What question did this study set out to answer?

To determine whether IgG4-related disease is a frequent underlying etiology in cases historically classified as idiopathic hypertrophic pachymeningitis.

June 21, 2013MedicineOpen Access

IgG4-Related Disease and Hypertrophic Pachymeningitis

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Authors

ZWZachary S. WallaceAmgen (United States)MCMollie N. CarruthersResearch Canada AKArezou KhosroshahiEmory University

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Implication

Case series reveals IgG4-related disease in noninfectious hypertrophic pachymeningitis, highlighting the necessity of tissue biopsy for accurate diagnosis.

Key Points

To determine whether IgG4-related disease is a frequent underlying etiology in cases historically classified as idiopathic hypertrophic pachymeningitis.
Reviewed pathology specimens from 14 cases of noninfectious hypertrophic pachymeningitis collected over a 25-year period at a single institution.
Re-analyzed hematoxylin and eosin slides and performed immunostaining to quantify IgG4+ plasma cell infiltration using consensus guidelines.
IgG4-related disease was diagnosed in 4 of 6 cases (66.6%) previously classified as idiopathic, accounting for 29% (4 of 14) of all reviewed cases.
Alternative etiologies included granulomatosis with polyangiitis (3 cases), lymphoma (2 cases), and 1 case each of rheumatoid arthritis, giant cell arteritis, and sarcoidosis, while 2 cases remained undifferentiated.
Clinical evaluations, serologic testing, cerebrospinal fluid analysis, and neuroimaging consistently failed to identify the underlying cause without biopsy.

Cite This Study

Wallace et al. (2013) studied this question.

synapsesocial.com/papers/6a00a6cf831589f3542de082 https://doi.org/10.1097/md.0b013e31829cce35

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Implication

Case series reveals IgG4-related disease in noninfectious hypertrophic pachymeningitis, highlighting the necessity of tissue biopsy for accurate diagnosis.

IgG4-Related Disease and Hypertrophic Pachymeningitis

Discussion

Member takes

Implication

Key Points

Cite This Study

IgG4-Related Disease and Hypertrophic Pachymeningitis

Discussion

Member takes

Implication

Key Points

Cite This Study

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