Retinal microvascular changes in patients with familial mediterranean fever: a study based on optical coherence tomography angiography

Erdem, Seyfettin; Karahan, Mine; Ava, Sedat; Dursun, Mehmet Emin; Cevik, Figen Ceylan; Özkul, Emin; Keklikci, Ugur; Çevik, Remzi

doi:10.5935/0004-2749.20220038

Brasil

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Original Article • Arq. Bras. Oftalmol. 85 (4) • 2022 • https://doi.org/10.5935/0004-2749.20220038 linkcopy

Retinal microvascular changes in patients with familial mediterranean fever: a study based on optical coherence tomography angiography

Alterações microvasculares da retina em pacientes com febre mediterrânea familiar: estudo baseado em angiotomografia de coerência óptica

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Purpose: In this study, we aimed to show whether a difference exists between retinal and choroidal microcirculation findings between patients with familial Mediterranean fever and healthy controls.

Methods: Thirty-two patients with familial Mediterranean fever and 30 healthy controls were included in the study. All the patients underwent a complete ophthalmologic examination, including best-corrected visual acuity and intraocular pressure measurement. The AngioVue optical coherence tomography angiography device (Optovue, Fremont, CA) with split-spectrum amplitude-decorrelation angiography was used to evaluate and examine the retinal microvascular structure. Three-dimensional en face Optical coherence tomography angiography images were obtained by examining the macula using the 3 x 3 mm scanning protocol in the Angio Retina mode and the optic nerve using the 3 x 3 mm scanning protocol in the Angio Disk mode. All the patients' right eyes were examined.

Results: A total of 62 subjects were included in the study, of whom 32 (53.3%) were female and 30 (46.7%) were male. No statistically significant difference was found between the two groups in terms of optic nerve head or radial peripapillary capillary vessel density. On examination, the superficial capillary plexuses were statistically similar between the two groups, but the deep capillary plexus vessel density in the parafovea, superior hemi, temporal, and superior areas were significantly lower in the patients with familial Mediterranean fever.

Conclusions: We found that the capillary plexus vessel density was significantly lower in the parafovea, superior hemi, temporal, and superior regions in the patients with familial Mediterranean fever than in the control group. Therefore, OCTA, a noninvasive study, may be useful for understanding the systemic effects of familial Mediterranean fever.

Keywords: Optical coherence tomography angiography; Familial Mediterranean fever; Retinal microcirculation; Superficial plexus; Deep vascular capillary plexus

Characteristic	Patients with FMF (n=32)	Control (n=30)	Significant (pvalue)
Age (years), mean ± SD	30.65 ± 8.64	34.10 ± 6.84	0.089
Sex, n (%)
Female (30, 48.4)	16 (50.0)	14 (46.7)	0.797
Male (32, 51.6)	16 (50.0)	16 (53.3)
VAS score	7.24 ± 1.99	-	-
Disease severity score	6.07 ± 2.54	-	-
Medication (%)	Colchicine (100)
Medication (%)	Biologic agent (9.4)
Disease duration (years)	15.00 ± 7.69	-	-
Diagnostic delay (years)	6.41± 7.73

MEFV mutation	Homozygous n (%)	Heterozygous n (%)	Compound heterozygous n (%)
M694V	4 (12,5)	7 (21,9)	5 (15,6)
V726A	1 (3,12)	4 (12,5)	1 (3,12)
M680I	3 (9,37)	-	-
E148Q	-	2 (6,25)	1 (3,12)
R202Q	-	2 (6,25)	2 (6,25)

Characteristic	ONH-VD^* * = Optic nerve head (ONH-VD) and			RPC-VD^ = radial peripapillary capillary density (RPC-VD) of the patients included in the study (%).
Characteristic	Patients with FMF	Control	Significant (p)	Patients with FMF	Control	Significant (p)
Whole Image	61.00 ± 2.16	60.43 ± 3.15	0.41	59.34 ± 2.35	58.83 ± 2.98	0.45
Inside disk	57.76 ± 4.08	56.05 ± 5.31	0.15	50.33 ± 9.34	50.04 ± 7.54	0.89
Peripapillary	63.30 ± 2.42	63.68 ± 2.98	0.57	65.08 ± 3.12	65.17 ± 3.35	0.90
Nasal	61.93 ± 2.89	62.50 ± 4.02	0.52	62.72 ± 3.46	63.35 ± 5.24	0.57
Inferonasal	64.26 ± 4.43	64.70 ± 5.00	0.71	65.89 ± 4.72	65.63 ± 5.67	0.84
Inferotemporal	66.64 ± 4.51	65.96 ± 4.44	0.55	69.48 ± 5.17	68.82 ± 4.50	0.59
Superotemporal	63.75 ± 4.66	65.00 ± 4.33	0.28	67.46 ± 4.52	68.33 ± 4.80	0.46
Superonasal	62.07 ± 4.87	63.64 ± 4.96	0.21	62.22 ± 5.97	64.07 ± 5.28	0.20
Temporal	63.47 ± 3.41	62.95 ± 4.80	0.62	65.75 ± 4.17	64.44 ± 4.75	0.25

Characteristic	SCP-VD			DCP-VD
Characteristic	Patients with FMF	Control	Significant (p value)	Patients with FMF	Control	Significant (p value)
Whole Image	53.81 ± 2.22	53.99 ± 1.75	0.71	60.32 ± 1.51	60.88 ± 1.63	0.16
Fovea	31.99 ± 6.24	30.05 ± 5.21	0.19	32.94 ± 6.66	29.86 ± 6.38	0.07
Parafovea	56.04 ± 2.71	56.38 ± 1.78	0.56	62.90 ± 1.71	63.79 ± 1.70	0.04^* * p<0.05.
Superior hemi	55.97 ± 2.67	56.33 ± 1.51	0.51	62.81 ± 1.72	63.88 ± 1.70	0.02^* * p<0.05.
Inferior hemi	56.11 ± 2.85	56.43 ± 2.20	0.62	62.99 ± 2.04	63.68 ± 1.86	0.16
Temporal	54.65 ± 2.75	55.18 ± 1.95	0.39	61.63 ± 2.03	62.68 ± 1.77	0.03^* * p<0.05.
Superior	56.82 ± 2.95	56.79 ± 2.39	0.96	63.77 ± 1.66	64.94 ± 1.96	0.01^* * p<0.05.
Nasal	55.79 ± 2.71	55.87 ± 2.13	0.90	62.33 ± 1.84	63.17 ± 2.12	0.10
Inferior	56.94 ± 3.20	57.42 ± 2.31	0.49	63.87 ± 2.31	64.37 ± 2.01	0.36

FAZ (mm²)	Patients with FMF	Control	Significant (p)
Superficial	0.308 ± 0.88	0.281 ± 0.10	0.28
Deep	0.336 ± 0.09	0.317 ± 0.09	0.42

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[TFN1] ^*
= Optic nerve head (ONH-VD) and

[TFN2] ^**
= radial peripapillary capillary density (RPC-VD) of the patients included in the study (%).