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

First author :  Delplanque M et al 

Review: J Nephrol

Reference :  doi: 10.1007/s40620-024-02038-y 

PMID: 39266930

Link to article :  Pregnancy occurring in AA amyloidosis: a series of 27 patients including 3 new French cases - PubMed (nih.gov)

Introduction:

AA amyloidosis (AAA) is a multisystemic disease caused by the deposition of serum amyloid A (SAA) protein in tissues, which complicates chronic inflammatory conditions. It is a potentially life-threatening complication, with renal involvement being the most common manifestation. Pregnancy in women with chronic kidney disease is considered a risk factor for specific pregnancy complications and worsening of their underlying kidney disease. Data on pregnancy occurring during AA amyloidosis are limited, highlighting the importance of studying maternal and fetal outcomes. The objective of this study was to report pregnancy cases in patients with AA amyloidosis discussed within our reference center and to review the literature on the subject.

Patients and Methods:

Francophone cases were collected via the national multidisciplinary consultation on AA amyloidosis. A literature review was conducted using the MEDLINE and EMBASE databases. The analyzed data included maternal age, pregnancy complications, outcomes, and renal and inflammatory parameters.

Results:

Three new cases are described: a Turkish woman with familial Mediterranean fever (FMF) and postpartum nephrotic syndrome, a woman with cryopyrinopathy who had an uncomplicated pregnancy, and a Georgian patient with FMF, a kidney transplant recipient, whose pregnancy was complicated by infection and preterm delivery.

Results from the 3 New Cases and the Literature Review:

Among the 27 cases, including 24 from the literature and 3 new ones, 8 patients were diagnosed with AA amyloidosis during or just after pregnancy. The median age at diagnosis was 25 years [19-32]. FMF was the main cause of AA amyloidosis (19 cases), followed by cryopyrinopathies (2 cases), chronic inflammatory bowel disease (2 cases), and infections (2 cases). Renal complications were common: 33% (3/9) of patients with an eGFR < 60 mL/min/1.73 m² experienced renal deterioration during pregnancy; 66% (8/12) had increased proteinuria. Ninety-two percent (23/25) of patients had obstetric complications, including preterm birth (11/25), intrauterine growth restriction (10/25), preeclampsia (4/25), and hypertension (3/25). The median gestational age at delivery was 36.5 weeks [32.5; 38], mostly by cesarean section (17/22), and no hemorrhagic complications were reported. Two patients had undergone kidney transplantation before pregnancy. Estimated glomerular filtration rate (eGFR) was known before pregnancy in 10 patients, with a median of 55 mL/min/1.73 m² [38; 57], and 8/15 had significant proteinuria (> 0.5g/24h). Among those with known baseline eGFR before pregnancy, 33% (3/9) experienced a decline in renal function during pregnancy, but all recovered afterward. Two patients whose prior kidney function was unknown required hemodialysis. Proteinuria increased during pregnancy in 66% of patients (8/12).

Conclusion:

Pregnancy is a critical moment in the natural course of AA amyloidosis in terms of diagnosis and the risk of renal and obstetric complications. When possible, pregnancy should be planned with pre-conception counseling, multidisciplinary management, and close monitoring to minimize these risks.