IgG4-related Disease Manifesting as Pachymeningitis and Aortitis

Lipton, Sarah; Warren, Garth; Pollock, Jeffrey M.; Schwab, Pascale

doi:10.3899/jrheum.121545

Cited by 10 publications

(6 citation statements)

References 10 publications

(7 reference statements)

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“…MTX was specifically assessed in a recent retrospective small study by Della-Torre et al and was reported to be effective in maintaining clinical and serological responses induced by glucocorticoids, enabling a substantial reduction in the overall dose of steroids in all patients and their withdrawal in six of ten patients within 6–12 months [161]. None of these patients had neurological disease secondary to IgG4-RD, and it should be noted that others have reported conflicting results of SSA use (predominantly MTX) in neurological disease caused by IgG4-RD [15, 63, 64, 69–71, 83, 118, 162, 163]. …”

Section: Treatmentmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-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 IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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Section: Treatmentmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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“…However, in some cases accompanying periaortitis, hypophysitis, dacryoadenitis, lung involvement has been described in the literature. [9][10][11] Pachymeningitis was the sole manifestation in our cases except for one patient in whom sinusitis, diabetes insipidus and hypophysitis were also identified, also later relapsed in the peritoneum.…”

Section: Mri Dural Thickening Locationmentioning

confidence: 77%

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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“…Ustanovljen mu je hipertrofični pahimeningitis u stražnjoj lubanjskoj jami i abdominalni periaortitis 15 . Drugi je slučaj iz 2013., a prikazuje 57-godišnju ženu koja se prezentirala glavoboljom desno temporalno unazad tri godine, gubitkom težine i umorom 16 . Uz to je imala šumove nad desnom karotidom i brahijalnom arterijom te nad femoralnom arterijom bilateralno 16 .…”

Section: Raspravaunclassified

“…Drugi je slučaj iz 2013., a prikazuje 57-godišnju ženu koja se prezentirala glavoboljom desno temporalno unazad tri godine, gubitkom težine i umorom 16 . Uz to je imala šumove nad desnom karotidom i brahijalnom arterijom te nad femoralnom arterijom bilateralno 16 . Bila je anemična, s ubrzanim SE i CRP-om unutar referentnih vrijednosti.…”

Section: Raspravaunclassified

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Hipertrofični pahimeningitis i aortitis kao manifestacije bolesti povezane s imunoglobulinom G4 i uspješno liječene rituksimabom

Šević

Javoran

Anić

et al. 2021

Med. Flum. (Online)

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Cilj: Prikazati pacijenta s bolesti povezanom s imunoglobulinom G4 (engl. immunoglobulin G4-related disease; IgG4-RD) te dijagnostičke izazove vezane uz ovu bolest, kao i terapijske mogućnosti s naglaskom na liječenje rituksimabom. Prikaz slučaja: Šezdesetogodišnji pacijent primljen je na hospitalnu obradu nakon pregleda u gastroenterološkoj ambulanti u koju je zaprimljen zbog neredovite stolice, slabosti i gubitka 20 kg unutar dva mjeseca. U statusu je ustanovljena lezija desnog ličnog živca, ptoza, lagoftalmus i otežano gutanje. Obradom su potvrđene lezije desnog oculomotoriusa, facialisa i trigeminusa, a otorinolaringološkim pregledom utvrđena je pareza desne glasnice. Laboratorijski nalazi pokazali su anemiju i povišene upalne pokazatelje. Ultrazvukom otkrivena lezija jetre definirana je kao hemangiom. Kompjutorizirana tomografija (engl. computed tomography; CT) i magnetska rezonancija (MR) mozga pokazale su hipertrofični pahimeningitis u području srednje i stražnje lubanjske jame, uz desni tentorij, kavernozni sinus, Meckelovu jamu i gornju orbitalnu fisuru. CT aortografija pokazala je aortitis infrarenalne aorte. Serumska razina IgG4 protutijela bila je povišena te su zadovoljeni kriteriji za moguću dijagnozu IgG4-RD i započeta je terapija metilprednizolonom. Iduće je godine ponovno hospitaliziran radi reevaluacije bolesti. MR mozga pokazao je dobru regresiju hipertrofičnog tkiva dure, ali CT aortografija nepotpunu regresiju bolesti te su upalni pokazatelji i dalje bili visoki. Uvedena je terapija rituksimabom koja je dovela do remisije. Zaključak: IgG4-RD je rijetka i složena bolest koja često oponaša druge i predstavlja velik dijagnostički izazov, stoga bi poznavanje ove bolesti uvelike pridonijelo njezinom ranom otkrivanju i boljim terapijskim ishodima. Iako su glukokortikoidi standardna terapija, često ne daju željene rezultate, a inovativna terapija rituksimabom pokazuje odličnu učinkovitost.

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IgG4-related Disease Manifesting as Pachymeningitis and Aortitis

Cited by 10 publications

References 10 publications

Neurological Manifestations of IgG4-Related Disease

Neurological Manifestations of IgG4-Related Disease

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

Hipertrofični pahimeningitis i aortitis kao manifestacije bolesti povezane s imunoglobulinom G4 i uspješno liječene rituksimabom

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