and since then has been described in virtually every organ. Spinal involvement resulting in pachymeningitis is rare, and there are only 8 reported cases of the same to date, with the cervicothoracic spine being the most commonly affected region. The authors describe 2 cases in which the patients presented with spinal compression result...
Published on Nov 1, 2016in American Journal of Otolaryngology0.93
Cameron C. Wick5
Estimated H-index: 5
(UTSW: University of Texas Southwestern Medical Center),
Joseph Zachariah1
Estimated H-index: 1
(St Mary's Hospital)
+ 5 AuthorsCliff A. Megerian28
Estimated H-index: 28
Abstract IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her rese...
Published on Sep 1, 2016in Zeitschrift Fur Rheumatologie0.90
Cory A. Perugino5
Estimated H-index: 5
,
John H. Stone67
Estimated H-index: 67
IgG4-related disease (IgG4-RD) is capable of causing great morbidity and even mortality if the condition remains undiagnosed or poorly treated, yet is typically a treatment-responsive disorder. Glucocorticoids have not been studied rigorously and practices with regard to dosing and duration of treatment remain largely empiric. In addition, IgG4-RD patients are often particularly susceptible to and intolerant of the deleterious effects of glucocorticoid therapy. B cell depletion with anti-CD20 mo...
Published on Sep 1, 2016in The Journal of Allergy and Clinical Immunology
Hamid Mattoo16
Estimated H-index: 16
(Harvard University),
Vinay S. Mahajan20
Estimated H-index: 20
(Harvard University)
+ 11 AuthorsMollie N. Carruthers15
Estimated H-index: 15
(Harvard University)
Background IgG 4 -related disease (IgG 4 -RD) is a systemic condition of unknown cause characterized by highly fibrotic lesions with dense lymphoplasmacytic infiltrates. CD4 + T cells constitute the major inflammatory cell population in IgG 4 -RD lesions. Objective We used an unbiased approach to characterize CD4 + T-cell subsets in patients with IgG 4 -RD based on their clonal expansion and ability to infiltrate affected tissue sites. Methods We used flow cytometry to identify CD4 + effector/me...
Published on Jul 18, 2016in Zeitschrift Fur Rheumatologie0.90
Cory A. Perugino5
Estimated H-index: 5
(Harvard University),
John H. Stone67
Estimated H-index: 67
(Harvard University)
IgG4-related disease (IgG4-RD) is capable of causing great morbidity and even mortality if the condition remains undiagnosed or poorly treated, yet is typically a treatment-responsive disorder. Glucocorticoids have not been studied rigorously and practices with regard to dosing and duration of treatment remain largely empiric. In addition, IgG4-RD patients are often particularly susceptible to and intolerant of the deleterious effects of glucocorticoid therapy. B cell depletion with anti-CD20 mo...
Published on Jun 1, 2016in Practical Neurology
Thomas Williams2
Estimated H-index: 2
(QMUL: Queen Mary University of London),
Monica Marta18
Estimated H-index: 18
(QMUL: Queen Mary University of London),
Gavin Giovannoni72
Estimated H-index: 72
(QMUL: Queen Mary University of London)
Hypertrophic pachymeningitis secondary to IgG4-related disease is a rare but sometimes devastating cause of intracranial hypertension. It has the potential for an excellent response to corticosteroids or rituximab. We discuss the clinical presentation, imaging, histology (with its difficult distinction from lymphoma), management and follow-up of a case, including relapse and re-treatment following an initial response to rituximab.
Published on May 1, 2016in European Spine Journal2.51
Rui Gu5
Estimated H-index: 5
(JLU: Jilin University),
Peng-yuan Hao1
Estimated H-index: 1
(JLU: Jilin University)
+ 2 AuthorsQingsan Zhu5
Estimated H-index: 5
(JLU: Jilin University)
Purpose To report a case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP).
Published on May 1, 2016in Medicine1.87
Mingju Hao2
Estimated H-index: 2
,
Min Liu1
Estimated H-index: 1
+ 2 AuthorsJinming Li17
Estimated H-index: 17
Many studies about serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD) have been reported. However, these studies had relatively small sample sizes and the diagnostic accuracy values varied much between them. The aim of this study was to perform a meta-analysis to evaluate the diagnostic value of serum IgG4 for IgG4-RD. We conducted a search of relevant articles using MEDLINE, EMBASE, Web of Science, SCOPUS, and Cochrane Library databases published before December 2015. Studies those ...
Cited By7
Newest
Published in Journal of Neuroimmunology2.83
Jingfang Lin (Sichuan University), Linmao Zheng (Sichuan University)+ -3 AuthorsZhen Hong (Sichuan University)
Abstract IgG4-related disease (IgG4-RD) is an immune-mediated condition that affects multiple organ systems with tumorlike swelling. Here, for the first time, we report a rare case of IgG4-RD that involves the cerebral parenchyma, intramedullary spinal cord and lymph nodes. The diagnosis of IgG4-RD was made based on histological features, multi organ involvement and high serum IgG4 levels. The patient responded well to glucocorticoid treatment. Our case broadens the phenotypic presentation for t...
Published on Jun 1, 2019in Journal of Neuroimmunology2.83
Carolina Soares (University of Porto), Diana F. Martins + 4 AuthorsAndreia Costa4
Estimated H-index: 4
(University of Porto)
Abstract We describe the case of a 69-year-old man who presented with symptoms of headache and severe vision loss due to G4 immunoglobulin (IgG4) hypertrophic pachymeningitis (HP). The patient was initially responsive to corticotherapy, but vision loss progressed when steroid therapy was first tapered. No improvement was noticed with intravenous rituximab. The patient showed clinical and radiological improvement after intrathecal rituximab, which can be an efficacious alternative treatment optio...
Published on May 1, 2019in Lancet Neurology28.75
Carina Bunschoten2
Estimated H-index: 2
(EUR: Erasmus University Rotterdam),
Bc Jacobs40
Estimated H-index: 40
(EUR: Erasmus University Rotterdam)
+ 2 AuthorsDavid R. Cornblath83
Estimated H-index: 83
(JHUSOM: Johns Hopkins University School of Medicine)
Summary Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intrav...
Published on Jul 1, 2019in Neuroimmunology and Neuroinflammation
M. Levraut , Mikael Cohen9
Estimated H-index: 9
+ 8 AuthorsNihal Martis3
Estimated H-index: 3
Objective Meningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP). Methods Two IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and cas...
Published on Jul 1, 2019in Deutsche Medizinische Wochenschrift0.64
D. Kiefer1
Estimated H-index: 1
(RUB: Ruhr University Bochum),
U. Kiltz15
Estimated H-index: 15
(RUB: Ruhr University Bochum),
Jürgen Braun93
Estimated H-index: 93
(RUB: Ruhr University Bochum)
Anamnese Eine 68-jahrige Patientin wurde mit seit 6 Monaten bestehender Verschlechterung des Allgemeinzustandes, Ruckenschmerzen, 20 kg Gewichtsverlust sowie akuten Paresen der Hande aufgenommen. Untersuchungen und Diagnose Es zeigten sich beidseitige Paresen der Hand- und Fingermuskulatur mit Nachweis entsprechender motorischer Defizite im EMG. Die MRT zeigte eine intraspinale Raumforderung mit Myelonkompression in HWS und BWS. Das Biopsat einer Hemilaminektomie bei HWK 7 zeigte IgG4-positive P...
Published on Apr 16, 2019in Molecular Genetics & Genomic Medicine
John H. Newman56
Estimated H-index: 56
(Vandy: Vanderbilt University),
Aaron C. Shaver3
Estimated H-index: 3
(Vandy: Vanderbilt University)
+ 209 AuthorsWilliam M. Stone (Ragon Institute of MGH, MIT and Harvard)
Published on Apr 1, 2019in The Neurohospitalist
Karan Topiwala (UConn: University of Connecticut), Christopher Hampton (UConn: University of Connecticut)+ 1 AuthorsDavid M. Waitzman18
Estimated H-index: 18
(UConn: University of Connecticut)