Article

[Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease?].

Department of Neurology, Social Insurance Chukyo Hospital.
Rinsho shinkeigaku = Clinical neurology 09/2009; 49(9):594-6.
Source: PubMed

ABSTRACT 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.

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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.
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