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IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease.

Published on Aug 1, 2009in The American Journal of Surgical Pathology6.16
· DOI :10.1097/PAS.0b013e3181abdfc2
Siu-Ki Chan1
Estimated H-index: 1
,
Wah Cheuk30
Estimated H-index: 30
+ 1 AuthorsJohn K. C. Chan79
Estimated H-index: 79
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Abstract
Abstract IgG4-related sclerosing disease is a distinctive mass-forming lesion with frequent systemic involvement, most frequently the pancreas, salivary glands, and lacrimal glands. This report describes a case manifesting with a previously unrecognized form of central nervous system involvement. The 37-year-old man presented with signs and symptoms of spinal cord compression at the thoracic level 9. Magnetic resonance imaging revealed an elongated dural mass extending from the fifth to tenth thoracic vertebra. Laminectomy and excision of the mass revealed dura expanded by a dense lymphoplasmacytic infiltrate accompanied by stromal fibrosis and phlebitis. IgG4+ plasma cells were increased and the proportion of IgG4+/IgG+ plasma cells was 85%. The patient also had a 1-year history of bilateral submandibular swelling due to chronic sialadenitis. Thus, IgG4-related sclerosing pachymeningitis represents a new member of the IgG4-related sclerosing disease family affecting the central nervous system. It seems that at least a proportion of cases described in the literature as idiopathic hypertrophic pachymeningitis belong to this disease, especially as some patients have other clinical manifestations compatible with IgG4-related sclerosing disease, such as cholangitis and orbital pseudotumor.
  • References (26)
  • Citations (119)
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References26
Newest
Published on Nov 1, 2008in The American Journal of Surgical Pathology6.16
Kyoko Yamashita3
Estimated H-index: 3
,
Hironori Haga42
Estimated H-index: 42
+ 3 AuthorsToshiaki Manabe37
Estimated H-index: 37
There have been a few reports on lung diseases associated with an increased number of infiltrating IgG4-positive plasma cells or lung involvement of IgG4-related sclerosing disease, although their characteristic histologic features have not been well described. Herein, we present 3 cases of interstitial lung disease with common histology and abundant IgG4-positive cell infiltration. Patient 1, a 65-year-old man, was incidentally noted to have a nodular lesion in the left hilar region. In patient...
Published on Aug 1, 2008in The American Journal of Surgical Pathology6.16
Wah Cheuk30
Estimated H-index: 30
,
Hunter K. L. Yuen MRCSEd12
Estimated H-index: 12
+ 6 AuthorsJohn K. C. Chan79
Estimated H-index: 79
IgG4-related sclerosing disease is a recently recognized inflammatory lesion frequently involving pancreas, submandibular gland, lacrimal gland, and lymph node. We report 3 cases of ocular adnexal lymphoma arising in IgG4-related chronic sclerosing dacryoadenitis, a phenomenon that has not been prev
Published on May 1, 2008in The American Journal of Surgical Pathology6.16
Wah Cheuk30
Estimated H-index: 30
,
Hunter K. L. Yuen MRCSEd12
Estimated H-index: 12
+ 3 AuthorsJohn K. C. Chan79
Estimated H-index: 79
IgG4-related sclerosing disease is a recently recognized syndrome characterized by mass-forming lesions in exocrine glands or extranodal tissues due to lymphoplasmacytic infiltrates and sclerosis, a raised serum IgG4 level and increased IgG4+ plasma cells in the involved tissues. We report the morph
Published on Jan 1, 2008in World Journal of Gastroenterology3.41
Terumi Kamisawa56
Estimated H-index: 56
,
Atsutake Okamoto32
Estimated H-index: 32
Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which t...
Published on Jan 1, 2008in Endocrine Journal1.94
Yoshihiro Isaka3
Estimated H-index: 3
,
Katsunobu Yoshioka15
Estimated H-index: 15
+ 6 AuthorsMasahito Imanishi27
Estimated H-index: 27
A 55-years-old man was admitted to our hospital with a 6-month history of general fatigue, purulent nasal discharge, polyuria, and polydipsia. Endocrinological findings revealed central diabetes insipidus (CDI) with mild anterior pituitary dysfunction. Imaging studies revealed thickening of the proximal end of the pituitary stalk just below the third ventricle, a mass in the paranasal sinus, and a mass encompassing the abdominal aorta. Histopathology of the mass in the paranasal sinus revealed a...
Published on Nov 1, 2007in Human Pathology2.74
Shun Wong2
Estimated H-index: 2
,
Wai Y. Lam1
Estimated H-index: 1
(Hong Kong Department of Health)
+ 1 AuthorsKam C. Lee1
Estimated H-index: 1
Summary Immunoglobulin (Ig) G4–related systemic disease is a recently characterized entity. The best-known manifestation is pancreatitis. Other systemic involvements are also described. Three cases of this disease with hypophyseal involvement have been reported in the literature, all diagnosed clinically. We herein present the first case of IgG4-related hypophysitis diagnosed histopathologically. The patient is a 77-year-old Chinese man with a pituitary tumor. Histologic examination of the resec...
Published on Nov 1, 2007in American Journal of Neuroradiology3.26
Vinay V. Pai1
Estimated H-index: 1
(UMKC: University of Missouri–Kansas City),
Lisa H. Lowe11
Estimated H-index: 11
(UMKC: University of Missouri–Kansas City)
+ 2 AuthorsLei Shao5
Estimated H-index: 5
(UMKC: University of Missouri–Kansas City)
SUMMARY: Although the association of spinal lumbosacral dysraphism and congenital spinal dermoid tumors is well known, the association of craniocervical spinal anomalies and posterior fossa dermoids has only been recognized recently. Advances in imaging technology and awareness of the association likely contribute to an increase in recently reported cases.
Published on Mar 1, 2007in American Journal of Neuroradiology3.26
S. Pai1
Estimated H-index: 1
,
Cynthia T. Welsh8
Estimated H-index: 8
+ 1 AuthorsZoran Rumboldt23
Estimated H-index: 23
(MUSC: Medical University of South Carolina)
SUMMARY: Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis (IHSP) was found on histology. In both patients, an extensive extramedullary mass of low T2 signal with peripheral contrast enhancement was compressing the spinal cord on MR imaging. This imaging appearance in patients with chronic progressive myelopathy should suggest the diagnosis of IHSP.
Cited By119
Newest
Published on Jun 1, 2019in World Neurosurgery1.72
Stephen J. Slade (Virginia Mason Medical Center), Erin M. Bauer (Virginia Mason Medical Center)+ 1 AuthorsAmish J. Dave (Virginia Mason Medical Center)
Abstract Background Back pain is a leading reason patients seek medical attention. While musculoskeletal causes are common, rarer etiologies can present. Case Description A 50-year-old male presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. Over the next 3 months, his pain worsened, and he developed lower extremity dysesthesias and subjective weakness despite a normal neurological exam. Non-revealing laboratory workup included norm...
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 May 1, 2019in Seminars in Arthritis and Rheumatism5.07
Cléa Melenotte5
Estimated H-index: 5
,
Julie Seguier1
Estimated H-index: 1
+ 20 AuthorsLaurent Daniel30
Estimated H-index: 30
Abstract Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymening...
Published on Feb 1, 2019in Rivista Di Neuroradiologia
Shahine Goulam-Houssein (St. Michael's Hospital), Jeffrey L Grenville1
Estimated H-index: 1
(St. Michael's Hospital)
+ 4 AuthorsParaskevi A. Vlachou3
Estimated H-index: 3
(St. Michael's Hospital)
IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG...
Published on Dec 1, 2017in Clinical Nuclear Medicine6.50
Qian Zhao , Aisheng Dong7
Estimated H-index: 7
+ 2 AuthorsChangjing Zuo7
Estimated H-index: 7
Published on Nov 1, 2017in World Neurosurgery1.72
Evan S. Marlin2
Estimated H-index: 2
(The Ohio State University Wexner Medical Center),
David Dornbos4
Estimated H-index: 4
(The Ohio State University Wexner Medical Center)
+ 2 AuthorsCiaran J. Powers15
Estimated H-index: 15
(The Ohio State University Wexner Medical Center)
Background Diffuse intracranial aneurysmal vasculopathy is a rare condition, previously described in patients with human immunodeficiency virus infection. IgG4-related disease (IgG4-RD) is a recognized inflammatory disease of systemic organs, leading to fibrosis of connective tissues. It also has been linked to inflammatory dilating aortic aneurysms, coronary vascular disease, hypophysitis, orbital pseudotumor, and pachymeningitis. It has not yet been described as a cause of diffuse intracranial...
Published on Nov 1, 2017in International Journal of Rheumatic Diseases1.94
Andreu Fernández Codina7
Estimated H-index: 7
(Autonomous University of Barcelona),
Manuel Hernández-González3
Estimated H-index: 3
(Autonomous University of Barcelona)
+ 3 AuthorsFernando Martínez-Valle7
Estimated H-index: 7
(Autonomous University of Barcelona)
Published on Sep 28, 2017in British Journal of Neurosurgery1.48
Kelly J. Bridges2
Estimated H-index: 2
(OHSU: Oregon Health & Science University),
Caitlin H. DeDeaux (OHSU: Oregon Health & Science University), Khoi D. Than5
Estimated H-index: 5
(OHSU: Oregon Health & Science University)
AbstractObject: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder affecting various anatomical sites, and only recently was identified to affect the dura of the spine. The authors present the second reported case of an intradural extramedullary lesion consistent with IgG4-related spinal disease.Methods: A literature review was performed that identified 15 other cases of spinal disease, and common features of all known reported spinal IgG4-RD are discussed.Results: Spinal IgG4-RD ty...
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