Department of Pediatrics, KIST Medical College Teaching Hospital, Imadol, Lalitpur, Nepal.
BMC Pediatr. 2013 Nov 5;13:179. doi: 10.1186/1471-2431-13-179.
Systemic lupus erythematosus (SLE) is known to present with a wide variety of clinical manifestations. Lymphadenopathy is frequently observed in children with SLE and may occasionally be the presenting feature. SLE presenting with granulomatous changes in lymph node biopsy is rare. These features may also cause diagnostic confusion with other causes of granulomatous lymphadenopathy.
We report 12 year-old female who presented with generalized lymphadenopathy associated with intermittent fever as well as weight loss for three years. She also had developed anasarca two years prior to presentation. On presentation, she had growth failure and delayed puberty. Lymph node biopsy revealed granulomatous features. She developed a malar rash, arthritis and positive ANA antibodies over the course of next two months and showed WHO class II lupus nephritis on renal biopsy, which confirmed the final diagnosis of SLE. She was started on oral prednisolone and hydroxychloroquine with which her clinical condition improved, and she is currently much better under regular follow up.
Generalized lymphadenopathy may be the presenting feature of SLE and it may preceed the other symptoms of SLE by many years as illustrated by this patient. Granulomatous changes may rarely be seen in lupus lymphadenitis. Although uncommon, in children who present with generalized lymphadenopathy along with prolonged fever and constitutional symptoms, non-infectious causes like SLE should also be considered as a diagnostic possibility.
系统性红斑狼疮(SLE)的临床表现多种多样。淋巴结病在儿童 SLE 中经常观察到,并且偶尔可能是首发症状。SLE 淋巴结活检表现为肉芽肿性改变则较为罕见。这些特征也可能导致与其他肉芽肿性淋巴结病的原因引起的诊断混淆。
我们报告了一例 12 岁女性,表现为全身性淋巴结病,伴有间歇性发热和三年体重减轻。她在发病前两年还出现了全身水肿。就诊时,她出现了生长发育迟缓,青春期延迟。淋巴结活检显示出肉芽肿特征。在接下来的两个月里,她出现了蝶形红斑、关节炎和 ANA 抗体阳性,在肾活检中显示为 WHO Ⅱ级狼疮肾炎,最终确诊为 SLE。她开始口服泼尼松龙和羟氯喹,病情得到改善,目前在定期随访中情况良好。
全身性淋巴结病可能是 SLE 的首发症状,正如本例患者所示,它可能在 SLE 的其他症状出现之前多年就存在。狼疮性淋巴结炎中可能很少见到肉芽肿性改变。虽然不常见,但对于出现全身性淋巴结病、长期发热和全身症状的儿童,除了感染性疾病外,还应考虑 SLE 等非感染性疾病作为诊断的可能性。