Pregnancy occurring in AA amyloidosis: a series of 27 patients including 3 new French cases

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.