M ikulicz病为临床罕见病。其临床特征为双侧对称性泪腺和唾液腺肿大，血清IgG4显著升高，泪腺、唾液腺活检可见IgG4＋浆细胞浸润。本病在组织学上以广泛淋巴细胞增生和浸润为特征，腺组织逐渐萎缩而被肉芽组织和结缔组织所取代［1］。...M ikulicz病为临床罕见病。其临床特征为双侧对称性泪腺和唾液腺肿大，血清IgG4显著升高，泪腺、唾液腺活检可见IgG4＋浆细胞浸润。本病在组织学上以广泛淋巴细胞增生和浸润为特征，腺组织逐渐萎缩而被肉芽组织和结缔组织所取代［1］。本病予糖皮质激素类药物治疗效果明显，但也有复发。由于病变内主要为淋巴细胞浸润，也可局部放射治疗。另外，由于肿物位置靠前，肿块较为局限，当临床诊断困难时，亦可给予手术切除，术后辅以糖皮质激素类药物治疗［2］。由于本病可能导致吞咽困难、视力下降等并发症，因此治疗过程中密切观察及有针对性的护理具有重要意义。现将北京协和医院免疫科2008－2010年收治的5例患者的护理体会报告如下。展开更多
Autoimmune pancreatitis(AIP)is a form of chronic pancreatitis that is categorized as type 1 or type 2according to the clinical profile.Type 1 AIP,which predominantly presents in a few Asian countries,is a hyper-IgG4-r...Autoimmune pancreatitis(AIP)is a form of chronic pancreatitis that is categorized as type 1 or type 2according to the clinical profile.Type 1 AIP,which predominantly presents in a few Asian countries,is a hyper-IgG4-related disease.We report a case of IgG4-related AIP overlapping with Mikulicz’s disease and lymphadenitis,which is rare and seldom reported in literature.A 63-year male from Northeast China was admitted for abdominal distension lasting for one year.He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo.He had a 2-year history of bilateral submandibular lymphadenopathy without pain.He underwent surgical excision of the right submandibular lymph node one year prior to admission.He denied any history of alcohol,tobacco,or illicit drug use.Serological examination revealed high fasting blood sugar level(8.8 mmol/L)and high level of IgG4(15.2 g/L).Anti-SSA or anti-SSB were negative.Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation.Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens.The patient received a dose of 30 mg/d of prednisone for three weeks.At this three-week follow-up,the patient reported no discomfort and his swollen salivary glands,neck lymph node and pancreas had returned to normal size.The patient received a maintenance dose of 10mg/d of prednisone for 6 mo,after which his illness had not recurred.展开更多
基金Supported by National Natural Scientific FoundationNo.8107037081270544(to Gao RP)and NIH 5R01AA016003(to Brigstock D)
文摘Autoimmune pancreatitis(AIP)is a form of chronic pancreatitis that is categorized as type 1 or type 2according to the clinical profile.Type 1 AIP,which predominantly presents in a few Asian countries,is a hyper-IgG4-related disease.We report a case of IgG4-related AIP overlapping with Mikulicz’s disease and lymphadenitis,which is rare and seldom reported in literature.A 63-year male from Northeast China was admitted for abdominal distension lasting for one year.He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo.He had a 2-year history of bilateral submandibular lymphadenopathy without pain.He underwent surgical excision of the right submandibular lymph node one year prior to admission.He denied any history of alcohol,tobacco,or illicit drug use.Serological examination revealed high fasting blood sugar level(8.8 mmol/L)and high level of IgG4(15.2 g/L).Anti-SSA or anti-SSB were negative.Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation.Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens.The patient received a dose of 30 mg/d of prednisone for three weeks.At this three-week follow-up,the patient reported no discomfort and his swollen salivary glands,neck lymph node and pancreas had returned to normal size.The patient received a maintenance dose of 10mg/d of prednisone for 6 mo,after which his illness had not recurred.
