IgG4-related disease, which is newly recognized, is characterized by lymphoplasmacytic infiltration with increased IgG4-secreting plasma cells. Although a favorable response to steroids has previously been reported, the durations of follow-up to confirm the long-term benefits and clinical courses were limited. We describe long-term favorable response of oral methotrexate in a patient with IgG4-related pachymeningitis who was resistant to steroid therapy.
A patient in his mid-60s with pathologically proven IgG4-related pachymeningitis who was resistant to steroid therapy and experienced an exacerbation of symptoms 4 times is described. Low-dose oral methotrexate induced significant clinical and radiological improvement, with sustained remission of the disease over 2 years without complications.
Conclusions and Relevance
The long-term favorable response to oral methotrexate in the current patient suggests that methotrexate is a useful alternative treatment option in patients with IgG4-related pachymeningitis who are resistant to steroid therapy or who experience adverse effects from steroids.
A, Orbital MRI showed dural thickening and enhancement (arrowheads) in the bilateral sphenoid and occipital dura, suggestive of chronic pachymeningitis. B, After the third attack, brain MRI disclosed extensive dural thickening and enhancement (arrowheads) extending into the internal auditory canal and frontotemporal dura, more severe than before. C, After 1 year of treatment, orbital MRI showed marked decrease of dural thickening and enhancement (arrowheads). D, After 2 years of treatment, orbital MRI revealed the further decrease of residual dural thickening (arrowheads).
A, Biopsy of dura revealed markedly thickened dura by lamellated collagen fiber deposition (Masson trichrome, original magnification ×12.5). B, Lymphoid follicles with prominent germinal centers (arrowhead) are scattered (hematoxylin-eosin, ×40). C and D, Perivascular infiltration of lymphoplasmacytic cells (C) (hematoxylin-eosin, ×200) and many IgG4-positive plasma cells among infiltrated inflammatory cells (D) (>50 IgG4-positive cells per high-power field, arrowhead), consistent with a diagnosis of IgG4-related pachymeningitis (original magnification ×200). E and F, Immunostaining with CD3 (E) (original magnification ×100) and CD20 (F) (original magnification ×40) showed infiltration of T and B cells (arrowheads) beside plasma cells in the dura.
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