Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hypertrophic pachymeningitis is a rare disease, and the fibrosing inflammatory process causes a thickening of the dura mater. A 62-year-old male undergoing corticosteroid therapy for autoimmune pancreatitis presented with headache and right facial numbness. Brain CT and MRI revealed thickened mass lesion around the tentorium. The specimen obtained by biopsy showed a small number of immunostain areas positive for IgG and IgG4. Systemic IgG4 related disease entity is proposed and analyzed from Japan, and pachymeningitis is also included in the examination. Some autoimmune mechanism is related to pachymeningitis, however, it is necessary to consider well if only the IgG4 has responsibility for the disease.
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PMID:[Hypertrophic pachymeningitis associated with autoimmune pancreatitis examined for IgG4 related disease: a case report]. 2179 26

Hypertrophic pachymeningitis is a chronic inflammatory disease, manifesting as a fibrous thickening of the dura mater. Recently, it was reported that IgG4 is related to the etiology of some diseases, such as pancreatitis. Here, we report a case of hypertrophic pachymeningitis related to infiltration of IgG4 positive plasma cells. A 68-year-old man presented at a nearby hospital with a headache and a low-grade fever. A blood test revealed inflammation, as well as elevation of IgG4 level. Magnetic resonance imaging(MRI)revealed diffuse thickening of the dura mater, dominantly in the posterior fossa and cerebellar tentorium. The lesion was enhanced significantly with gadolinium(Gd). An open biopsy was performed to determine pathological diagnosis. Hematoxylin and eosin staining showed infiltration of inflammatory cells, including plasma cells. The infiltrating cells were positive for the IgG4. Post-operatively, the patient was treated with glucocorticoid, and both the inflammation and patient symptoms were improved. In conclusion, IgG4 is related to the etiology of hypertrophic pachymeningitis and glucocorticoid therapy is effective for this disease.
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PMID:[A Case of IgG4-related Hypertrophic Pachymeningitis]. 3057 8