The multimodal imaging features and outcomes of multifocal choroiditis/punctate inner choroidopathy lesion with multiple evanescent white dot syndrome-like features: a retrospective study.

BACKGROUND: Multiple evanescent white dot syndrome (MEWDS)-like features is a rare condition triggered by a macular disease or iatrogenic injury, exhibiting MEWDS changes in the fundus. This study aims to describe the multimodal imaging features and outcomes of multifocal choroiditis/punctate inner choroidopathy (MFC/PIC) lesions with MEWDS-like features. METHODS: Six cases were studied retrospectively. All cases were given regional and oral corticosteroids. RESULTS: All cases showed an isolated juxtafoveal yellowish-white MFC/PIC lesion with disruption of RPE-Bruch's membrane-choriocapillaris complex (RPE-BM-CC), subretinal hyperreflective materials and choroidal thickening on optical coherence tomography. Two weeks after presentation, the grayish-white dots disappeared spontaneously and the corticosteroids were given. After four weeks, the ellipsoid zone (EZ) around the lesion and hyper-autofluorescence resolved. After 13 weeks, five cases showed shrinkage of the juxtafoveal lesion and restoration of foveal EZ. After six months, the juxtafoveal lesion became pigmented. Only one case developed type 2 choroidal neovascularization. CONCLUSIONS: The clinical course of MEWDS-like manifestations is still evanescent in our cases. The yellowish-white juxtafoveal MFC/PIC lesions with disruption of RPE-BM-CC and choroidal thickening showed a well-controlled prognosis after corticosteroid treatment.

A case of tubulointerstitial nephritis and uveitis syndrome accompanied by subclinical choroiditis.

BACKGROUND: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uveits characterized by complications of idiopathic acute tubulointerstitial nephritis, and most cases present only anterior uveitis. We report a case of TINU syndrome in which the presence of choroiditis was revealed by multimodal imaging. CASE PRESENTATION: A 12-year-old male visited our hospital with a 6-day history of ocular pain and hyperemia. Conjunctival and ciliary injections, 1 + flare and 3 + cells of anterior chamber inflammation with mutton fat keratic precipitates were observed in both eyes (OU), together with redness and swelling of the optic disc OU. Laboratory tests showed slightly high levels of soluble IL-2R and serum ß2 microglobulin and markedly high levels of urinary ß2 microglobulin. The diagnosis of probable TINU syndrome was established on the basis of bilateral uveitis and urinalysis results in accordance with a clinical criteria of tubulointerstitial nephritis. With treatment with oral prednisolone (PSL) at 20 mg/day, ocular findings improved, and the dose of PSL was gradually reduced and withdrawn 6 months later. However, 1 month later from the withdrawal, ocular inflammation recurred with the presence of retinal exudates and snowball vitreous opacities in the peripheral retina OU. Fluorescein angiography showed leakages from peripheral retinal vessels and staining corresponding to retinal exudates. Indocyanine green angiography showed hypofluorescent dots scattered over the ocular fundus. Optical coherence tomography revealed the presence of choroidal thickening. Laser speckle flowgraphy color map showed a relatively cooler color. Findings from these multimodal images indicated the presence of subclinical choroiditis; therefore, oral PSL was administered again, and ocular inflammatory findings were improved. CONCLUSIONS: TINU syndrome can exhibit subclinical choroiditis detected with multimodal imaging. Further studies are necessary to determine the frequency of subclinical choroiditis in TINU syndrome.

Polypoidal choroidal vasculopathy 20 years after resolution of tubercular choroiditis.

We report the case of a 72-year-old white woman with blurring of vision in both eyes of 15 days' duration. She had a history of treatment for bilateral tuberculosis choroiditis 20 years before. She was diagnosed with polypoidal choroidal vasculopathy in the right eye and inflammatory choroidal neovascular membrane in the left eye, based on multimodal imaging, including optical coherence tomography, fundus fluorescein angiography, and indocyanine green angiography. The right eye received 3 intravitreal injections of aflibercept and showed complete resolution. The left eye was treated with a single intravitreal injection of aflibercept.

Punctate inner choroidopathy in common variable immunodeficiency associated with a pathogenic variant in the tumour necrosis factor receptor superfamily 13b (TNFRSF13B) gene - Case report and review of the literature.

BACKGROUND: Common variable immunodeficiency (CVID) has been recognised as the most common primary immunodeficiency in adulthood, and is characterised by increased susceptibility to infection, autoimmunity and increased risk of malignancies. Although ocular manifestations are not common in CVID, rare associated inflammatory eye conditions have been reported including submacular choroiditis. OBJECTIVE: To report a case of punctate inner choroidopathy in a patient with common variable immunodeficiency. CASE PRESENTATION: A 40-year-old lady with CVID and associated autoimmune thrombocytopenia, who was treated with immunoglobulin replacement and Eltrombopag, experienced gradually deteriorating right eye vision. Fundal examination and optical coherence tomography (OCT) revealed right multifocal retinal choroidal lesions consistent with a diagnosis of unilateral punctate inner choroidopathy (PIC) with secondary choroidal neovascularisation (CNV). Anti-VEGF injections led to stabilised fundal appearances. Genetic testing revealed a heterozygous sequence change c.260 T > Ap.(IIe87Asn), pathogenic variant in the Tumour Necrosis Factor Superfamily 13B (TNFRSF13B) gene, which is reported as being associated with ∼10% of CVID cases. CONCLUSION: Autoimmunity may be the dominant clinical presenting feature of CVID. Punctuate inner choroidopathy is an idiopathic inflammatory chorioretinopathy, and to the best of our knowledge, has not been previously reported in CVID. A better understanding of the molecular bases of autoimmune diseases in CVID may provide novel therapeutic targets for autoimmune diseases in this patient population.

Intraocular and neuro-cysticercosis with diffuse stromal choroiditis.

Intraocular cysticercosis with central nervous system involvement is not that rare. We report a male child with a right-sided painful blind eye who had intraocular cysticercosis and granuloma in the left frontal lobe of the brain. There was an incidental finding of chronic inflammation in the choroid of that eye supported by histopathology. Immunohistochemistry for T-cells marker and B-cells marker was variable. The patient was treated with antiparasitic, anti-epileptic medications, and oral steroids subsequently.

Bilateral Ampiginous Choroiditis following Confirmed SARS-CoV-2 Infection.

INTRODUCTION: Ampiginous Choroiditis is a rare posterior uveitis that combines clinical features of Acute Multifocal Posterior Placoid Pigment Epitheliopathy and Serpiginous Chorioretinitis. Its pathophysiology is poorly understood and further studies are necessary to understand which mechanisms start the immunologic reaction. CASE REPORT: The purpose of this article is to report a well-documented case of Ampiginous Choroiditis following in seven days a RT-PCR confirmed SARS-CoV-2 infection, suggesting that the infection might have contributed as a trigger. CONCLUSION: Timely diagnosis and correct treatment are paramount to improve the visual outcomes, and the patient had successful response to systemic steroids.

Multifocal Serpiginoid Choroiditis Due to Mycobacterium Mageritense following Laparoscopic Hysterectomy in an Immunocompetent Host.

PURPOSE: To report nontubercular choroiditis, caused by Mycobacterium mageritense isolated using MALDI-TOF MS, following laparoscopic hysterectomy in an immunocompetent patient. CASE REPORT: A 42-year-old female presented with gradual, painless diminution of vision in the right eye for six weeks. She had undergone laparoscopic hysterectomy two months back. BCVA was 2/60 OD. Clinical presentation and imaging all pointed to a diagnosis of serpiginous-like choroiditis. Matrix-Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry (MALDI-TOF MS) had led to isolation of Mycobacterium mageritense. Patient was treated with systemic antibiotics. At 3 months follow up, BCVA improved to 6/60; fundus showed healed choroiditis and chorioretinal thinning. CONCLUSION: Atypical mycobacterial infections associated with laparoscopic surgeries can rarely lead to debilitating systemic bacteremia resulting in profound visual impairment. The importance of timely diagnosis using specific and sophisticated methods such as MALDI-TOF MS as well as maintaining aseptic precautions perioperatively during major surgeries cannot be overemphasized.

Recurrence of tubercular choroiditis following anti-SARS-CoV-2 vaccination.

PURPOSE: To report recurrence of tubercular choroiditis following anti-SARS-CoV-2 vaccination in two patients with quiescent disease activity for more than a year. METHODS: Retrospective observational case reports. RESULTS: Two patients (one female and one male) under follow-up for posterior uveitis having stable course with absence of ocular inflammation for more than a year presented with recurrence of choroiditis lesions 2-6 weeks following anti-SARS-CoV-2 vaccination. Both the patients were managed with intravitreal dexamethasone implant (Ozurdex®, Allergan, Inc., Irvine, CA, USA) and showed resolution of choroiditis lesions upon follow-up. CONCLUSIONS: Acute onset recurrence of inflammation, in absence of any change in health status or treatment suggests the potential role of vaccination being the trigger of this reactivation. Given large-scale vaccination against novel coronavirus- SARS-CoV-2, careful vigilance is warranted to pick up the disease recurrence in patients with posterior uveitis.

Multifocal choroiditis and choroidal tuberculoma: Signs of presumed ocular tuberculosis.

The diagnosis of OTB (Ocular tuberculosis) is usually difficult to make. Definitive diagnosis requires the identification of M. tuberculosis organisms in ocular tissues or fluids, but samples are often difficult to obtain, and biopsy may be hard to justify. We describe a 50-years-old Maghreb male, who presented a multifocal choroiditis associated with a choroidal tuberculoma on the left eye. Based on positive QuantiFERON-TB-Gold test and suggestive clinical and radiographic findings, a diagnosis of presumed ocular tuberculosis was made. Serial swept-source optical coherence tomography (SS-OCT) and widefield fundus retinographies during subsequent follow-up visits demonstrated the characterization of the atypical tuberculosis presentation and allowed the assessment of response to antitubercular therapy and oral steroids.

Primary Sjögren's syndrome-related Choroiditis in a Newly Diagnosed Older Adult.

PURPOSE: To report a case of secondary bilateral choroiditis in a patient with primary Sjögren's syndrome(pSS). STUDY DESIGN: Case report. RESULTS: A 69-year-old woman visited our hospital for consultation due to decreased visual acuity in both eyes for 1 month. At the first visit, best corrected visual acuity (BCVA) was 0.2 and 0.3 in her right and left eyes, respectively. Intraocular pressure values were 15 mmHg and 16 mmHg in her right and left eyes, respectively. Examination revealed edema of the eyelids and conjunctiva,and corneal fluorescence staining was positive. No inflammation in the anterior chamber or vitreous opacities were observed. Bilateral multiple retinal detachments were observed on the posterior fundus, and optical coherence tomography revealed bilateral multiple areas of retinal neuroepithelial detachment, choroidal thickening, and choroidal folds. No abnormal fluorescence leakage was observed on fundus fluorescein angiography or indocyanine green angiography. In addition, systematic manifestations included recurrent bilateral parotid gland enlargement. Labial gland biopsy revealed dilated glandular ducts, scattered interstitial glands, and lymphocytic foci. Salivary gland scintigraphy revealed severe impairment of glandular excretory function. Moreover, blood tests for anti-Ro/SSA and anti-La/SSBantibodies were positive. The patient was diagnosed with primary Sjögren's syndrome. After 2 months treatment with oral prednisolone acetate combined with hydroxychloroquine, her BCVAimproved to 0.8 and 1.0 in the right and left eyes, respectively. The fundus also recovered to normal, and no recurrence was observed during the 1-year follow-up period. CONCLUSIONS: The current case highlights that pSS, which usually manifests with dry eye and keratoconjunctivitis, may manifest with chronic choroiditis in both eyes as well. Based on our experience with this case, patients with clinically suspected bilateral choroiditis should be evaluated for pSS.

PUNCTATE INNER CHOROIDOPATHY-LIKE REACTIONS IN UNRELATED RETINAL DISEASES.

PURPOSE: To report a cohort of patients with a punctate inner choroidopathy (PIC)-like reaction in concurrent, unrelated, chorioretinal disorders. METHODS: This was a retrospective observational study of patients seen at two referral centers with lesions consistent with PIC on multimodal imaging; patients with lesions resembling idiopathic multifocal choroiditis were also included. Active PIC-like lesions appeared as focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane (RPE/BrM) complex on optical coherence tomography. Chronic PIC-like lesions included subretinal fibrosis, multifocal punched-out chorioretinal atrophy, and curvilinear streaks. Patients' demographics, additional imaging features, and treatment responses were collected and summarized. RESULTS: Twenty-two eyes of 16 patients with a PIC-like reaction were included (75% females; median age 40 years). Underlying diagnoses included hereditary retinal conditions (10 patients, 63%) and acquired etiologies, all characterized by the RPE/BrM or outer retinal disruption. Fifteen eyes (68%) had active PIC-like lesions; seven eyes (32%) had chronic PIC-like lesions. Active PIC-like lesions regressed with time and responded to systemic steroids. Subretinal fibrosis (3 eyes, 20%), macular atrophy (3 eyes, 20%), and concomitant subretinal fibrosis and macular atrophy (5 eyes, 33%) developed on follow-up. Recurrences occurred in five eyes (23%). CONCLUSION: RPE/BrM or outer retina disruption may trigger a PIC-like reaction in susceptible patients, presumably because of the loss of immune privilege. A PIC-like reaction may influence the clinical progression and the visual prognosis of the primary chorioretinal disease.

Clinical case: Serpiginous-like choroiditis with macular involvement and good response after treatment with adalimumab.

Clinical case of a 42-year-old woman who consulted for decreased vision in the left eye over two months, associated with multifocal choroiditis. Different diagnostic tests were performed to rule out infectious pathologies, as well as a diagnostic vitrectomy test, which was negative. QuantiFERON® was found to be positive and a diagnosis of serpeginous-like choroiditis (SLC) was made. Anti-tuberculosis treatment was started with worsening of vision and multifocal choroiditis. Once conventional immunotherapy and biological treatment were added, the patient presented a significant improvement in vision. To conclude, in cases of CSL, it is not only necessary to treat the infection but also the inflammatory component either with steroids or conventional immunotherapy or immunosuppression with biologics, thus reducing the number of reactivations and improving the visual prognosis.

Idiopathic Multifocal Choroiditis and Punctate Inner Choroidopathy - Evaluation of Risk Factors for Increased Relapse Rate: A 2-Year Prospective Observational Cohort Study.

INTRODUCTION: The aim of this study was to describe the course of disease in patients with idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and to identify risk factors associated with an increased relapse rate of disease activity. METHODS: In this prospective observational cohort study, demographical and clinical data were collected concerning the relapses rate of disease activity, the conclusions of the multimodal imaging results, treatment, complications, and self-reported quality of life. Disease activity was defined as new inflammatory lesions or active inflammation in preexisting chorioretinal lesions either with or without active choroidal neovascularization (CNV). Linear regression analysis was performed to identify risk factors associated with an increased relapse rate. RESULTS: In total, 122 eyes of 82 patients (93% females) were included with a median age (IQR) of 45 (37-54) years. A history of secondary CNV was present in 66% of the eyes. During follow-up, the best-corrected visual acuity remained stable despite a median relapse rate (IQR) of 1.0 (0.25-3). Cycles of oral corticosteroids were given in 59% of the patients, 72% were treated at baseline or started treatment during follow-up with a disease-modifying antirheumatic drug (DMARD), and 35% with a biological agent in addition to the DMARD. Both a history of secondary CNV (B = 1.2, 95% CI: 0.7-1.7, p = 3.6 × 10-5) and high myopia (<-6 diopters) (B = 0.6, 95% CI: 0.1-1.1, p = 0.02) independently increased the relapse rate of disease activity. DISCUSSION/CONCLUSION: A history of secondary CNV and high myopia were associated with an increased relapse rate of disease activity. Moreover, the results of this study emphasize the challenging character of treating patients with MFC/PIC.

Caso clínico: coroiditis serpiginosa-like con afectación macular y buena evolución tras tratamiento con adalimumab / Clinical case: Serpiginous-like choroiditis with macular involvement and good response after treatment with adalimumab

Se presenta el caso clínico de una mujer de 42 años, que consultó por disminución de visión del ojo izquierdo de 2 meses evolución, asociada a coroiditis multifocal. Se realizaron diferentes pruebas diagnósticas para descartar enfermedad infecciosa, al igual que se realizó una vitrectomía diagnóstica, que resultó negativa. Se encontró QuantiFERON® positivo y se realizó el diagnóstico de coroiditis serpiginosa-like (CS-L). Se inició tratamiento antituberculoso con empeoramiento de la visión y de la coroiditis multifocal. Una vez que se agregaron inmunoterapia convencional y tratamiento biológico, la paciente presentó mejoría significativa de la visión. Para concluir, en casos de CS-L no solo es necesario el tratamiento de la infección, sino también del componente inflamatorio, sea con inmunoterapia convencional o inmunosupresión con biológicos, y así disminuir el número de reactivaciones mejorando el pronóstico visual (AU)

Clinical case of a 42-year-old woman who consulted for decreased vision in the left eye over 2months, associated with multifocal choroiditis. Different diagnostic tests were performed to rule out infectious pathologies, as well as a diagnostic vitrectomy test, which was negative. QuantiFERON® was found to be positive and a diagnosis of serpiginous-like choroiditis (SLC) was made. Anti-tuberculosis treatment was started with worsening of vision and multifocal choroiditis. Once conventional immunotherapy and biological treatment were added, the patient presented a significant improvement in vision. To conclude, in cases of SLC, it is not only necessary to treat the infection but also the inflammatory component either with steroids or conventional immunotherapy or immunosuppression with biologics, thus reducing the number of reactivations and improving the visual prognosis (AU)

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.

PURPOSE: To report nine cases of multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. METHODS: A retrospective observational case series of eyes with multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. Multimodal imaging findings were reviewed and presented. RESULTS: Fifteen eyes of 9 patients (6 women and 3 men), with a mean age of 48.1 years (median, 46 years; range, 23-74 years), presented with multifocal choroiditis serpiginous-like peripapillary chorioretinal atrophy. All 15 eyes presented with serpiginoid peripapillary changes and had discrete patches of atrophy or punched-out scars in the posterior pole or periphery. Eleven eyes (73.3%) had cone-shaped retinal pigment epithelium elevations on optical coherence tomography, 10 eyes (66.7%) had mild vitritis, and 4 eyes (26.7%) had peripheral curvilinear streak lesions. Three eyes (20%) had choroidal neovascularization. All patients responded well to treatment with systemic immunosuppression, local corticosteroid injections, and/or intravitreal anti-vascular endothelial growth factor injections. CONCLUSION: Multifocal choroiditis may present with peripapillary chorioretinal changes resembling a serpiginous-like choroiditis in addition to the classic findings of patches of atrophy or punched-out scars in the posterior pole or periphery, cone-shaped retinal pigment epithelium elevated on optical coherence tomography and peripheral curvilinear streak lesions.

Outcomes of adalimumab therapy in refractory punctate inner choroidopathy and multifocal choroiditis.

PURPOSE: This study aims to evaluate the outcomes of adalimumab (ADA) therapy in patients with refractory punctate inner choroidopathy (PIC) and multifocal choroiditis (MFC). METHODS: Demographic and clinical data, including LogMAR best-corrected visual acuity (BCVA), were retrospectively collected. Doses of prednisone, immunomodulatory therapies (IMT), and anti-vascular endothelial growth factor (VEGF) injections before and after baseline (ADA initiation) were recorded, as well as the time to clinical remission, time to first flare, and drug-associated adverse events. RESULTS: Seven patients (4 females, ten eyes) were included. The mean follow-up after baseline was 17.8 ± 11.1 months (range 6-33). The mean LogMAR BCVA was 0.35 ± 0.77 at 6 months before baseline and remained stable throughout 12 months after baseline (0.31 ± 0.46 at 12 months; p = 0.47). The mean dose of prednisone decreased from 17.3 ± 19.6 mg/day 6 months before baseline (range 0-60) to 2.6 ± 2.4 mg/day at the last follow-up (range 0-6, p = 0.03). The mean number of flares decreased significantly from 1.43 ± 0.79 over a 6-month period before baseline to 0.2 ± 0.45 (p = 0.02) at 6-12 months after baseline. The mean number of anti-VEGF injections was 4.17 ± 3.92 over the 12-month period before baseline, and it was 2.17 ± 3.06 (p = 0.31) during the first 12 months after baseline. No adalimumab-related adverse events were noted. CONCLUSION: Adalimumab therapy for refractory PIC/MFC enabled a significant steroid-sparing effect, decreased disease flares, and preserved vision over a mean follow-up of 17.8 months.

Clinical profile, multimodal imaging, and treatment response in macular serpiginous choroiditis.

PURPOSE: To describe the clinical profile, multimodal imaging, and treatment response in macular serpiginous choroiditis (MSC). METHODS: Clinical records of 16 eyes (14 patients) with MSC presenting to a tertiary eye care institute between 2015 and 2019 were analyzed retrospectively. RESULTS: Mean age of 14 patients presenting with MSC was 33 ± 13 yrs with 64% males and 36% females. Mean visual acuity of the eyes with MSC at presentation was 0.43 ± 0.46 (logMAR) improving to 0.16 ± 0.28 (logMAR) at final visit. Thirteen eyes (81.3%) had active lesion at presentation. Mantoux test was positive in seven patients (50%) and QuantiFERON TB gold test positive in 10 patients (71%). HRCT chest showed latent tuberculosis in seven patients (50%). All patients underwent multimodal imaging. All patients received oral steroids as treatment therapy; 11 patients also received immunosuppressives, nine patients received additional anti-tubercular therapy (ATT). Mean duration of follow-up for the patients was 18 ± 10 months. A total of eight (50%) eyes had recurrence of lesions after an average duration of 14 ± 14 (3-36) months and were restarted on the treatment as per the requirement. At final follow-up, all eyes showed a good response to treatment and had healed lesions. Comparing the final BCVA to the initial BCVA, 38% (n = 6) showed improvement, 56% (n = 9) remained stable, and 6% (n = 1) eyes worsened at the final follow-up. CONCLUSION: Clinical profile and presentation of MSC is similar to that of CSC, and combination treatment with intravenous methyl prednisolone (IVMP), steroids, immunosuppressives, and ATT can salvage vision. A high suspicion of associated tuberculosis in endemic regions should be kept in mind.

Serpiginous choroiditis presenting after SARS-CoV-2 infection: A new immunological trigger?

INTRODUCTION: To report the first case of a serpiginous choroiditis presenting after SARS-CoV-2 infection in a previously healthy young woman. CASE DESCRIPTION: A 41-year-old woman reported blurry vision OS 1 month after a mild SARS-CoV-2 infection. Left eye fundus examination revealed multiple peripapillary atrophic lesions, adjacent to a larger diffuse, ill-defined, yellow-whitish deep amoeboid-like patch, involving the peripapillary region and extending temporally to the fovea. Multimodal imaging including fluorescein angiography, indocyanine-green angiography, fundus autofluorescence and optical coherence tomography was consistent with serpiginous choroiditis. A complete systemic work-up was performed to exclude potential infectious or inflammatory etiologies. The active choroidal lesions responded to high dose corticosteroids, with functional improvement. Immunomodulatory therapy with methotrexate was initiated for long-term management. CONCLUSION: Serpiginous choroiditis is a rare but important sight-threatening condition that has been previously associated to viral infections, which seem to have a role in the induction and/or perpetuation of choroidal inflammation. SARS-CoV-2 infection appears to have played a role as a possible trigger for intraocular inflammation in this case. Therefore, COVID-19 patients reporting visual symptoms should be carefully evaluated in order to obtain adequate ophthalmological management to avoid irreversible visual damage.

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

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.