Objective To observe the clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Methods The clinical data of 3 patients with IRVAN syndrome which were diagnosed by systemic examination, fundus photography and fundus fluorescein angiography (FFA) were retrospectively analyzed. Results Idiopathic retinal vasculitis, which was induced by retinal arterial inflammation, multiple macroaneurysms of optic disc and retinal vessels, edema of optic disc, and exudation around the optic disc, was found in all of the 3 patients, multiple arteriolar aneurysms of optic disc and retinal vascular and exudative neuroretinitis. Two patients had peripheral retinal vascular nonperfusion area, which belonged to typical IRVAN syndrome. Conclusions The clinical characteristics of IRVAN syndrome include idiopathic retinal vasculitis which only involved in artery, multiple retinal macroaneurysms which located on the dissepiment of optic disc and retinal artery, and the neuroretinitis induced by exudation of retina and optic disc because of vasculitis and aneurysms. (Chin J Ocul Fundus Dis, 2007, 23: 180-183)
Objective To explore clinical features and risk factors of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with pulmonary involvement. Methods A retrospectively study of clinical data of 113 AAV patients with pulmonary involvement was conducted in the First Affiliated Hospital of Soochow University from January 2015 to December 2020. The differences in general characteristics, treatment and prognosis of different types of AAV with pulmonary involvement were compared. In addition, the clinical characteristics and survival status between the pulmonary involvement group and the non-pulmonary involvement group (n=69) were analyzed. Multivariate logistic regression model was used to analyze the risk factors. Results A total of 113 patients (57 males and 56 females) of AAV with pulmonary involvement were enrolled, including 86 cases of microscopic polyangiitis (MPA), 21 cases of granulomatosis polyangiitis (GPA), 6 cases of eosinophilia granulomatosis (EGPA). The average age was (67±11) years old. There was no significant difference in the age and gender distribution. The clinical manifestations were non-specific. Interstitial lung disease was common imaging feature of MPA, multiple nodules or mass was common in GPA, the incidence of sinusitis in EGPA was significantly higher (P<0.05). Seventy-three patients were complicated with extrapulmonary involvement. The most common organ involved was the kidney, followed by the cardiovascular and nervous system. The most important organs involved in MPA, EGPA and GPA were kidney, heart, and ear, nose, throat respectively. Compared with the non-pulmonary involvement group, the proportions of Birmingham vasculitis activity score (BVAS) ≥15 points, higher antineutrophil cytoplasmic antibody titer and lower complement C3 or C4, pulmonary infection, mechanical ventilation and plasmapheresis in the pulmonary involvement group were significantly higher (P<0.05). Forty patients died during the follow-up. One-year cumulative survival was further calculated using the Kaplan-Meier method, which demonstrated that pulmonary involvement was a risk factor for higher mortality in AAV patients. Compared with the survival group, the proportions of coronary heart disease, multiple organs involvement (n≥3), BVAS≥15 points, serum creatinine≥500 μmol/L, hemoglobin≤90 g/L, C-reactive protein≥10 mg/L, pulmonary infection, requiring mechanical ventilation, continuous renal replacement therapy and plasmapheresis in the death group were significantly higher (P<0.05). Conclusions AAV with pulmonary involvement is more common in the elderly, the morbidity is similar between male and female, and the clinical manifestations are usually non-specific. The chest imaging manifestations are mainly pulmonary interstitial changes, multiple nodules and masses. Multiple organs involvement occurs more often. BVAS≥15 points is independent risk factor for pulmonary involvement in AAV patients. The prognosis of AAV patients with pulmonary involvement is relatively poor. Combined with coronary heart disease, pulmonary infection and CRP≥10 mg/L are independent risk factors of poor prognosis.
ObjectiveTo observe and analyze the multimodal imaging characteristics of fundus in patients with idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome. MethodsA retrospective study. From June 2015 to March 2024, 6 patients (11 eyes) diagnosed with IRVAN syndrome in Shaanxi Eye Hospital were included in the study. All patients underwent examinations including best-corrected visual acuity (BCVA), color fundus photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT) and OCT angiography (OCTA). At the same time, FFA combined with indocyanine green angiography (ICGA) was performed in 6 eyes. Follow-up ranged from 2 to 23 months. Multimodal imaging features were analyzed retrospectively. The number of retinal aneurysms detected by FFA, ICGA, and OCTA was compared by using the Wilcoxon signed-rank test. ResultsIn 11 eyes of 6 cases, a total of 1 male (2 eyes) and 5 females (9 eyes) with the mean age of (31.67±12.91) years were included in this cohort. Color fundus photography showed clear optic disc boundaries in 5 eyes, optic disc aneurysms in 8 eyes, retinal aneurysms in 4 eyes; exudation in 9 eyes, localized around aneurysms. On OCT, vitreous high reflective dots and epiretinal membrane on optic disc in all 11 eyes, and macular epiretinal membrane in 3 eyes were revealed. FFA showed optic disc aneurysms and retinal aneurysms in 9 eyes, late optic disc hyperfluorescence in 11 eyes; local arterial leakage in 3 eyes, local venous leakage in 8 eyes, non-perfusion area in all 11 eyes, and retina neovascularization in 3 eyes. Optic disc aneurysms and retinal aneurysms in 5 eyes (total 18 aneurysms) on ICGA were shown compared with optic disc aneurysms in 4 eyes and retinal aneurysms in 5 eyes (total 13 aneurysms) on simultaneous FFA. OCTA revealed neovascularization on the optic disc in 2 eyes, optic disc aneurysmsin 8 eyes, retinal aneurysms in 1 eye (total 2 aneurysms); while on simultaneous FFA, optic disc aneurysms in 8 eyes and retinal aneurysms in 3 eyes (total 5 aneurysms) with no optic disc were displayed. During OCTA follow-up, new aneurysms appeared at the bifurcation of arteries with an increasing angle between them and non-perfusion area enlargement on FFA. Compared with FFA and ICGA, OCTA in detecting the number of aneurysms had no statistics significance (Z=−1.342, −1.342; P>0.05). ConclusionMultimodal imaging can demonstrate characteristics of IRVAN syndrome, ICGA provides superior visualization of optic disc and retinal aneurysms, while OCTA confirms optic disc neovascularization and enlargement of artery angles at arterial bifurcations.