Background: Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjögren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjögren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course.

Materials and methods: We investigated the clinical pictures and histopathological findings of three cases with both presence of Sjögren syndrome and localized nodular AL amyloidosis, paying a special attention to the distribution of amyloidoma.

Results: All three cases were middle-aged females. In two of three cases localized deposits of AL amyloid preceded Sjögren syndrome. Amyloidoma was detected in scalp, eyelid, cheek, larynx, trachea, lung and breast, and around these amyloid-deposited lesions infiltration of plasma cells was seen. Pulmonary amyloidosis was consistently accompanied with parenchymal cystic lesions, but this amyloidosis did not produce any significant respiratory symptoms. Some of large pulmonary amyloidomas showed cavity formation and subsequent shrinkage. In two cases amyloid deposition was found on gastric mucosa. Two cases received small doses of oral prednisone, with no further appearance of amyloidoma.

Conclusion: Sjögren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis.

背景：在不同的组织/器官中均可见局部的AL淀粉样结节状沉积物。然而，这种淀粉样变性病的发病机理仍不清楚。最近，已经注意到Sjögren综合征合并局部结节性AL淀粉样变性。在这里，我们报告了Sjögren综合征病例，显示多灶性结节性AL淀粉样变性和随后的良性病程。

材料和方法：我们调查了3例同时存在Sjögren综合征和局部结节性AL淀粉样变性的病例的临床图片和组织病理学发现，特别注意淀粉样瘤的分布。

结果：三例均为中年女性。在三分之二的病例中，AL淀粉样蛋白的局部沉积先于Sjögren综合征。在头皮，眼睑，脸颊，喉，气管，肺和乳房中检测到淀粉样瘤，并且在这些淀粉样沉积病灶周围可见浆细胞浸润。肺淀粉样变性常伴有实质性囊性病变，但这种淀粉样变性并未产生任何明显的呼吸道症状。一些大的肺淀粉样瘤显示出腔形成和随后的萎缩。在两种情况下，在胃粘膜上发现淀粉样蛋白沉积。2例接受小剂量口服泼尼松治疗，未再出现淀粉样瘤。

结论： Sjögren综合征相关的浆细胞疾病可能是造成这种独特的多灶性结节性AL淀粉样变性的原因。