Stey C, Marincek B, Salomon F
Departement für Innere Medizin, Universitätsspital Zürich.
Dtsch Med Wochenschr. 1995 Jun 16;120(24):874-8. doi: 10.1055/s-2008-1055420.
A 71-year-old woman with a seven-year history of seronegative polyarthritis had to be intubated and ventilated because of acute respiratory failure. The chest radiograph and computed tomography revealed bilateral pleural effusions and massive enlargement of the thyroid which compressed the trachea. Concentration of the thyroid-stimulating hormone was slightly reduced, while that of peripheral thyroid hormone was normal. Histological examination of a goitre specimen after strumectomy showed macrofollicular goitre with massive amyloid deposits. Amyloid was subsequently also demonstrated in the gastrointestinal tract. The patient died 5 months later from respiratory failure. A second patient, 47 years old at hospitalization, had been suffering from arthritic psoriasis since she was a child and had been undergoing haemodialysis for renal failure caused by amyloid. She had undergone angiography because the haemodialysis shunt had become occluded. She had been admitted because of an increase in goitre size during the last 6 months and a malignancy was suspected. Computed tomography showed the thyroid enlargement with retrosternal extension and a nodule in the thyroid isthmus. After injection of contrast medium for the angiography she developed, on the basis of probably already existing hyperthyroidism with an increased level of thyroid-stimulating immunoglobulin, clinically and biochemically manifest hyperthyroidism, which was treated with thiamazole (15 mg daily) and propranolol (40 mg twice daily). Cytological examination of a fine-needle biopsy of thyroid tissue revealed amyloid deposits. The patient died 4 years later, shortly after bilateral hip replacement for femoral neck fractures. At autopsy a large amyloid goitre and amyloid deposits in the kidneys, gastrointestinal tract and coronary arteries were found.
一名患有血清阴性多关节炎7年的71岁女性因急性呼吸衰竭不得不进行气管插管和机械通气。胸部X线片和计算机断层扫描显示双侧胸腔积液以及甲状腺肿大压迫气管。促甲状腺激素浓度略有降低,而外周甲状腺激素浓度正常。甲状腺切除术后甲状腺肿标本的组织学检查显示为大滤泡性甲状腺肿伴大量淀粉样沉积。随后在胃肠道也发现了淀粉样物质。患者5个月后死于呼吸衰竭。第二名患者住院时47岁,自幼患有关节病性银屑病,因淀粉样变导致肾衰竭一直在接受血液透析。因血液透析分流管堵塞而进行了血管造影。因过去6个月甲状腺肿大增大且怀疑有恶性病变而入院。计算机断层扫描显示甲状腺肿大并向胸骨后延伸,甲状腺峡部有一个结节。在血管造影注射造影剂后,基于可能已存在的甲状腺刺激免疫球蛋白水平升高的甲状腺功能亢进症,她出现了临床和生化表现明显的甲状腺功能亢进症,用甲巯咪唑(每日15毫克)和普萘洛尔(每日40毫克,分两次服用)进行治疗。甲状腺组织细针穿刺活检的细胞学检查发现有淀粉样沉积。患者4年后在因股骨颈骨折进行双侧髋关节置换术后不久死亡。尸检发现一个大的淀粉样甲状腺肿以及肾脏、胃肠道和冠状动脉中有淀粉样沉积。