Delplanque Marion, Savey Léa, Cognard Noelle, Boffa Jean Jacques, Buob David, Georgin-Lavialle Sophie
Department of Internal Medicine, Centre de référence des maladies autoinflammatoires et des amyloses (CEREMAIA), DMU i3d, AP-HP, Hôpital Tenon, Service de Médecine Interne, Sorbonne University, Tenon Hospital, 75020, Paris, France.
Department of Nephrology and Transplantation, Strasbourg University Hospital, Strasbourg, France.
J Nephrol. 2024 Dec;37(9):2509-2519. doi: 10.1007/s40620-024-02038-y. Epub 2024 Sep 12.
AA amyloidosis (AAA) is a multisystem disease related to the deposition in tissues of serum amyloid A protein which complicates chronic inflammation. It is a potentially fatal complication. Renal involvement is the most common manifestation of AAA. Pregnancy in women with chronic kidney disease is considered to be at risk for specific pregnancy complications and the worsening of their underlying renal dysfunction. Our aim was to report pregnancy in our AAA patients and discuss the outcome through a literature review.
French cases were identified through the Reference Center for Auto-Inflammatory Diseases and Amyloidosis and a systematic literature review was performed.
Three new patients were identified: two with Familial Mediterranean fever (FMF) and one with cryopyrin-associated periodic syndrome; one was under anakinra therapy and one had received a kidney transplantation before her pregnancy. One patient was diagnosed with AAA following the detection of post-partum nephrotic syndrome. Among the 27 patients from literature and our case, FMF was the main cause of AAA (69%). Eight of the patients were diagnosed with AAA during their pregnancy or in immediate post-partum and gestational complications appeared in 23/25 cases, mostly intrauterine growth retardation (n = 10), prematurity (n = 11) and preeclampsia (n = 4). No bleeding complication was reported.
Pregnancy can occur in patients (eight overall) with AAA and the diagnosis is frequently made during pregnancy. Pregnant women with AAA are at risk for adverse pregnancy-associated outcomes and require special and closer monitoring.
AA型淀粉样变性（AAA）是一种多系统疾病，与血清淀粉样蛋白A在组织中的沉积有关，常并发于慢性炎症。它是一种潜在的致命并发症。肾脏受累是AAA最常见的表现。慢性肾病女性怀孕被认为有特定妊娠并发症及基础肾功能恶化的风险。我们的目的是报告我们的AAA患者的妊娠情况，并通过文献综述讨论其结局。
通过自身炎症性疾病和淀粉样变性参考中心确定法国的病例，并进行系统的文献综述。
确定了3例新患者：2例患有家族性地中海热（FMF），1例患有冷吡啉相关周期性综合征；1例接受阿那白滞素治疗，1例在怀孕前接受了肾脏移植。1例患者在产后肾病综合征检测后被诊断为AAA。在文献报道的27例患者及我们的病例中，FMF是AAA的主要病因（69%）。8例患者在孕期或产后立即被诊断为AAA，25例中有23例出现妊娠并发症，主要是宫内生长受限（n = 10）、早产（n = 11）和先兆子痫（n = 4）。未报告出血并发症。
AAA患者（共8例）可以怀孕，且诊断常在孕期做出。患有AAA的孕妇有不良妊娠相关结局的风险，需要特殊且密切的监测。
