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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.


Gadolinium-enhanced T1-weighted axial and coronal views in patient 2. A: Postcontrast T1-weighted MRI showed an ill-defined enhancement along the anterior temporal area, superior orbital fissure, and parapharyngeal space. B: After 7 months of corticosteroid and azathioprine combined treatment, extensive dural thickening and enhancement at the right tentorium and posterior fossa appeared. C: After treatment with rituximab, the 4-month follow-up MRI showed marked decreases in dural thickening and enhancement.
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Figure 3: Gadolinium-enhanced T1-weighted axial and coronal views in patient 2. A: Postcontrast T1-weighted MRI showed an ill-defined enhancement along the anterior temporal area, superior orbital fissure, and parapharyngeal space. B: After 7 months of corticosteroid and azathioprine combined treatment, extensive dural thickening and enhancement at the right tentorium and posterior fossa appeared. C: After treatment with rituximab, the 4-month follow-up MRI showed marked decreases in dural thickening and enhancement.

Mentions: A 54-year-old man suffered a severe occipital headache with multiple cranial nerve palsies on the right side, manifesting as hoarseness, dysphagia, uvular deviation, tongue deviation, and shoulder elevation weakness. Brain T1-weighted MRI with gadolinium revealed an ill-defined enhancement along the right anterior temporal area, superior orbital fissure, and parapharyngeal space (Fig. 3A). A biopsy at the right Rosenm├╝ller fossa demonstrated chronic inflammation with plasma cell infiltration, showing focal positivity in IgG4 immunostaining (IgG4/IgG ratio<0.3) (Fig. 4).


Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis
Gadolinium-enhanced T1-weighted axial and coronal views in patient 2. A: Postcontrast T1-weighted MRI showed an ill-defined enhancement along the anterior temporal area, superior orbital fissure, and parapharyngeal space. B: After 7 months of corticosteroid and azathioprine combined treatment, extensive dural thickening and enhancement at the right tentorium and posterior fossa appeared. C: After treatment with rituximab, the 4-month follow-up MRI showed marked decreases in dural thickening and enhancement.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Gadolinium-enhanced T1-weighted axial and coronal views in patient 2. A: Postcontrast T1-weighted MRI showed an ill-defined enhancement along the anterior temporal area, superior orbital fissure, and parapharyngeal space. B: After 7 months of corticosteroid and azathioprine combined treatment, extensive dural thickening and enhancement at the right tentorium and posterior fossa appeared. C: After treatment with rituximab, the 4-month follow-up MRI showed marked decreases in dural thickening and enhancement.
Mentions: A 54-year-old man suffered a severe occipital headache with multiple cranial nerve palsies on the right side, manifesting as hoarseness, dysphagia, uvular deviation, tongue deviation, and shoulder elevation weakness. Brain T1-weighted MRI with gadolinium revealed an ill-defined enhancement along the right anterior temporal area, superior orbital fissure, and parapharyngeal space (Fig. 3A). A biopsy at the right Rosenm├╝ller fossa demonstrated chronic inflammation with plasma cell infiltration, showing focal positivity in IgG4 immunostaining (IgG4/IgG ratio<0.3) (Fig. 4).

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.