A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.

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Immunoglobulin G4-related hypertrophic pachymeningitis

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000568 ◽

2019 ◽

Vol 6 (4) ◽

pp. e568 ◽

Cited By ~ 6

Author(s):

Michaël Levraut ◽

Mikaël Cohen ◽

Saskia Bresch ◽

Caroline Giordana ◽

Fanny Burel-Vandenbos ◽

...

Keyword(s):

Steroid Therapy ◽

Case Reports ◽

Treatment Algorithm ◽

Elevated Serum ◽

Case Series ◽

Hypertrophic Pachymeningitis ◽

Immunoglobulin G4 ◽

Systemic Involvement ◽

Meningeal Involvement ◽

Serum Igg4

ObjectiveMeningeal 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).MethodsTwo 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 case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

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SAT0337 Hypertrophic Pachymeningitis Associated with ANCA Vasculitis; A Case Series of 16 Patients

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2016-eular.1544 ◽

2016 ◽

Vol 75 (Suppl 2) ◽

pp. 789.2-789

Author(s):

T. Sakairi ◽

K. Hiromura ◽

N. Sakurai ◽

H. Hamatani ◽

H. Ikeuchi ◽

...

Keyword(s):

Case Series ◽

Hypertrophic Pachymeningitis ◽

Anca Vasculitis

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Idiopathic orbital inflammation with extraorbital extension: case series and review

Eye ◽

10.1038/sj.eye.6701780 ◽

2005 ◽

Vol 20 (1) ◽

pp. 107-113 ◽

Cited By ~ 25

Author(s):

B Zborowska ◽

R Ghabrial ◽

D Selva ◽

P McCluskey

Keyword(s):

Case Series ◽

Orbital Inflammation ◽

Idiopathic Orbital Inflammation

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Clinical Aspects of IgG4-Related Orbital Inflammation in a Case Series of Ocular Adnexal Lymphoproliferative Disorders

International Journal of Rheumatology ◽

10.1155/2012/635473 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 43

Author(s):

Masayuki Takahira ◽

Yoshiaki Ozawa ◽

Mitsuhiro Kawano ◽

Yoh Zen ◽

Shoko Hamaoka ◽

...

Keyword(s):

Malt Lymphoma ◽

Case Series ◽

B Cell Lymphoma ◽

Lymphoid Hyperplasia ◽

Lymphoproliferative Disorders ◽

Clinical Aspects ◽

Lacrimal Glands ◽

Malignant Lymphomas ◽

Orbital Inflammation ◽

Adnexal Tumors

The most frequent ocular adnexal tumors and simulating lesions are lymphoproliferative disorders (LPDs), including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration. IgG4-related orbital inflammation (IgG4-ROI) often involves lacrimal glands and other orbital tissues and is an important differential diagnosis. The present study evaluated clinical aspects of IgG4-ROI in a case series of orbital LPD. Sixty-two consecutive cases of orbital LPD, pathologically diagnosed from November, 2004, through March, 2011, were investigated. Histological types were 22 cases with MALT lymphoma, 11 cases with diffuse large B-cell lymphoma (DLBCL), 3 cases with other malignant lymphomas, 16 cases with IgG4-ROI, and 10 cases with non-IgG4-ROI. Ages of the IgG4-ROI group (56±10?yrs) were significantly lower than the MALT lymphoma (71±12?yrs) and DLBCL (75±14?yrs) groups. Orbital lesions other than lacrimal glands were present in six cases including extraocular muscle swelling, mass lesions surrounding the optic nerve, and supraorbital and infraorbital nerves enlargements. Although none of the malignant lymphomas were related to IgG4, previous evidence suggested that malignant lymphomas can arise from IgG4-ROI. Based on this study (26%) and another report (33%), it is likely that nearly a quarter of orbital LPD are IgG4-ROI.

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