Mañá Juan, Montero Abelardo, Vidal Marta, Marcoval Joaquim, Pujol Ramón
Internal Medicine Service, University Hospital of Bellvitge, University of Barcelona, Barcelona, Spain.
Sarcoidosis Vasc Diffuse Lung Dis. 2003 Oct;20(3):212-21.
Recurrence of sarcoidosis following complete remission and after a prolonged time of inactivity is considered a very unusual clinical event. The aim of this study was to investigate the clinical characteristics of a series of patients diagnosed as having recurrent sarcoidosis.
Recurrent sarcoidosis was defined as reappearance of the disease following complete spontaneous clinical, radiographic and other markers of activity resolution or following resolution after corticosteroid treatment and maintained for at least during 3 years without therapy. The clinical records of patients diagnosed as having episodes of recurrent sarcoidosis during a period of 28 years at the university hospital of Bellvitge in Barcelona, Spain, were reviewed.
Seventeen patients suffering from 24 recurrences were identified. All but one patient were women. The mean follow-up time was 143 +/- 80 months (range: 52 to 282 months). Two patients had 3 recurrences, 3 had 2 and 12 had 1. The disease-free interval without treatment between the time when the disease became inactive after the initial presentation until the first recurrence varied from 10 months to more than 17 years, and the time of inactive disease without therapy between recurrences fluctuated from 23 months to more than 12 years. All the cases but one at onset and 17 at recurrence presented as Löfgren syndrome. At the time of the last control, 14 patients showed complete remission of the disease, one of them under corticosteroid therapy, 2 still had activity because of a recent recurrence, and 1 developed into chronic sarcoidosis.
Acute sarcoidosis, and particularly Löfgren's syndrome, may recur many years after complete remission and, in general, still has a good outcome. In consequence, a long-term follow-up is recommended even in patients with inactive disease. This clinical observation strongly suggests that a re-exposure to or reinfection by an extrinsic antigen triggers the new flare-ups of the disease.
结节病在完全缓解且经过长时间静止后复发被认为是一种非常罕见的临床事件。本研究旨在调查一系列被诊断为复发性结节病患者的临床特征。
复发性结节病定义为疾病在临床、影像学及其他活动指标完全自发缓解后再次出现，或在糖皮质激素治疗后缓解，并在至少3年未治疗的情况下保持缓解状态。回顾了西班牙巴塞罗那贝尔维特大学医院28年间被诊断为复发性结节病发作患者的临床记录。
共识别出17例患者出现24次复发。除1例患者外均为女性。平均随访时间为143±80个月（范围：52至282个月）。2例患者有3次复发，3例有2次复发，12例有1次复发。从疾病首次出现后静止到首次复发的无治疗无病间期从10个月到超过17年不等，复发之间的无治疗静止疾病时间从23个月到超过12年波动。除1例发病时和17例复发时外，所有病例均表现为 Löfgren 综合征。在最后一次复查时，14例患者疾病完全缓解，其中1例正在接受糖皮质激素治疗，2例因近期复发仍有活动，1例发展为慢性结节病。
急性结节病，尤其是 Löfgren 综合征，可能在完全缓解多年后复发，且总体预后仍然良好。因此，即使是疾病静止的患者也建议进行长期随访。这一临床观察强烈提示，再次接触或再次感染外部抗原会引发疾病的新发作。
