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Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis

View Article: PubMed Central - PubMed

ABSTRACT

Background and purpose: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX).

Methods: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m2). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI.

Results: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients.

Conclusions: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.

No MeSH data available.


Related in: MedlinePlus

Gadolinium-enhanced T1-weighted axial and coronal views and a T2-weighted FLAIR axial view in patient 1. A: Postcontrast T1-weighted MRI showed extensive thickening and enhancement of the dura mater along the left frontal parietal convexities. T2-weighted FLAIR MRI revealed diffuse cortical edema under the thickened dura mater. B: After treatment with corticosteroid, brain MRI disclosed marked decreases in dural thickening and cortical swelling. C: After 7 months of corticosteroid treatment, aggravation of dural thickening and cortical swelling were prominent. D: After treatment with rituximab, 3-month follow-up MRI showed normal cerebral dura mater and cortex. FLAIR: fluid attenuated inversion recovery.
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Figure 1: Gadolinium-enhanced T1-weighted axial and coronal views and a T2-weighted FLAIR axial view in patient 1. A: Postcontrast T1-weighted MRI showed extensive thickening and enhancement of the dura mater along the left frontal parietal convexities. T2-weighted FLAIR MRI revealed diffuse cortical edema under the thickened dura mater. B: After treatment with corticosteroid, brain MRI disclosed marked decreases in dural thickening and cortical swelling. C: After 7 months of corticosteroid treatment, aggravation of dural thickening and cortical swelling were prominent. D: After treatment with rituximab, 3-month follow-up MRI showed normal cerebral dura mater and cortex. FLAIR: fluid attenuated inversion recovery.

Mentions: A 22-year-old woman developed paresthesia in the distal extremities. The condition began insidiously, and the patient experienced a transient severe tingling sensation on the right arm for 2 hours on the day prior to her emergency room (ER) visit. She presented at the ER with generalized clonic seizure, and MRI revealed pachymeningeal and leptomeningeal enhancement in the left frontal area (Fig. 1A). A brain biopsy showed fibrotic tissue with lymphoplasmacytic infiltration, showing focal positivity of IgG4 immunostaining (IgG4/IgG ratio <0.1) consistent with pachymeningitis (Fig. 2).


Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis
Gadolinium-enhanced T1-weighted axial and coronal views and a T2-weighted FLAIR axial view in patient 1. A: Postcontrast T1-weighted MRI showed extensive thickening and enhancement of the dura mater along the left frontal parietal convexities. T2-weighted FLAIR MRI revealed diffuse cortical edema under the thickened dura mater. B: After treatment with corticosteroid, brain MRI disclosed marked decreases in dural thickening and cortical swelling. C: After 7 months of corticosteroid treatment, aggravation of dural thickening and cortical swelling were prominent. D: After treatment with rituximab, 3-month follow-up MRI showed normal cerebral dura mater and cortex. FLAIR: fluid attenuated inversion recovery.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5392457&req=5

Figure 1: Gadolinium-enhanced T1-weighted axial and coronal views and a T2-weighted FLAIR axial view in patient 1. A: Postcontrast T1-weighted MRI showed extensive thickening and enhancement of the dura mater along the left frontal parietal convexities. T2-weighted FLAIR MRI revealed diffuse cortical edema under the thickened dura mater. B: After treatment with corticosteroid, brain MRI disclosed marked decreases in dural thickening and cortical swelling. C: After 7 months of corticosteroid treatment, aggravation of dural thickening and cortical swelling were prominent. D: After treatment with rituximab, 3-month follow-up MRI showed normal cerebral dura mater and cortex. FLAIR: fluid attenuated inversion recovery.
Mentions: A 22-year-old woman developed paresthesia in the distal extremities. The condition began insidiously, and the patient experienced a transient severe tingling sensation on the right arm for 2 hours on the day prior to her emergency room (ER) visit. She presented at the ER with generalized clonic seizure, and MRI revealed pachymeningeal and leptomeningeal enhancement in the left frontal area (Fig. 1A). A brain biopsy showed fibrotic tissue with lymphoplasmacytic infiltration, showing focal positivity of IgG4 immunostaining (IgG4/IgG ratio <0.1) consistent with pachymeningitis (Fig. 2).

View Article: PubMed Central - PubMed

ABSTRACT

Background and purpose: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX).

Methods: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m2). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI.

Results: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients.

Conclusions: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.

No MeSH data available.


Related in: MedlinePlus