Idiopathic multifocal choroiditis and punctate inner choroidopathy: an evaluation in pregnancy

Abstract

Purpose: To evaluate the clinical course of idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and the efficacy and safety of treatment options during pregnancy.

Methods: Patients with MFC or PIC and a pregnancy in 2011-2019 from two academic centres were enrolled. For the most recent pregnancy, data on best-corrected visual acuity (BCVA) before and after pregnancy, relapse rate in pregnancy and postpartum period and obstetric, maternal and neonatal outcomes were collected. Treatment regimens consisted of a wait-and-see regime and an immunosuppressive treatment regime with systemic corticosteroids and/or azathioprine, both combined with intravitreal antivascular endothelial growth factor injections when indicated.

Results: Sixteen women (26 affected eyes) were included. Median Snellen BCVA was 20/19 before pregnancy and 20/18 after delivery. In seven pregnancies a wait-and-see regime and in nine pregnancies an immunosuppressive treatment regime was carried out. Fourteen intravitreal anti-VEGF injections were given in six pregnancies. The relapse rate during pregnancy was 44% and in the postpartum period 31%. Maternal/obstetrical and fetal complications occurred in 31% and 13% of the pregnancies, respectively. Fifteen healthy children were born and one pregnancy ended in a stillbirth in a patient with a complicated obstetrical history. One patient treated with azathioprine developed intrahepatic cholestasis of pregnancy (ICP).

Conclusions: Among women with MFC and PIC BCVA remained stable during pregnancy despite a relapse rate of 44% in pregnancy. No major maternal, obstetric and fetal complications occurred in pregnant patients treated with systemic corticosteroids, azathioprine or intravitreal anti-VEGF injections, though one patient developed ICP while treated with azathioprine.

Keywords: MFC; anti-VEGF; immunomodulatory therapy; multifocal choroiditis; pregnancy; punctate inner choroidopathy.

Figure 1

Disease activity in pregnancy and postpartum period in case 8. (A, B) Colour fundus pictures taken at gestational age (GA) of 5 weeks (A) and 11 weeks postpartum (B) showing growth of central choroidal lesions during pregnancy and the postpartum period. (C, D) Optical coherence tomography scans on the Heidelberg Spectralis® with eye‐tracker function. (C) No disease activity at GA 10 weeks. (D) A relapse of disease activity at GA of 20 weeks. (E, F) A relapse of disease activity at 11 weeks postpartum. Fluorescein (E) and indocyanine green (F) pictures at 20 min showing (E) leakage of active CNV and (F) dark choroidal lesions with blurred boundaries indicating inflammatory activity.

Figure 2

Relapse‐free survival during pregnancy. (A) Demonstrates the relapse‐free survival during pregnancy of all treatment regimens. (B) Demonstrates the relapse‐free survival during pregnancy split out in treatment regimens. The continuing line represents the patients treated with a wait‐and‐see regime, the dashed line represents the patients treated with an immunosuppressive treatment regime with systemic corticosteroids and/or azathioprine.