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An orphan disease: IgG4-related spinal pachymeningitis: report of 2 cases.

Published on Dec 1, 2016in Journal of Neurosurgery
· DOI :10.3171/2016.4.SPINE1674
Bishan Dass Radotra20
Estimated H-index: 20
,
Ashish Aggarwal5
Estimated H-index: 5
+ 2 AuthorsDebajyoti Chatterjee5
Estimated H-index: 5
Sources
Abstract
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 resulting in myeloradiculopathy (Case 1) and radiculopathy (Case 2). Imaging of spine in both cases revealed an ill-defined contrast-enhancing lesion at the lumbar level. Preoperatively, a diagnosis of spinal tumor was made, but intraoperatively no spinal tumor was found. The diagnosis was established histopathologically. The disease has no particular defining features clinically or radiologically and can mimic common spinal tumors. It is important to accurately diagnose this rare entity because of its multisystem involv...
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References9
Newest
#1Arezou Khosroshahi (Emory University)H-Index: 26
#2Zachary S. Wallace (Harvard University)H-Index: 18
Last. John H. Stone (Harvard University)H-Index: 70
view all 42 authors...
A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T....
288 CitationsSource
#1John H. StoneH-Index: 70
#2Yoh ZenH-Index: 63
Last. DeshpandeH-Index: 4
view all 3 authors...
1,323 CitationsSource
#1Kazuichi Okazaki (Kansai Medical University)H-Index: 63
#2Kazushige Uchida (Kansai Medical University)H-Index: 35
Last. Makoto Takaoka (Kansai Medical University)H-Index: 23
view all 5 authors...
Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more...
204 CitationsSource
#1Katherine M. Lindstrom (UVA: University of Virginia)H-Index: 1
#2John B. Cousar (UVA: University of Virginia)H-Index: 31
Last. M. Beatriz S. Lopes (UVA: University of Virginia)H-Index: 31
view all 3 authors...
IgG4-related disease has evolved from originally being recognized as a form of pancreatitis to encompass diseases of numerous organs including the hypophysis and one reported case of dural involvement. A search of the University of Virginia, Division of Neuropathology files for 10 years identified ten cases of unexplained lymphoplasmacytic meningeal inflammation that we then evaluated using immunohistochemical stains for IgG4 and IgG. Ten control cases including sarcoidosis (4), tuberculosis (1)...
118 CitationsSource
#1Mollie Dahlgren (Harvard University)H-Index: 2
#2Arezou Khosroshahi (Harvard University)H-Index: 26
Last. John H. Stone (Harvard University)H-Index: 70
view all 5 authors...
Objective Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with “multifocal fibrosclerosis.” IgG4-related systemic disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4-related systemic disease spectrum. Methods We searched our institution's pathology database using the terms “Riedel's,” “struma,” “thyroid,” and “fibrosis,” and identified 3 cases of Rie...
181 CitationsSource
Autoimmune pancreatitis (AIP) is a unique form of pancreatitis that is suspected to involve autoimmune mechanisms. Given that AIP responds dramatically to steroid therapy, it is important to distinguish it from pancreatic cancer. AIP may be one manifestation of IgG4-related sclerosing disease, which is a systemic disease in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs. Kamisawa and colleagues describe the clinical and pathophysiological features of AIP...
145 CitationsSource
#1John H. Stone (Harvard University)H-Index: 70
#2Arezou Khosroshahi (Harvard University)H-Index: 26
Last. James R. Stone (Harvard University)H-Index: 51
view all 6 authors...
We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4-related systemic disease, linking IgG4-related systemic disease with a newly-recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in ...
143 CitationsSource
#1Takako SaekiH-Index: 24
#2Akihiko Saito (Niigata University)H-Index: 25
Last. Fumitake Gejyo (Niigata University)H-Index: 46
view all 8 authors...
We report three elderly men with high serum IgG4 concentrations and multiple lymphoplasmacytic inflammation of the salivary glands, lymph nodes, pancreas, and renal interstitium. The infiltrating plasma cells had strong immunoreactivity for IgG4, even in patients without pancreatic lesions. These cases show that IgG4 is associated not only with autoimmune pancreatitis, but also with other systemic lymphoplasmacytic disease.
88 CitationsSource
#1Terumi KamisawaH-Index: 56
#2Nobuaki FunataH-Index: 33
Last. Hitoshi NakajimaH-Index: 6
view all 9 authors...
Background. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. Methods. To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies. Results. In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancre...
858 CitationsSource
Cited By7
Newest
#1Brian Park (UIHC: University of Iowa Hospitals and Clinics)H-Index: 4
#2Rachel D. Starks (UIHC: University of Iowa Hospitals and Clinics)H-Index: 1
Last. Brian J. Dlouhy (Roy J. and Lucille A. Carver College of Medicine)H-Index: 15
view all 5 authors...
Abstract Background The majority of the abnormalities and diseases that affect the craniovertebral junction (CVJ) have already been reported, and therefore it is exceedingly rare to identify new pathology that affects the CVJ. IgG4 related disease (IgG4-RD) is an immune mediated process treated with immunosuppressive medications. This is the first reported case of IgG4-RD affecting the CVJ. Case Description The authors report the case of a 71-year-old woman with IgG4-RD of the CVJ. She presented...
Source
IgG4-related disease (IgG4-RD) is a disorder with various clinical manifestations. Central nervous system (CNS) involvement is well recognized, with hypertrophic pachymeningitis and hypophysitis being the most common manifestations. Spinal cord involvement is an extremely rare manifestation. We present the first case of an IgG4-RD patient with spinal cord parenchymal disease and concurrent hypophysitis. We review also the current literature about CNS parenchymal involvement in the context of IgG...
Source
#1Cléa Melenotte (AMU: Aix-Marseille University)H-Index: 8
#2Julie Seguier (AMU: Aix-Marseille University)H-Index: 2
Last. Nicolas Schleinitz (AMU: Aix-Marseille University)H-Index: 28
view all 23 authors...
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...
Source
#1Stephen J. Slade (Virginia Mason Medical Center)
#2Erin M. Bauer (Virginia Mason Medical Center)
Last. Amish J. Dave (Virginia Mason Medical Center)
view all 4 authors...
Background Back pain is a leading reason for patients to seek medical attention. Although musculoskeletal causes are common, patients can also present with rarer etiologies. Case Description A 50-year-old man presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. During the next 3 months, his pain worsened, and he developed lower extremity dysesthesia and subjective weakness, despite normal neurological examination findings. Nonreveali...
Source
#1Kelly J. Bridges (OHSU: Oregon Health & Science University)H-Index: 3
#2Caitlin H. DeDeaux (OHSU: Oregon Health & Science University)H-Index: 1
Last. Khoi D. Than (OHSU: Oregon Health & Science University)H-Index: 24
view all 3 authors...
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 pre...
3 CitationsSource
#1Aruna Nambirajan (AIIMS: All India Institute of Medical Sciences)H-Index: 5
#2M. Chand Sharma (AIIMS: All India Institute of Medical Sciences)
Last. Vivek Suri (AIIMS: All India Institute of Medical Sciences)H-Index: 1
view all 6 authors...
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...
Source
#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
Last. John H. Stone (Harvard University)H-Index: 70
view all 3 authors...
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...
11 CitationsSource
1 CitationsSource
#1Bernardo BaptistaH-Index: 1
#2Alina Casian (Guy's and St Thomas' NHS Foundation Trust)H-Index: 7
Last. Claire M Rice (Southmead Hospital)H-Index: 18
view all 5 authors...
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG...
13 CitationsSource