Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease?
Both multifocal fibrosclerosis and hypertrophic pachymeningitis are rare disorders of unknown etiology, characterised by chronic inflammation leading to dense fibrosis. There have been several reports of multifocal fibrosclerosis with hypertrophic pachymeningitis. Autoimmune pancreatitis is frequently associated with various extrapancreatic lesions, their pathological similarities such as dense inflammatory fibrosis with lymphoplasmacytic infiltration strongly suggests a close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Recently, autoimmune pancreatitis including these systemic fibrosing disorders may be classified as IgG4-related systemic disease. However, the relationship between HP and IgG4-related systemic disease is still uncertain. We performed immunohistochemical examinations in autopsy specimens from a patients with HP. Histological findings can be summarized as follows: sever interstitial fibrosis and diffuse inflammatory cells infiltration, presenting nonspecific inflammatory changes. Immunohistochemically, diffuse infiltrates in the dura consisted predominantly of UCHL-1 positive T or L-26 positive B lymphocytes. Many IgG4 positive plasma cells were also infiltrated. To our knowledge, this may be the first report which showed IgG4 positive plasma cells infiltration in the dura in a patient with HP. It is postulated that HP may be a dural lesion of IgG4-related systemic disease.
Available from: Hiroshi Nawashiro
- "IgG4-related intracranial hypertrophic pachymeningitis is extremely rare. To the best of our knowledge, only 10 cases have been reported in the literature, including the present case3,5,6,7,8,9,11). A summary of the cases is shown in Table 1. "
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ABSTRACT: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Recently, the possibility that IgG4-related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. We herein report the tenth case of IgG4-related intracranial hypertrophic pachymeningitis and review the previous literature. A 45-year-old male presented with left-sided focal seizures with generalization. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. The surgically resected specimens exhibited the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells. The ratio of IgG4-positive plasma cells to the overall IgG-positive cells was 45% in the area containing the highest infiltration of plasma cells. On the basis of the above findings, IgG4-related sclerosing disease arising from the dura mater was suspected. IgG4-related sclerosing disease should be added to the pachymeningitis spectrum.
Available from: Prabal Deb
- "Rossi et al. demonstrated fibrosis and prominent CD4+ T-cell inflammatory infiltrate on dural biopsy in HP, suggesting a probable pathogenetic role for cell-mediated immunity. Riku et al. have shown that HP may be a dural lesion of IgG4-related systemic disease. Tolosa-Hunt Syndrome (THS) may be a focal manifestation of pachymeningitis involving the walls of cavernous sinuses, and IHCPM may be a localized manifestation of multifocal fibrosclerosis (MF). "
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ABSTRACT: Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier.
Available from: Ryosuke Tomio
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ABSTRACT: We report the first documented case of IgG4-related inflammatory pseudotumours (IPTs) along the bilateral oculomotor nerves. A man in his 60s complained of decreased vision. He exhibited bilateral optic nerve atrophy without any extraocular movement deficits. MRI revealed enhanced masses that reached from the bilateral cavernous sinus to within the bilateral orbits. The tumours extended along the lines of the bilateral oculomotor nerves. The patient's serum level of IgG4 was high, 147 mg/dl. A biopsy specimen showed inflammatory cell-rich lesions against a collagenous stroma. Immunostaining revealed infiltration of CD138-positive plasma cells, which were mainly IgG and IgG4 positive. The IgG4/IgG ratio was greater than 0.4. These factors led us to a diagnosis of IgG4-related IPTs. Oral administration of prednisolone (30 mg/day) was started 3 months after the operation and continued for 6 months with gradual tapering. The tumour was significantly reduced by prednisolone.
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