A dural biopsy is usually essential for a definitive diagnosis. 1, 3 When no cause is found, it is termed idiopathic hypertrophic pachymeningitis. Therefore, it has a broad range of neoplastic, chronic inflammatory, infectious, and hemorrhagic conditions associated with it, which can be a diagnostic challenge. Neurological presentation is less common and usually presents as hypophysitis followed by hypertrophic pachymeningitis (HP), 3 which is a rare inflammatory disorder that causes localized or diffuse thickening of intracranial dura mater. 4 Thus, additional immunosuppressive therapy may also be required. Most patients experience disease flares during or after glucocorticoid tapers. IgG4-RD is a steroid-responsive disease, and early recognition and treatment are essential because of the indolent nature of the condition and the risk of progression from an inflammatory stage to poorly responsive fibrotic disease and ultimately severe organ damage. Diagnosis of IgG4-RD requires the identification of characteristic findings upon biopsy of affected tissue, which is interpreted in the context of clinical, serologic, and radiologic data. 1 It was first reported in the pancreas in early 2000, but gradually it has been reported in other sites, 2, 3 particularly in salivary, bile duct, and lacrimal glands. It can involve any organ and form multiple stupefactive lesions.
Immunoglobulin G4-related disease (IgG4-RD) is recently recognized as a multisystemic syndrome characterized by an immune-mediated fibroinflammatory condition with unique clinical, serological, and pathological features.
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