Mustak M, Boltuch-Sherif J, Horvath-Mechtler B, Kowalski-Bodzenta J, Erlacher L
Abteilung für Rheumatologie und Osteologie, Kaiser Franz Josef Spital Wien.
Dtsch Med Wochenschr. 2011 Sep;136(37):1842-4. doi: 10.1055/s-0031-1286354. Epub 2011 Sep 6.
MEDICAL HISTORY AND CLINICAL FINDINGS: A 70-year-old female patient suffered from steatorrhea and upper abdominal discomfort for 8 weeks combined with new onset of arthralgia in both hands. Additionally she reported elevated fasting blood glucose levels. The physical examination was without pathological findings except for mild upper abdominal pressure pain.
Imaging studies, including MRI and ultrasound examinations showed diffuse pancreatic enlargement without peripancreatic vessel involvement. Serological examinations showed elevated Cancer Associated Antigen 19 - 9 (1289 U/ml) and hyperglobulinemia with an IgG level of 170 mg/dl. The inflammatory markers were within normal ranges other than a slightly elevated erythrocyte sedimentation rate (35mm/1 h). Subsequent pancreatic biopsy showed lymphoplasmocellular, neutrophile and eosinophile granulocyte infiltration causing damage of the acinar pancreatic cells, typical for autoimmune pancreatitis (AIP). Magnetic resonance imaging (MRI) confirmed arthritis of both hands.
Medical treatment was started with oral prednisolone (50 mg/day) for one week, tapered to 25 mg/day for another 2 weeks, followed by dose reductions of 5 mg/day every 2 weeks with a final maintenance dose of 5 mg/day for 8 months. After the first week of steroid therapy methotrexate (MTX) was started with an initial dose of 10 mg/week. Dose was raised until a final dosage of 30 mg/week. After 8 months without relapse, the maintenance therapy was reduced to 20 mg/week MTX and corticosteroids were stopped.
With this treatment regimen the patient has showed complete remission of AIP and arthritis for 36 months. MTX may be successful as an initial basic treatment to reach better control of autoimmune-related extrapancreatic manifestations.
病史及临床检查结果:一名70岁女性患者出现脂肪泻和上腹部不适8周,双手关节痛新发。此外,她报告空腹血糖水平升高。体格检查除轻度上腹部压痛外无病理发现。
影像学检查,包括MRI和超声检查显示胰腺弥漫性肿大,胰周血管未受累。血清学检查显示癌胚抗原19-9升高(1289 U/ml),球蛋白血症伴IgG水平为170 mg/dl。炎症标志物除红细胞沉降率略有升高(35mm/1小时)外均在正常范围内。随后的胰腺活检显示淋巴细胞、浆细胞、中性粒细胞和嗜酸性粒细胞浸润,导致胰腺腺泡细胞受损,这是自身免疫性胰腺炎(AIP)的典型表现。磁共振成像(MRI)证实双手关节炎。
开始口服泼尼松龙(50mg/天)治疗1周,然后减至25mg/天再治疗2周,随后每2周剂量减少5mg/天,最终维持剂量为5mg/天,持续8个月。在类固醇治疗的第一周后开始使用甲氨蝶呤(MTX),初始剂量为10mg/周。剂量逐渐增加直至最终剂量为30mg/周。8个月无复发后,维持治疗减至MTX 20mg/周,停用皮质类固醇。
采用该治疗方案,患者的AIP和关节炎已完全缓解36个月。MTX作为初始基础治疗可能成功,以更好地控制自身免疫相关的胰腺外表现。