Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis.
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.
['Jang|Yoonhyuk|Y|', 'Lee|Soon Tae|ST|', 'Jung|Keun Hwa|KH|', 'Chu|Kon|K|', 'Lee|Sang Kun|SK|']
rituximab idiopathic hypertrophic pachymeningitis steroid-refractory hypertrophic pachymeningitis