Objective To observe the clinical features and treatment outcomes of presumed tubercular retinal vasculitis. Methods This is a retrospective non-comparative interventional clinical research. A total of nine patients (11 eyes) with major presentation of retinal vasculitis were included in this study. Patients first consulted the eye clinic and were diagnosed presumed tubercular retinal vasculitis. The patients, seven males and two females, aged from 19 to 66 years, with an average of 43.89 years. The time interval from symptoms to diagnosis ranged from two weeks to six months with an average of 76.27 days. Visual acuity, slit lamp ophthalmoscopy, fundus fluorescein angiography (FFA), optical coherence tomography (OCT), hematological and tuberculosis related investigations were examined and analyzed. All patients had standard anti-tuberculosis treatment. Treatment outcomes were followed for six to 37 months with an average of 14.11 months. Results Baseline visual acuity ranged from hand movement to 0.8 with an average of 0.28. Among 11 eyes, six presented mild to moderate vitritis, five presented as retinal vein occlusion with no obvious vitirits. Fundus examination showed six cases with retinal hemorrhage, four cases with macular edema, two with macular epiretinal membrane, and two with vitreous hemorrhage. FFA revealed 11 cases with leakage of vessels, 11 with nonperfusion area, four with macular edema, three with retinal neovascularization, and two with choroidal lesions. OCT of nine eyes suggested six eyes with retinal edema, three with macular edema, three with macular epiretinal membrane. TST of seven patients were all b positive. T-SPOT.TB of four patients were all positive. Three of eight patients who had chest X-ray or chest CT were suggested tuberculosis infection. Four to six weeks after the start of anti-tuberculosis treatment, vitritis, exudates, retinal and macular edema subsided. During follow up, inflammation was stable with no recurrence observed. The visual acuity of last follow-up ranged from 0.15 to 0.8 with an average of 0.51. Conclusions The main presentations of presumed tubercular retinal vasculitis are vitritis, retinal vein occlusion, and retinal hemorrhage. Standard anti-tuberculosis treatment can improve inflammation and retinal hemorrhage.
Objective To analyze the association between histocompatibility leukocyte antigen （HLA-A/B,HLA-DRB/DQB） alleles and Eales disease, and to explore susceptible genes and protective genes associated with Eales disease in a population of Han from ZUN YI city in Guizhou province. Methods The subjects were analyzed by casecontrol study consisted of two groups such as normal control group and Eales disease group. DNA samples from 100 healthy people and 26 patients with Eales disease were detected by polymeriase chain reaction with sequencespecific primers (PCR-SSP). The alleles of HLA-A/B and HLA-DRB/DQB from Eales disease group were compared with those from control group by SPSS 13.0. Results The distribution frequency in Eales disease was HLAA01(P=0.041, OR=20.5), A02(P=0.000, OR=54.667, OR 95%CI:11.837-252.473), B55 (P=0.047, OR=4.524; OR 95%CI:1.200-17.047), HLA-DRB01(P=0.048, OR=5.879, OR95%CI:1.227-28.169). DQB05 (P=0.021, OR=2.769, OR95%CI=1.145-6.692) alleles, and obviously higher than control, but the frequency of HLAA11 (P=0.031, OR=0.383, OR95%CI=0.158-0.930) was obviously lower than control (P＜0.05). Conclusion The results showed that the alleles of HLAA01, A02, B55, DRB01, DQB05 may associate with an antagonist effect in Eales disease, but HLAA11 may be a protective gene of this disease.
Objective To investigate the characteristics of fundus photography and fundus fluorescein angiography (FFA) of IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome and Eales disease. Methods The fundus photography and FFA data of 4 cases (8 eyes) with IRVAN syndrome and 43 cases (68 eyes) with Eales disease were retrospectively analyzed. All patients received ophthalmic routine examinations, including visual acuity, intraocular pressure, slit-lamp microscope and indirect ophthalmoscope. All patients had taken fundus photography and FFA for both eyes, except 4 patients of Eales disease who had vitreous hemorrhage in one eye. All 4 cases(1 male/3 female )with IRVAN syndrome were bilateral and aged 1643 years old( mean age 2700plusmn;1293 years old). 43 cases (32 male/11 female) of Eales disease aged 6-59 years old( mean 30.79plusmn;11.46 years old), 29 cases were bilateral and 14 cases were unilateral. Both diseases had retinal vascular whitesheath or white threadlike changes, exudative retinal hemorrhage and vitreous hemorrhage. Results Both arteries and veins of posterior pole of all eyes with IRVAN syndrome were involved and shown multiple retinal macroaneurysms. Other signs of IRVAN syndrome included capillary occlusion and nonperfusion (7/8 eyes, 87.5%),fluorescein leakage and edema of optic disc (5/8 eyes,62.5%), optic atrophy(2/8 eyes,25%), vitreous hemorrhage(1/8 eyes,12.5%), neovascularization of optic disc(2/8 eyes,25%), retinal neovascularization(4/8 eyes,50%) and macular edema(4/8 eyes,50%). The signs of Eales disease included fluorescein leakage of peripheral retinal vein (68/68 eyes, 100%), fluorescein leakage of posterior retinal vein (32/68 eyes, 47.06%), artery involvement (5/68 eyes, 7.35%), peripheral capillary occlusion and nonperfusion (38/68 eyes, 55.88%), fluorescein leakage of optic disc(29/68 eyes, 42.65%), neovascularization of optic disc(4/68 eyes,5.88%), retinal neovascularization(26/68 eyes,38.2%) and macular edema(15/68 eyes,22.06%). Compared IRVAN syndrome with Eales disease, the difference of artery inflammation, vein inflammation, retinal macroaneurysms in posterior area had statistics significance(all P=000,Plt;005), and that of edema of optic disc, retinal vascular nonperfusion area, neovascularization of optic disc, neovascularization elsewhere, and macular edema had no statistics significance(chi;2=0.479，P＞0.05；P=0.131，P＞0.05；chi;2=1.449，P＞0.05；chi;2=0.068，P＞0.05；chi;2=1.676，P＞0.05). Conclusions Both IRVAN syndrome and Eales disease may have vein and artery inflammation in posterior pole of the eye, and may result in neuroretinitis. IRVAN syndrome has much more vein and artery inflammation in posterior pole than Eales disease. Posterior retinal macroaneurysms is the most important sign for the diagnosis and differential diagnosis of IRVAN syndrome and Eales disease.
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)
ObjectiveTo analyze the clinical characteristics and renal outcome of elderly patients with antineutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) with renal involvement. MethodsWe retrospectively analyzed the clinical data of 147 patients with ANCA relate vasculitis treated between June 2006 and June 2012. Based on the age, the patients were divided into elderly group (65 years or older, n=50) and non-elderly group (younger than 65, n=97). The disease course, clinical characteristics, ANCA serological indexes, renal pathological change and prognosis of patients in the two groups were compared and studied. ResultsIn the elderly group, there were 3 cases of Wegener granulomatosis (WG), 45 of microscopic polyangiitis (MPA), and 2 of pauci-immune crescentic glomerulonephritis (PICGN). The non-elderly group had 8 cases of WG, 82 of MPA, 6 of PICGN, and 1 of allergic angitis granulomatosis. There were 5 cases of positive cANCA and 44 of positive pANCA in the elderly group. The elderly patients had significantly more pulmonary involvement than the younger patients (P=0.030). No significant difference was detected between the two groups in combined pulmonary infection (P=0.281) or combined infectious index C-reactive protein (P=0.326). Elderly patients were less likely to respond to sufficient treatment with pulse intravenous methylprednisolone therapy (P=0.035) and cyclophosphamide (P=0.043), and had worse renal outcome than younger patients (P=0.040). ConclusionElderly patients with AAV have more prevalent pulmonary involvement and have severe complication of pulmonary infection, which affects mortality and morbidity of ANCA-associated systemic vasculitis.
ObjectiveTo investigate the regulation mechanism of neutrophils extracellular traps (NETs) on inflammatory diseases and to provide guidance for the treatment of related inflammatory diseases.MethodThe literatures about the relationship between NETs and inflammatory diseases were reviewed.ResultsNETs played an important role in sepsis, anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, acute pancreatitis, inflammatory bowel disease, and other inflammatory diseases, which were related to the development or activity of the diseases. By regulating the formation of NETs pathway, reducing the production of NETs, we could ultimately reduce the inflammation of the disease.ConclusionNETs is involved in the course of sepsis, ANCA-associated vasculitis, acute pancreatitis, inflammatory bowel disease, and other inflammatory diseases, but further investigation and clinical inflammatory verification are needed.
Objective To investigate the clinical characteristics and prognosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with acute kidney injury (AKI) as the first manifestation, and provide new ideas for the prevention and treatment of this disease. Methods A retrospective analysis was performed on 144 patients diagnosed with ANCA-associated vasculitis in Affiliated Hospital of Southwest Medical University between August 2013 and March 2020. The patients were divided into AKI group and non-AKI group according to whether they were complicated with AKI at admission, and the differences in clinical characteristics were analyzed. The risk factors were screened by multiple logistic regression analysis. Results Among the 144 patients with ANCA-associated vasculitis, 30 cases (20.8%) were complicated with AKI at admission, and 70 cases (48.6%) died by the end of follow-up. There were 16 death cases (53.3%) in the AKI group, and 54 death cases (47.4%) in the non-AKI group, but the difference was not statistically significant (P>0.05). Single-factor analyses showed that in the AKI group, the pre-admission incidence of hematuria, neutrophil count, serum creatinine, systolic blood pressure, and Birmingham Vasculitis Activity Score were higher than those in the non-AKI group, while the red blood cell count and estimated glomerular filtration rate (eGFR) were lower than those in the non-AKI group, and the differences were statistically significant (P<0.05). Multiple logistic regression analysis showed that the neutrophil count [odds ratio (OR)=1.172, 95% confidence interval (CI) (1.003, 1.371), P=0.046] and eGFR [OR=0.942, 95%CI (0.907, 0.979), P=0.002] were independent influencing factors for AKI. Conclusions Elevated neutrophil count is an independent risk factor for ANCA-associated vasculitis complicated with AKI. It has certain guiding significance for clinical work. Early identification and intervention of these patients may contribute to reduce the case fatality rate and improve prognosis.
Objective To observe the multimodal imaging characteristics of the eyes in patients with presumed tuberculous retinal vasculitis. Methods A retrospective case series study. A total of 15 patients (22 eyes) diagnosed with presumed tuberculous retinal vasculitis and receiving anti-tuberculosis treatment (ATT) effectively in Department of Ophthalmology, Subei People's Hospital Affiliated to Yangzhou University from January 2018 to April 2021 were included. Among them, there were 5 males and 10 females. Seven had bilateral involvement and 8 had unilateral involvement. The age was 49.3±11.1 years old. The best corrected visual acuity (BCVA), fundus colour photography, wide-angle fundus fluorescein angiography (FFA), and optical coherence tomography (OCT) were performed in all patients. Indocyanine green angiography (ICGA) was performed in 7 eyes. The BCVA examination was performed with the international standard visual acuity chart, which was converted into the logarithm of minimal angel resolution vision (logMAR). Systemic tuberculosis-related examinations included chest CT, serum T-spot, purified protein derivative and other tuberculosis-related tests. All patients were treated with systemic anti-tuberculosis therapy. The follow-up time was ＞12 months. The multimodal imaging characteristics for affected eyes. Nonparametric test was used to compare BCVA before and after treatment. ResultsThe retinal vessels of all the affected eyes were tortuously dilated, including 3 eyes with vascular white scabbard, 5 eyes with scattered bleeding point at the retina inculding 3 eyes walking along the vessels. The lesions were mainly distributed in the middle and periphery of the retina, and some of them involved the posterior pole; 12 eyes (54.5%, 12/22) with simple retinal vasculitis and 10 eyes (45.5%, 10/22) with retinal vasculitis complicated with choroiditis. Tuberculous retinal vasculitis showed different degrees of retinal vascular leakage on FFA, mainly retinal vein and capillary leakage, not involving arteries; 16 eyes (72.7%, 16/22) of retinal vasculitis showed peripheral occlusive retinal vasculitis and 4 eyes (18.2%, 4/22) were associated with retinal neovascularization. In 10 eyes with choroiditis, there were multiple focal choroiditis lesions of different sizes under the retina. Of the 7 eyes examined by ICGA, the choroidal inflammatory lesions showed hypofluorescent dark dots (HDD) in 5 eyes (71.4%,5/7), showing HDDs of different sizes, most of which were distributed in the posterior pole and middle periphery. In 10 eyes with retinal vasculitis complicated with choroiditis after ATT, the accumulation of hyper-reflective substances above and below the retinal pigment epithelium layer of the retina was gradually absorbed, but not completely disappeared, and most of the disorders of retinal structure could not be recovered. The average logMAR visual acuity was 0.61±0.57 before treatment and 0.36±0.55 after treatment. The BCVA after treatment was significantly higher than that before treatment (Z=－3.102, P＜0.01). ConclusionsPeripheral occlusive retinal vasculitis is the most common manifestation of tuberculous retinal vasculitis in FFA, which may be accompanied by focal choroidal inflammatory lesions. Wide-angle FFA and ICGA are more important in the diagnosis of tuberculous retinal vasculitis. OCT can be used for monitoring the changes of inflammation.
Objective To present the pooled quantitative evidence of clinical features and current treatments of programmed death 1 (PD-1) / programmed death-ligand 1 (PD-L1) inhibitor-associated vasculitis. Methods Medline, Embase, EBM, CNKI, WanFang Data and VIP databases were searched for all available studies reporting PD-1/PD-L1 inhibitor-associated vasculitis till March 23, 2022. We summarized and systematically reviewed the included articles, and analyzed the data results with descriptive statistical methods. Results A total of 38 articles were included, including 43 patients. The median age [median (minimum, maximum)] was 62 (31, 89) years, and most of patients were male (64.3%). Lung cancer was the most common tumor (47.6%). The median onset time of vasculitis [median (minimum, maximum)] was 12 (1, 120) weeks after medication. Small vasculitis (62.8%) and cutaneous vasculitis (26.7%) were the most common types. The Common Terminology Criteria for Adverse Events of vasculitis was predominantly 3-4 (83.7%). After diagnosed with vasculitis, PD-1/PD-L1 inhibitors were discontinued in 81.6% of patients, and glucocorticoid was administrated in 88.4% of patients. After treatment, 90.0% of patients had significant improvement during follow-up. However, when the discontinuation of PD-1/PD-L1 inhibitors, 55.6% of patients tumor progressions, and 35.0% of patients dead. Conclusions Special attention should be paid to the occurrence of vasculitis when using PD-1/PD-L1 inhibitors for malignant tumor therapies. Stopping PD-1/PD-L1 inhibitors and using glucocorticoid are the essential methods to treat vasculitis, but the above treatments may bring a high risk of tumor progression.
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.