Immunoglobulin G4–Related Hypertrophic Pachymeningitis of the Spine: A Case Report and Systematic Review of the Literature

Sbeih, Ibrahim; Darwazeh, Rami; Shehadeh, Mahmoud; Al-Kanash, Rasha; Abu-Farsakh, Hussam; Sbeih, Aseel

doi:10.1016/j.wneu.2020.07.227

Cited by 6 publications

(8 citation statements)

References 57 publications

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“…Obliterative phlebitis is rare in cases of IgG4-RSP ( 12 , 23 , 38 ). The present study revealed that 22 patients had IgG4/IgG+ of >40% ( 8 – 10 , 12 , 13 , 22 , 23 , 25 , 29 , 34 , 35 , 38 , 40 , 42 – 44 ), and 33 patients had IgG4+ plasma cells of >10/HPF ( 8 – 12 , 15 , 19 , 22 – 32 , 34 – 38 , 40 , 42 , 44 ). Although there is no international consensus criteria for the pathological diagnosis of IgG4-RSP, dura mater biopsy is presently the best-supported approach.…”

Section: Discussionsupporting

confidence: 48%

“…The most common symptoms in IgG4-RSP patients are fatigue and motor sensory dysfunction of the extremities, which occur in varying degrees. Among the 45 cases obtained for the present study, 22 patients experienced symptoms that gradually progressed over days to months, and these symptoms presented as severe myeloid symptoms ( 8 , 10 , 11 , 17 , 19 , 20 , 22 , 23 , 27 , 29 – 31 , 33 – 35 , 39 , 41 , 43 , 44 ). Therefore, the progressive exacerbation of neurological symptoms may be associated with a secondary spinal cord injury caused by chronic spinal cord ischemia, and the acute exacerbation of inflammatory cascade activation ( 49 , 50 ).…”

Section: Discussionmentioning

confidence: 83%

“…Furthermore, most IgG4-RD patients have elevated serum IgG4 (>135 mg/dL), but there is no significant reference range of IgG levels. Among the 22 cases of IgG4-RSP reported by Sbeih et al ( 10 ), 13 patients had serum IgG4 of >135 mg/dL. These authors considered that pre-treatment serum IgG4 levels may be used as a marker for the diagnosis of IgG4-RSP.…”

Section: Discussionmentioning

confidence: 98%

“…IgG4-RSP can cause spinal compression that mimics metastatic disease or hemorrhage, and this condition is frequently misdiagnosed. The infiltration of various plasma cells in histopathological sections is a characteristic of IgG4-RSP ( 10 ). The present study presents three cases of IgG4-RSP, and discusses the clinical symptoms, radiological findings, treatment and prognosis.…”

Section: Introductionmentioning

confidence: 99%

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Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

et al. 2023

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PurposeHypertrophic pachymeningitis associated with immunoglobulin G4-related disease (IgG4-RD) has been rarely reported, and there is little information and no clear consensus on the management of IgG4-related spinal pachymeningitis (IgG4-RSP). The present study described its possible clinical features, including the symptoms, imaging, treatment and prognosis of patients with IgG4-RSP.MethodsWe report three patients who presented with progressive neurological dysfunction due to spinal cord compression. Relevant articles were searched from the PubMed, Web of Science, and Embase databases, and the resulting literature was reviewed.ResultsThe literature review provided a summary of 45 available cases, which included three cases from our center. Progressive worsening of neurological impairment was observed in 22 patients (48.9%). The lesions involved the thoracic spine (n=28, 62.2%), cervical spine (n=26, 57.8%), lumbar spine (n=9, 20.0%), and sacral spine (n=1, 2.2%). Furthermore, the lesions were located in the dura mater (n=18, 40.0%), epidural space (n=17, 37.8%), intradural-extramedullary space (n=9, 20.0%), and intramedullary space (n=1, 2.2%). On magnetic resonance imaging (MRI), the lesions generally appeared as striated, fusiform, or less often lobulated oval changes, with homogeneous (n=17,44.7%) and dorsal (n=15,39.5%) patterns being the most common. Thirty-five patients had homogeneous T1 gadolinium enhancement. Early surgical decompression, corticosteroid treatment, and steroid-sparing agents offered significant therapeutic advantages. A good therapeutic response to disease recurrence was observed with the medication.ConclusionThe number of reported cases of IgG4-RSP remains limited, and patients often have progressive worsening of their neurological symptoms. The features of masses identified on the MRI should be considered. The prognosis was better with decompression surgery combined with immunosuppressive therapy. Long-term corticosteroid treatment and steroid-sparing agent maintenance therapy should be ensured. A systemic examination is recommended to identify the presence of other pathologies.

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Section: Discussionsupporting

confidence: 48%

Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

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Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

et al. 2023

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“…Some skull base locations of IgG4-RD documented in the literature include the meninges, sphenoid bone, sphenoid and maxillary sinus, orbit, clivus, internal auditory canal, brain parenchyma, infratemporal fossa, pterygopalatine fossa, suprasellar region, and temporal bone (9)(10)(11)(12)(13). The most recent guidelines from the American College of Rheumatologists and European League Against Rheumatism Guidelines (5) have shifted toward a point-based system where IgG4-RD diagnosis is based on a spectrum of classic features and absence of other plausible diagnoses.…”

Section: Introductionmentioning

confidence: 99%

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

et al. 2022

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ObjectiveIgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone.Databases ReviewedOvid MEDLINE, EMBASE, Cochrane Library, and Google Scholar.MethodsWe used the following search keywords: “lgG4-RD,” “skull,” “skull base,” “cranial,” “temporal bone,” “inner ear.” We additionally manually searched the bibliographies of relevant articles. The JBI Critical Appraisal Checklist for Case Reports and Case Series was used to assess the risk of bias; because of the scarcity of the reports, data were available through limited case series and reports; thus, data synthesis was not possible.ResultsWe identified 17 studies with 22 cases with temporal bone involvement. The most common presenting symptoms were hearing loss, otalgia, and headache. The mastoid and petrous bone were the most affected anatomical areas. Both computed tomography and magnetic resonance imaging were used. Biopsies showed the characteristic lymphoplasmacytic infiltrate in all cases, with histopathology being the diagnostic modality that set the diagnosis. Most patients were treated with corticosteroids ± surgery or a combination of corticosteroids and immunosuppressants with 95.5% symptomatic response and disease control.ConclusionIgG4-RD of the temporal bone radiologically manifests as space-occupying, lytic lesions; clinically, it presents with vague otological symptoms. Diagnosis involves a thorough workup, with histopathology being crucial in setting a definite diagnosis. IgG4-RD tends to respond well to systemic corticosteroids, whereas surgery is mostly required for diagnostic purposes.

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IgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two Cases

Cler¹,

Sharifai

Baker³

et al. 2021

World Neurosurgery

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Immunoglobulin G4–Related Hypertrophic Pachymeningitis of the Spine: A Case Report and Systematic Review of the Literature

Cited by 6 publications

References 57 publications

Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

IgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two Cases

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