Korean guidelines for the management of gout

Gout is the most common form of arthritis, with the prevalence increasing worldwide. The present treatment guidelines provide recommendations for the appropriate treatment of acute gout, management during the inter-critical period, and prevention of chronic complications. The guidelines were developed based on evidence-based medicine and draft recommendations finalized after expert consensus. These guidelines are designed to provide clinicians with clinical evidence to enable efficient treatment of gout.

Korean guidelines for the management of gout

Gout is the most common form of arthritis, with the prevalence increasing worldwide. The present treatment guidelines provide recommendations for the appropriate treatment of acute gout, management during the inter-critical period, and prevention of chronic complications. The guidelines were developed based on evidence-based medicine and draft recommendations finalized after expert consensus. These guidelines are designed to provide clinicians with clinical evidence to enable efficient treatment of gout.

Long-term Clinical Course and Prognostic Factor of Branch Retinal Artery Occlusion

Purpose@#To investigate the long-term clinical course and prognostic factors of branch retinal artery occlusion (BRAO). @*Methods@#The medical records of patients diagnosed with BRAO were reviewed retrospectively. Visual acuity (VA) and central retinal thickness (CRT) at diagnosis were compared with those measured at the final visit. Patients with a decimal VA ≥ 0.6 (good prognosis group) were compared with those with a decimal VA ≤ 0.5 (poor prognosis group) at the final visit. @*Results@#Fifty-five patients were enrolled and the mean follow-up period was 45.8 ± 27.8 months. The mean logarithm of minimum angle of resolution improved from 0.53 ± 0.57 at diagnosis to 0.36 ± 0.61 at the final visit (p = 0.026). The decimal VA was ≤ 0.1 in 13 (23.6%) patients, ≥ 0.2 and ≤ 0.5 in 16 (29.1%) patients, and ≥ 0.6 in 26 (47.3%) patients at diagnosis; the respective values were 9 (16.4%), 8 (14.5%), and 38 (69.1%) at the final visit. The mean CRT significantly decreased from 273.9 ± 34.7 µm at diagnosis to 248.9 ± 27.0 µm at the final visit (p < 0.001). The poor prognosis group (n = 17) was older (p = 0.044) and had a higher incidence of papillomacular bundle involvement (p < 0.001) than the good prognosis group (n = 38). @*Conclusions@#Patients with BRAO generally showed relatively favorable long-term outcomes. However, the final VA was ≤ 0.1 in 16.4% of them, suggesting the need for further treatment modalities to improve the outcome of patients with a poor prognosis.

Course of Neovascular Age-related Macular Degeneration that Showed Limited Response to Both Ranibizumab and Aflibercept

Purpose@#To evaluate the clinical course of neovascular age-related macular degeneration (nAMD) that has shown limited response to ranibizumab and aflibercept. @*Methods@#This retrospective study included 14 eyes with nAMD that showed a limited response to initial treatment using ranibizumab and aflibercept. The changes in visual acuity (VA) during the follow-up period and the incidence and timing of VA deterioration to the level of 0.1 or worse were identified. In cases involving bevacizumab treatment, the follow-up duration and the proportion of patients maintaining a VA of 0.2 or better were identified. @*Results@#During the mean 43.3 ± 33.2 months of follow-up, the VA deteriorated significantly from a mean logarithm of minimum angle of resolution (logMAR) of 0.42 ± 0.34 at diagnosis to 0.91 ± 0.68 at the final follow-up (p = 0.021). A logMAR of 0.3 or greater degree of VA deterioration was noted in six eyes (42.9%). Among the 13 eyes exhibiting a VA of 0.2 or better after the initial treatment, a deterioration to a VA of 0.1 or worse was noted in 6 eyes (46.2%) at a mean 29.7 ± 18.3 months. In eight eyes undergoing bevacizumab treatment, four eyes (50.0%) maintained 0.2 or better VA during the 51.4 ± 35.5 months of follow-up. @*Conclusions@#The clinical course of patients with limited response to ranibizumab and aflibercept was generally unfavorable. However, a relatively long period was required for the deterioration in VA to reach 0.1 or worse. In addition, 0.2 or better VA was maintained in approximately half of the patients.

Effect of Stent Placement on Survival in Patients with Malignant Portal Vein Stenosis:A Propensity Score–Matched Study

Objective@#Percutaneous portal vein (PV) stent placement can be an effective treatment for symptoms associated with portal hypertension. This study aimed to evaluate the effect of PV stenting on the overall survival (OS) in patients with malignant PV stenosis. @*Materials and Methods@#Two groups of patients with malignant PV stenosis were compared in this retrospective study involving two institutions. A total of 197 patients who underwent PV stenting between November 2016 and August 2019 were established as the stent group, whereas 29 patients with PV stenosis who were treated conservatively between July 2013 and October 2016 constituted the no-stent group. OS was compared between the two groups before and after propensity score matching (PSM). Risk factors associated with OS were evaluated using the Cox proportional hazards model. Procedureassociated adverse events were also evaluated. @*Results@#The stent group finally included 100 patients (median age, 65 [interquartile range, 58–71] years; 64 male). The nostent group included 22 patients (69 [61–75] years, 13 male). Stent placement was successful in 95% of attempted cases, and the 1- and 2-year stent occlusion–free survival rate was 56% (95% confidence interval, 45%–69%) and 44% (32%–60%), respectively. The median stent occlusion–free survival time was 176 (interquartile range, 70–440) days. OS was significantly longer in the stent group than in the no-stent group (median 294 vs. 87 days, p < 0.001 before PSM, p = 0.011 after PSM).The 1- and 3-year OS rates before PSM were 40% and 11%, respectively, in the stent group. The 1-year OS rate after PSM was 32% and 5% in the stent and no-stent groups, respectively. Anemia requiring transfusion (n = 2) and acute thrombosis necessitating re-stenting (n = 1) occurred in three patients in the stent group within 1 week. @*Conclusion@#Percutaneous placement of a PV stent may be effective in improving OS in patients with malignant PV stenosis.

Anti-anger Effects of Herbal Medicine: A Mini-Review of Rat Studies / 中国结合医学杂志

OBJECTIVE@#To analyze the available data on the anti-anger effects of herbal medicines (HMs) as well as their underlying mechanisms in rat models.@*METHODS@#From 6 electronic databases [PubMed, EMBASE, China National Knowledge Infrastructure (CNKI), Wanfang, Oriental Medicine Advanced Searching Integrated System (OASIS), and Research Information Sharing Service (RISS)], relevant animal experiments were searched by using "anger," "rats," and "animal" as search keywords. The last search was conducted on November 22, 2019, and all experiments involving rat models of anger and treatment using HMs published until the date of the search were considered.@*RESULTS@#A total of 24 studies with 16 kinds of HMs were included. Most studies have used the "tail irritating method" and "social isolation and resident intruder" method to establish anger models. According to the included studies, the therapeutic mechanisms of HMs for anger regulation and important herbs by their frequency and/or preclinical evidence mainly incladed regulation of hemorheology (Bupleuri Radix, Paeoniae Radix Alba, and Glycyrrhizae Radix), regulation of sex hormones (Bupleuri Radix, Cyperi Rhizoma, and Paeoniae Radix Alba), regulation of neurotransmitters (Cyperi Rhizoma), regulation of anger-related genes (Bupleuri Radix, Glycyrrhizae Radix, and Paeoniae Radix Alba), and other effects. Overall, Liver (Gan) qi-smoothing herbs including Bupleuri Radix and Cyperi Rhizoma were the most frequently used.@*CONCLUSIONS@#This review found the frequent methods to establish an anger model, and major mechanisms of anti-anger effects of HMs. Interestingly, some Liver qi-smoothing herbs have been frequently used to investigate the anti-anger effects of HM. These findings provide insight into the role and relevance of HMs in the field of anger management.

Outcome-Based Decision-Making Algorithm for Treating Patients with Primary Aldosteronism

Background@#Optimal management of primary aldosteronism (PA) is crucial due to the increased risk of cardiovascular and cerebrovascular diseases. Adrenal venous sampling (AVS) is the gold standard method for determining subtype but is technically challenging and invasive. Some PA patients do not benefit clinically from surgery. We sought to develop an algorithm to improve decision- making before engaging in AVS and surgery in clinical practice. @*Methods@#We conducted the ongoing Korean Primary Aldosteronism Study at two tertiary centers. Study A involved PA patients with successful catheterization and a unilateral nodule on computed tomography and aimed to predict unilateral aldosterone-producing adenoma (n=367). Study B involved similar patients who underwent adrenalectomy and aimed to predict postoperative outcome (n=330). In study A, we implemented important feature selection using the least absolute shrinkage and selection operator regression. @*Results@#We developed a unilateral PA prediction model using logistic regression analysis: lowest serum potassium level ≤3.4 mEq/L, aldosterone-to-renin ratio ≥150, plasma aldosterone concentration ≥30 ng/mL, and body mass index <25 kg/m2 (area under the curve, 0.819; 95% confidence interval, 0.774 to 0.865; sensitivity, 97.6%; specificity, 25.5%). In study B, we identified female, hypertension duration <5 years, anti-hypertension medication <2.5 daily defined dose, and the absence of coronary artery disease as predictors of clinical success, using stepwise logistic regression models (sensitivity, 94.2%; specificity, 49.3%). We validated our algorithm in the independent validation dataset (n=53). @*Conclusion@#We propose this new outcome-driven diagnostic algorithm, simultaneously considering unilateral aldosterone excess and clinical surgical benefits in PA patients.

Changes in Human Gene Expression After Sleep Deprivation

Objectives@#Sleep is fundamental to maintaining homeostatic control and has behavioral and psychological effects on humans. To better understand the function and pathophysiology of sleep, specific gene expressions in reference to sleep deprivation have been studied. In this study, we investigated the gene expression of peripheral blood mononuclear cells after sleep deprivation to better understand the functional consequence of sleep. @*Methods@#In eight healthy men, 24 h sleep deprivation was induced. Blood was sampled at 14:00, before and after sleep deprivation.mRNA was isolated and analyzed via microarrays. cDNAs before and after sleep deprivation were coupled to Cy3 or Cy5, respectively, and normalized cDNAs were selected with a ratio greater than two as a significant gene. Results are expressed as mean. @*Results@#Among 41174 transcripts, 38852 genes were selected as reliable, and only a small minority (< 1%) of the genes were up-or down-regulated. Total six and eleven genes were selected as significant upregulated and downregulated genes, respectively. Protein tyrosine phosphatase receptor type O was most upregulated (6.9-fold), and low-density lipoprotein receptor-related protein 5-like protein showed the most substantial inhibition (0.06-fold). @*Conclusions@#This study showed significant associations between sleep deprivation and the immune system. Acute sleep deprivation affects pathways in proinflammatory cytokines as well as metabolic pathways of glutamate and purine, neurotransmitters related to sleep and wake cycle.

Clinical Characteristics at Initial Diagnosis of Korean Patients with Retinitis Pigmentosa

Purpose@#To evaluate the clinical characteristics of Korean patients diagnosed with retinitis pigmentosa. @*Methods@#We retrospectively reviewed the medical records of patients diagnosed with retinitis pigmentosa from January 2014 to December 2019. We evaluated age, gender, the chief complaints, posterior subcapsular cataract status, abnormalities on optical coherence tomography, visual field test results, and electrooculograms. @*Results@#A total of 492 eyes of 246 patients were included. The mean patient age was 48.0 ± 16.0 years and the chief complaints were decreased vision and night blindness. The mean logarithm of the minimal angle of resolution (logMAR) best‐corrected visual acuity (BCVA) was 0.31 ± 0.50. The BCVA was 0.5 or better in 368 eyes (74.8%). A total of 328 (71.0%) of 462 eyes that underwent visual field testing exhibited visual field defects within 10º. The mean Arden ratio was 1.28 ± 0.28 for the 242 eyes that underwent electro‐oculography. Optical coherence tomography revealed vitreomacular traction/an epiretinal membrane, cystoid macular edema, and retinal thinning in 135 (27.4%), 48 (9.8%), and 112 (22.8%) eyes, respectively. The ellipsoid zone was intact in 222 eyes (45.1%), disrupted in 220 (44.7%), and absent in 50 (10.2%). @*Conclusions@#Most patients with retinitis pigmentosa exhibited visual acuity of 0.5 or better, but also had central visual field defects. Various abnormalities were noted on optical coherence tomography of most patients.

Update on Transradial Access for Percutaneous Transcatheter Visceral Artery Embolization

Transfemoral access (TFA) is a widely used first-line approach for most peripheral vascular interventions. Since its introduction in cardiologic and neurointerventional procedures, several advantages of transradial access (TRA) over TFA have been demonstrated, such as patient preference, lower complication rates, early ambulation, and shorter hospital stay. However, studies reporting the safety and efficacy of this approach for peripheral vascular interventions performed by interventional radiologists are relatively few. This review aimed to summarize the technique and clinical applications of TRA in percutaneous transcatheter visceral artery embolization and the management of complications.

Liver Directed Interventional Treatments for Pancreatic Neuroendocrine Tumor with Liver Metastasis / 대한췌담도학회지

Pancreatic neuroendocrine tumors (PNETs) are rare, but the frequency of detection is constantly increasing due to recent advances in diagnostic technology. Since liver metastasis (LM) of PNETs is highly correlated with long-term survival, active treatment is important. Liver-directed treatment is recommended for patients with unresectable LM from PNET if symptomatic or progressing despite medical management. Liverdirected intervention treatment, including locally ablative techniques and hepatic arterial embolotherapy has a vital role in controlling symptoms and improving overall survival rates. The purpose of this article is to address the recent advances in liverdirected intervention treatments for the treatment of LM of PNETs.

Imaging Features and Interventional Treatment for Liver Injuries and Their Complications

Liver injury is a common consequence of blunt abdominopelvic trauma. Contrast-enhanced CT allows for the rapid detection and evaluation of liver injury. The treatment strategy for blunt liver injury has shifted from surgical to nonoperative management, which has been widely complemented by interventional management to treat both liver injury and its complications. In this article, we review the major imaging features of liver injury and the role of interventional management for the treatment of liver injury.

Outcomes of Re-fixation after the First Intraocular Lens Scleral Fixation

Purpose@#To investigate the outcomes of re-fixation after the first intraocular lens (IOL) scleral fixation. @*Methods@#We retrospectively reviewed the charts of patients who underwent second IOL scleral fixation and vitrectomy for dislocation of IOL after the first IOL scleral fixation. We compared the best-corrected visual acuity (BCVA) and spherical equivalent (SE) after 1 month of the first and second surgery, and noted the complications. @*Results@#We included 21 eyes that underwent second IOL scleral fixation: 13 eyes (61.9%) with IOL exchange and eight (38.1%) with one-haptic fixation. Mean BCVAs (LogMAR) were 0.17 ± 0.25 and 0.11 ± 0.23 after the first and second surgery, respectively (p = 0.073); mean SEs were -0.94 ± 1.69 and -0.58 ± 1.46 diopters after the first and second surgery, respectively (p = 0.076). Postoperative complications occurred in eight eyes (38.1%), including temporarily increased intraocular pressure and suture knots exposure. However, none of the complications required re-operation. @*Conclusions@#The outcomes of primary and secondary IOL fixation were similar, and there were no serious complications of the second surgery.

Limited Treatment Response during Follow-up after Switching to Aflibercept in Neovascular Age-related Macular Degeneration

Purpose@#To investigate the limited response to aflibercept after switching to aflibercept in neovascular age-related macular degeneration (AMD). @*Methods@#This retrospective study included 70 eyes with neovascular AMD that were initially treated with ranibizumab and then switched to aflibercept. The incidence and timing of the limited response to aflibercept were identified and visual outcome was compared between eyes with and without limited response. In addition, factors predictive of limited response were analyzed. @*Results@#A limited response to aflibercept was noted in approximately 1/5 of the patients who underwent switching to aflibercept in neovascular AMD. Switching to aflibercept was performed at a mean of 16.2 ± 12.7 months after diagnosis. During the mean 34.7 months of follow-up after switching, limited response was noted in 15 eyes (21.4%) at a mean of 22.0 ± 13.9 months after switching. The degree of reduction in visual acuity was mean logMAR 0.34 ± 0.41 in eyes with limited response and mean 0.06 ± 0.20 in eyes without (p = 0.002). In addition, the duration between the diagnosis and the switching was shorter (p = 0.012), and the number of ranibizumab injections before switching was lower (p = 0.016) in eyes with limited response than in eyes without. @*Conclusions@#Patients who showed limited response to aflibercept after switching to aflibercept showed a worse visual outcome. The probability of having a limited response is higher when the switching is performed earlier.

Long-term Clinical Outcomes of Macular Hole Surgery Using Internal Limiting Membrane Flap or Insertion

Purpose@#To evaluate the long-term outcomes of inverted internal limiting membrane (ILM) flap and ILM insertion in idiopathic macular hole (MH) and to compare the outcomes between the two techniques. @*Methods@#We retrospectively reviewed the medical records of 17 eyes that underwent an inverted ILM flap procedure (flap group, n = 9) or ILM insertion procedure (insertion group, n = 8) for idiopathic MH. Within each group, best-corrected visual acuity (BCVA) before surgery was compared with that at the final follow-up. The BCVA at the final follow-up was compared between the two groups. In addition, the incidence of restoration of the external limiting membrane (ELM) and ellipsoid zone was evaluated. @*Results@#The mean follow-up period was 25.1 ± 13.7 months after surgery. All MHs were closed after the surgery. In all 17 eyes, the logarithm of minimal angle of resolution BCVA had improved significantly from a mean value of 0.88 ± 0.23 before surgery to 0.42 ± 0.23 at the final follow-up (p < 0.001). The BCVA was significantly improved in both the flap group (from 0.92 ± 0.25 to 0.37 ± 0.29, p = 0.007) and the insertion group (from 0.83 ± 0.19 to 0.48 ± 0.15, p = 0.018). There was no significant difference in BCVA at the final follow-up between the two groups (p = 0.075). The incidence of restoration of the ELM and ellipsoid zone was significantly higher in the flap group (seven eyes, 77.8%) than in the insertion group (one eye, 12.5%). @*Conclusions@#Both inverted ILM flap and ILM insertion are effective in MH treatment. The restoration of retinal outer layers was better in the flap group. Further studies with a larger study population are needed to evaluate the long-term visual outcomes of the two methods.

The Characteristics and the Clinical Courses of Best Disease in Korean Patients

Purpose@#To evaluate the clinical characteristics and courses of Best disease in Korean patients. @*Methods@#We retrospectively reviewed the medical records of patients with Best disease followed-up for more than 12 months after diagnosis. The disease stage and best-corrected visual acuity (BCVA) at diagnosis, and stage progression during follow-up, were evaluated. The BCVA at diagnosis was compared to that at the final follow-up. The BCVA changes were compared between those evidencing stage progression and not. @*Results@#A total of 16 patients (32 eyes) were enrolled. The mean follow-up period was 52.1 ± 31.8 months. At diagnosis, three eyes had stage 1, nine stage 2, seven stage 3, 11 stage 4, and two stage 5 disease. During follow-up, stage progression was observed in nine eyes (28.1%). The mean logarithm of the minimal angle of resolution (logMAR) BCVA significantly worsened from 0.19 ± 0.19 at baseline to 0.30 ± 0.27 at the final follow-up (p = 0.004). In six eyes (18.8%), a deterioration of logMAR 0.2 or greater was noted. The extent of deterioration was greater in the nine eyes (0.25 ± 0.26) exhibiting stage progression than in the 24 eyes (0.05 ± 0.08) lacking such progression (p = 0.001). @*Conclusions@#The visual acuity of Best disease patients was relatively good at diagnosis. However, stage progression and visual deterioration were noted in some patients. Stage progression was associated with a poor visual prognosis.

Long-term Clinical Outcomes of Cataract Surgery in Patients with Retinitis Pigmentosa

Purpose@#The long-term clinical outcomes of cataract surgery in patients with retinitis pigmentosa (RP) were evaluated. @*Methods@#A retrospective analysis of medical records was performed for patients who were diagnosed with RP and underwent cataract surgery. Preoperative best-corrected visual acuity (BCVA) was compared with BCVA at 1 month postoperatively and at the final visit. The proportion of patients with cystoid macular edema development or aggravation after surgery was evaluated, as was the proportion of patients with intraocular lens subluxation/dislocation. For patients who underwent optical coherence tomography, factors associated with a BCVA of 0.5 or better at 1 month were analyzed. @*Results@#In total, 133 eyes were included and the mean follow-up period was 58.7 months. The mean logarithm of minimal angle of resolution BCVA was 0.69 ± 0.65 at diagnosis. The BCVA was significantly improved to 0.51 ± 0.47 at 1 month postoperatively (p < 0.001). However, the BCVA at the final visit (0.70 ± 0.81) was similar to the baseline value (p = 1.000). Cystoid macular edema development or aggravation was noted in 8 eyes (6.0%) and intraocular lens subluxation/dislocation was noted in 4 eyes (3.0%). A subgroup analysis involving 108 eyes revealed that preoperative BCVA (p < 0.001) and ellipsoid zone status (p = 0.001) were associated with postoperative visual acuity. @*Conclusions@#Short-term outcomes of cataract surgery in patients with RP were comparatively good. However, long-term visual deterioration was noted with disease progression. Preoperative BCVA and ellipsoid zone status are useful markers for predicting short-term visual outcomes.

Proportion and Reasons for Ineligibility to Re-register for Extended Health Insurance in Neovascular Age-related Macular Degeneration

Purpose@#To evaluate the proportion and reasons for ineligibility to re-register for extended health insurance at 5 years in patients diagnosed with neovascular age-related macular degeneration (AMD) and registered for extended health insurance. @*Methods@#This retrospective study was performed in patients diagnosed with neovascular AMD and registered for extended health insurance with follow-up for at least 5 years. The criteria for re-registration for extended health insurance were determined along with the ineligibility for re-registration. @*Results@#In total, 263 patients were included in the analysis. Of these, 148 (56.3%) did not satisfy the criteria for re-registration. No active treatment was performed in 98 patients during the last 6 months of the study period (no recurrence, 51 patients; additional treatment was not considered beneficial due to retinal damage even without disciform scar formation, 44 patients). Macular disciform scar formation was noted in 50 patients (33.8%). Older age (p = 0.013), poor visual acuity (p = 0.004), and retinal angiomatous proliferation (p < 0.001) were associated with ineligibility for re-registration due to severe retinal damage. @*Conclusions@#Among the patients who were initially registered for extended health insurance, 56.3% failed to satisfy the re-registration criteria. The primary reason was advanced retinal damage. The results of this study provide useful information for patient education and to establish long-term treatment strategies.

Proportion of and Reason for Bevacizumab Usage in the Treatment of Wet Age-related Macular Degeneration

Purpose@#To evaluate the proportion of bevacizumab and the reason for its usage in wet age-related macular degeneration (AMD). @*Methods@#Retrospective analysis of medical records was performed for 1,541 patients who received ranibizumab, aflibercept, or bevacizumab injection to treat wet AMD. The proportion of bevacizumab among the entire set of injections was identified. The reason for selecting bevacizumab was additionally identified. @*Results@#During the study period, a total of 2,929 anti-vascular endothelial growth factor (anti-VEGF) injections were performed; 2,236 (76.3%) were ranibizumab or aflibercept injections and 693 (23.7%) were bevacizumab injections. The most common reason for bevacizumab usage was ‘having a 0.1 or worse best-corrected visual acuity or being unable to assure reimbursement due to the development of extensive scarring or geographic atrophy’ (297 bevacizumab injections, 42.9%). The second most common reason was ‘the inability to assure reimbursement such as extrafoveal choroidal neovascularization (CNV) or early CNV without definite fluid in the foveal region’ (201 bevacizumab injections, 29.0%). @*Conclusions@#Bevacizumab was used in 23.7% of the anti-VEGF injections to treat wet AMD. When analyzing patients’ treatment burden and financial impact, the results of the present study may provide useful information. Further multi-center studies are required to evaluate more precisely the usage of anti-VEGF drugs.

Long-term Clinical Course of Korean Patients Diagnosed with Macular Telangiectasia Type 2

Purpose@#To evaluate long-term visual prognosis and changes in the retinal microstructure in patients with macular telangiectasia type 2. @*Methods@#Retrospective analysis of medical records were performed for 61 eyes (32 patients) diagnosed with macular telangiectasia type 2. The visual acuity at diagnosis was compared with that at the final visit. In addition, cases were classified into three grades based on optical coherence tomography (OCT) findings: grade 1, inner retinal cavities only; grade 2, outer retinal cavities with ellipsoid zone disruption; and grade 3, ellipsoid zone disruption with proliferation of retinal pigment epithelium. Change in the grade during the follow-up was verified. In addition, visual acuities were compared between eyes with and without a change in grade. @*Results@#The mean follow-up period was 41.5 ± 33.2 months. Visual acuity had deteriorated significantly from the mean logarithm of the minimal angle of resolution 0.23 ± 0.26 at diagnosis to a mean of 0.31 ± 0.31 at the final visit (p < 0.001). When classified using OCT, 65.6%, 22.9%, and 11.5% were grades 1, 2, and 3, respectively, at diagnosis and 47.5%, 36.1%, and 16.4% at the final visit. Progression of grade was noted in 22.9%. A higher degree of visual deterioration was noted in eyes showing progression than in eyes without (p = 0.002). @*Conclusions@#During the long-term follow-up period, visual deterioration was noted in patients with macular telangiectasia type 2. However, the degree of deterioration was not high. Progression in the disruption of the retinal microstructure was the likely cause of visual deterioration.