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IgG4-related pachymeningitis-Long term follow up and outcome of six patients
Gozde Kubra Yardimci1,2, Doruk Arslan3, Berrin Babaoğlu4
1Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey.
Insights
Immunoglobulin G4-related disease (IgG4-RD) with pachymeningitis primarily affects older males with neurological symptoms. Steroids plus rituximab offer effective long-term management without relapse.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a complex fibroinflammatory condition with increasing recognition.
- Pachymeningitis, inflammation of the dura mater, can be a manifestation of IgG4-RD, but diagnostic and management strategies remain uncertain.
Purpose of the Study:
- To evaluate the characteristics, diagnosis, and treatment outcomes of IgG4-RD patients with pachymeningitis.
- To identify key clinical, imaging, and serological features aiding in the diagnosis of IgG4-RD-associated pachymeningitis.
Main Methods:
- Retrospective analysis of the HUVAC database for patients diagnosed with IgG4-related disease and pachymeningitis.
- Detailed review of demographic, clinical, serological, imaging, and histopathological data, along with treatment information.
Main Results:
- Six out of 97 (6.2%) IgG4-RD patients presented with pachymeningitis, predominantly older males with isolated neurological involvement.
- Commonly affected sites included the tentorium cerebelli and transverse sinus dura; serum IgG4 levels were often normal, and extracranial manifestations were absent.
- Median follow-up of 18 months with steroid +/- rituximab treatment showed no relapses of pachymeningitis.
Conclusions:
- IgG4-RD-associated pachymeningitis often presents with non-specific headaches and normal serum IgG4 levels, making diagnosis challenging.
- Radiological findings like tentorial thickening, alongside potential hypophysitis, should prompt consideration of IgG4-RD and early biopsy.
- Combination therapy with steroids and rituximab demonstrated efficacy in preventing relapse of meningeal involvement in the long term.
Objective:
Our 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.
Methods:
HUVAC 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.
Results:
Among 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.
Conclusion:
Our 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.
