Metachronous Involvement, Diagnostic Imprecision of Serum Immunoglobulin G4 Levels, and Discordance Between Clinical and Radiological Findings in Immunoglobulin G4–Related Pachymeningitis

Waheed, Waqar; Skidd, Philip; Borden, Neil M.; Gibson, Pamela Church; Babi, Mohamed Ali; Tandan, Rup

doi:10.1097/rhu.0000000000000488

Cited by 5 publications

(4 citation statements)

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“…Three steroid-refractory HP patients treated with RTX for 4 weeks showed clinical improvements and exhibited prominent decreases in dural thickness (61). Thus, RTX has been suggested to be a second-line therapy for steroid-refractory HP, especially for IgG4-RD (52)(53)(54)(55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65).…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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Aims: To investigate the causes, clinical characteristics, imaging features, and therapeutic implications of hypertrophic pachymeningitis (HP) in a southern Chinese population. Methods: We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, and neuroimaging results were evaluated in all HP patients. Results: The mean age at onset was 50 ± 12 years. The most common diagnosis was idiopathic HP (67%), followed by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). Headache was the most common symptom. The most frequently changed laboratory finding was elevated erythrocyte sedimentation rate (ESR). Imaging was characterized by cerebral or spinal dura mater enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal, and occipital lobes for ANCA-related HP; and posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment was applied in most cases. Conclusions: The etiology of HP varied among patients, with idiopathic HP being the most common. MRI showed enhancement of the dura mater, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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“…Three steroid-refractory HP patients treated with RTX for four weeks showed clinical improvements and exhibited prominent decreases in dural thickness [55]. Thus, RTX has been suggested to be a second-line therapy for steroid-refractory HP, especially for IgG4-RD [56][57][58][59][60].…”

Section: Treatmentmentioning

confidence: 99%

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Xiao

Feng

2020

Preprint

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Background Hypertrophic pachymeningitis (HP) is a fibrotic disorder featuring a thickening of the dura matter. Most HP studies were from Caucasian population and only a few studies of HP are available in China. In this study, we investigated the causes, clinical and imaging features and therapeutic implications of HP in a southern Chinese population.Methods We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, neuroimaging results, and clinical course were evaluated in all HP patients.Results The mean age at onset was 50±12 years. The most common diagnosis was idiopathic HP (67%), followed by ANCA-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). The main clinical manifestations were headache and cranial nerve deficits. The most frequently changed laboratory finding was elevated ESR. Imaging was characterized by cerebral or spinal dura matter enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal and occipital lobes for ANCA-related HP; and the posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment were applied in most cases.Conclusions HP was diagnosed based on clinical manifestations, imaging and laboratory results. Etiology varied among patients, with idiopathic HP being the most common. MRI with contrast showed enhancement of the dura matter, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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“…IgG4‐RHP seems to have a similar course to other IgG4‐RD patients. Most IgG4‐RHP respond to corticosteroids, but rapid recurrence of symptoms of HP is reported in different case series necessitating a corticosteroid‐sparing agent such as methotrexate, azathioprine, rituximab, and rarely mycophenolate mofetil 15–17 . As we considered meningeal disease was an urgent condition, all of our cases were treated with pulse corticosteroids, showing clinical improvement.…”

Section: Age At Onset/sex Symptoms Serum Igg4 At Diagnosis (Mg/dl) An...mentioning

confidence: 87%

“…13 A case-control study, suggested that CSF IgG4 quantification could be an alternative diagnostic tool with a cut off value of 2.27 mg/dL. [15][16][17] As we considered meningeal disease was an urgent condition, all of our cases were treated with pulse corticosteroids, showing clinical improvement. Four of them received rituximab, and 1 patient received azathioprine.…”

Section: Mri Dural Thickening Locationmentioning

confidence: 98%

IgG4‐related pachymeningitis—Long term follow up and outcome of six patients

et al. 2023

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ObjectiveOur understanding of IgG4‐RD and pachymeningitis has grown substantially, but the optimal approach for diagnosis, management, and long‐term outcomes is still an area of uncertainty.MethodsHUVAC is a database for IgG4‐RD patients, this database was retrospectively evaluated for pachymeningeal disease. Demographic, clinical, serological, imaging, histopathological data, and treatment details were re‐interpreted in patients with pachymeningitis.ResultsAmong 97 patients with IgG4‐RD, 6 (6.2%) had pachymeningitis. None of these patients had extracranial features, and also, in most of the patients, serum IgG4 levels were normal. Tentorium cerebelli and transverse sinus dura were the most commonly involved in the posterior fossa. During 18 months of median follow‐up on steroid+‐rituximab, none of them relapsed as pachymeningitis.ConclusionOur patients were mainly older males with sole neurological involvement. Non‐specific headache was the most common manifestation, and serum IgG4 levels were not useful for diagnosis. Typical radiology and tentorial thickening should suggest IgG4‐RD and prompt an early biopsy. Moreover, accompanying hypophysitis could also be a clue. With steroids+ rituximab treatment, no relapse related to meningeal involvement was seen in long‐term follow‐up.

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Metachronous Involvement, Diagnostic Imprecision of Serum Immunoglobulin G4 Levels, and Discordance Between Clinical and Radiological Findings in Immunoglobulin G4–Related Pachymeningitis

Cited by 5 publications

References 30 publications

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

IgG4‐related pachymeningitis—Long term follow up and outcome of six patients

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