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IgG4-Related Disease and Hypertrophic Pachymeningitis

Published on Jul 1, 2013in Medicine 1.87
· DOI :10.1097/MD.0b013e31829cce35
Zachary S. Wallace17
Estimated H-index: 17
,
Mollie N. Carruthers15
Estimated H-index: 15
+ 5 AuthorsJohn H. Stone67
Estimated H-index: 67
Cite
Abstract
Hypertrophic pachymeningitis (HP) is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from mass effect, nerve compression, or vascular compromise. The differential diagnosis of HP includes immune-mediated conditions such as rheumatoid arthritis and vasculitis, malignancies, and infections. Many times, no diagnosis is reached; in such cases, the disease has been described as idiopathic HP. IgG4-related disease (IgG4-RD) is a recently described inflammatory condition known to cause tumefactive lesions at myriad anatomical locations. Both IgG4-RD and idiopathic HP share similar demographics, histopathology, and natural history. We hypothesized that IgG4-RD is a common cause of idiopathic HP. To investigate this hypothesis, we identified all pathology specimens diagnosed as noninfectious HP during 25 years at our institution. Fourteen cases had stained slides and paraffin blocks to permit review of the original hematoxylin and eosin stained slides as well as immunostaining of cell blocks. Recently published consensus guidelines describing characteristic histopathology and the necessary quantity of IgG4+ plasma cell infiltrate were used to diagnose IgG4-RD. Four cases (66.6%) that had been regarded previously as representing idiopathic HP were diagnosed as IgG4-RD; of all the reviewed cases, IgG4-RD represented 29% of cases. Of the remaining cases, 3 cases were associated with granulomatosis with polyangiitis (GPA), 2 with lymphoma, and 1 each with rheumatoid arthritis, giant cell arteritis, and sarcoidosis. Two of the cases could not be diagnosed more precisely and were classified as undifferentiated HP. Clinical history, serologic tests, cerebrospinal fluid studies, and radiology alone could not identify the cause of HP. Rather, biopsy with histopathology and immunostaining was necessary to reach an accurate diagnosis. Significant IgG4+ plasma cell infiltrates were observed in rheumatoid arthritis, granulomatosis with polyangiitis, and lymphoma, underscoring the importance of histopathology in making the diagnosis of IgG4-RD. This case series demonstrates that IgG4-RD may be the most common etiology of noninfectious HP and highlights the necessity of biopsy for accurate diagnosis.
  • References (51)
  • Citations (82)
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References51
Newest
Published on Mar 1, 2013in Medicine 1.87
Arezou Khosroshahi25
Estimated H-index: 25
,
Mollie N. Carruthers15
Estimated H-index: 15
+ 4 AuthorsVikram V. Deshpande85
Estimated H-index: 85
Idiopathic retroperitoneal fibrosis (RPF) is a periaortic sclerotic disease that encases adjacent retroperitoneal structures, particularly the ureters. A subset of idiopathic RPF cases can be associated with IgG4-related disease, but the frequency of this association is not clear. We selected 23 cases of idiopathic RPF and identified IgG4-related RPF cases based on the presence of IgG4+ plasma cells in the tissue, using an IgG4/IgG ratio cutoff of >40%. We then compared the IgG4-related RPF pati...
Published on Sep 1, 2012in Modern Pathology 6.37
Vikram V. Deshpande85
Estimated H-index: 85
(Harvard University),
Yoh Zen62
Estimated H-index: 62
(University of Cambridge)
+ 38 AuthorsJudith A. Ferry57
Estimated H-index: 57
(Harvard University)
IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; and-often but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Ital...
Published on Aug 7, 2012in Neurology 8.69
Kevin Shapiro15
Estimated H-index: 15
,
Riley Bove17
Estimated H-index: 17
+ 2 AuthorsJohn H. Stone67
Estimated H-index: 67
Immunoglobulin G4–related disease (IgG4-RD) is a newly recognized immune-mediated condition that involves multiple organ systems and is frequently characterized by tumefactive lesions. Histopathology demonstrates lymphoplasmacytic infiltrates, storiform fibrosis, and abundant IgG4-positive plasma cells within affected tissues.1 Neurologic manifestations include spinal2,3 and intracranial pachymeningitis.4 One retrospective series suggests that some patients diagnosed with “idiopathic” hypertroph...
Published on Apr 1, 2012in Clinical Radiology 2.08
Koya Nakatani8
Estimated H-index: 8
(Kyoto University),
Yuji Nakamoto44
Estimated H-index: 44
(Kyoto University),
Kaori Togashi58
Estimated H-index: 58
(Kyoto University)
IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma c...
Published on Mar 6, 2012in Annals of Internal Medicine 19.32
Emanuel Della Torre3
Estimated H-index: 3
(UniSR: Vita-Salute San Raffaele University),
E. Bozzolo17
Estimated H-index: 17
(UniSR: Vita-Salute San Raffaele University)
+ 2 AuthorsMaria Grazia Sabbadini35
Estimated H-index: 35
(UniSR: Vita-Salute San Raffaele University)
Published on Feb 9, 2012in The New England Journal of Medicine 70.67
John H. Stone67
Estimated H-index: 67
,
Yoh Zen62
Estimated H-index: 62
,
Deshpande5
Estimated H-index: 5
Published on Jan 1, 2012in Survey of Ophthalmology 3.96
Zachary S. Wallace17
Estimated H-index: 17
(Harvard University),
Arezou Khosroshahi25
Estimated H-index: 25
(Harvard University)
+ 5 AuthorsJohn H. Stone67
Estimated H-index: 67
(Harvard University)
IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intract...
Published on Jan 1, 2012in Medicine 1.87
Arezou Khosroshahi25
Estimated H-index: 25
,
Mollie N. Carruthers15
Estimated H-index: 15
+ 3 AuthorsJohn H. Stone67
Estimated H-index: 67
AbstractPatients with IgG4-related disease (IgG4-RD) typically have elevated serum concentrations of IgG4 and share histopathologic features that are similar across affected organ(s). IgG4-RD patients frequently require prolonged treatment with glucocorticoids and are often unable to taper these med
Published on Jan 1, 2012in Current Opinion in Rheumatology 3.85
Mollie N. Carruthers15
Estimated H-index: 15
,
John H. Stone67
Estimated H-index: 67
,
Arezou Khosroshahi25
Estimated H-index: 25
Cited By82
Newest
Published on Jun 1, 2019in World Neurosurgery 1.72
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 Apr 24, 2019in BJR|case reports
Muram El-Nayir (East Sussex County Council), Ash Subramanian (East Sussex County Council)+ 1 AuthorsDavid C. Howlett19
Estimated H-index: 19
(East Sussex County Council)
IgG4-mediated sclerosing fibroinflammatory disease is a rare systemic disease which has the ability to form masses in multiple organs and may mimic malignancy. In this case we describe a 53-year-ol...
Published in Frontiers in Immunology 4.72
Debby van den Elshout-den Uyl (UU: Utrecht University), Clothaire P.E. Spoto1
Estimated H-index: 1
(UU: Utrecht University)
+ -3 AuthorsRoos J. Leguit7
Estimated H-index: 7
(UU: Utrecht University)
IgG4 related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, but so far no reports of bone marrow involvement have been published. Here we present a case of a 76-year-old male with IgG4 related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storif...
Published on May 9, 2019in Childs Nervous System 1.33
Aruna Nambirajan4
Estimated H-index: 4
,
M. Chand Sharma + 3 AuthorsVivek Suri1
Estimated H-index: 1
Background: IgG4-related disease is an autoimmune process that presents with tumefactive lesions characterized by storiform fibrosis, a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, obliterative phlebitis, and often elevated serum IgG4 levels. Central nervous system IgG4-related disease is very rare and usually occurs in the form of hypertrophic pachymeningitis or hypophysitis. Presentation as a large solitary meningioma-like mass with overlying hyperostosis in a young adult has...
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 Wochenschrift 0.64
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 Jun 30, 2019in The Neurohospitalist
Angela M. Parsons1
Estimated H-index: 1
(Mayo Clinic),
Fawad Aslam2
Estimated H-index: 2
(Mayo Clinic)
+ 2 AuthorsBrent P. Goodman10
Estimated H-index: 10
(Mayo Clinic)
Published on May 4, 2019in Scandinavian Journal of Rheumatology 2.71
Toru Sakairi14
Estimated H-index: 14
(Gunma University),
Noriyuki Sakurai11
Estimated H-index: 11
+ 5 AuthorsK. Hiromura5
Estimated H-index: 5
(Gunma University)
Objective: We aimed to describe the clinical characteristics and treatment course of hypertrophic pachymeningitis (HPM) in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods: We retrospectively analysed 15 patients (11 men and four women). HPM was diagnosed based on thickening and enhancing of the brain and/or spinal dura mater on gadolinium-enhanced magnetic resonance imaging (MRI) T1 sequence.Results: The median age at HPM onset was 60 years. Headache ...
Published on May 1, 2019in Journal of Neuroimmunology 2.83
Xu Zhang1
Estimated H-index: 1
(Kyushu University),
Takayuki Fujii2
Estimated H-index: 2
(Kyushu University)
+ 6 AuthorsJun-ichi Kira53
Estimated H-index: 53
(Kyushu University)
Abstract Hypertrophic pachymeningitis (HP) is a rare neurologic disease causing inflammatory fibrous thickening of the brain and spinal dura mater. We investigated the cerebrospinal fluid cytokine profile of HP by measuring 28 cytokines/chemokines/growth factors with a multiplexed fluorescent immunoassay in 8 patients with HP (6 idiopathic, 1 IgG4-related, 1 anti-neutrophil cytoplasmic antibody-related), and 11 with other non-inflammatory neurologic diseases (OND). Interleukin (IL)-4, IL-5, IL-9...
Published on May 1, 2019in Seminars in Arthritis and Rheumatism 5.07
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...