Objective: This study aims to explore the association between oral lichen planus (OLP) and Hashimoto’s thyroiditis (HT) and its anti-thyroid antibodies to provide clinical evidence for thyroid disease screening in patients with OLP. Methods: This study was approved by the institutional ethics committee. A total of 125 clinically and histopathologically confirmed patients with OLP were enrolled as the case group, and they were matched with 125 non-OLP controls based on sex and age. Demographic data (gender, age, lesion type, and disease duration) were collected from both groups. Serum levels of thyroid peroxidase antibodies (TPOAb) and thyroglobulin antibodies (TgAb) were measured to analyze their associations with sex, age, lesion type, and disease duration in patients with OLP. Result: The prevalence of HT in patients with OLP was 31.20%, significantly higher than that in the control group (9.60%) (χ2=18.504, P<0.001). The prevalence of HT in female patients with OLP (39.13%) was significantly higher than that in male patients (9.09%)（χ2=10.93，P<0.001）. The positivity rate of thyroid peroxidase antibodies (TPOAb) in patients with OLP (17.6%) was significantly higher than in the control group (4.0%) (χ2=10.989, P<0.001). The TPOAb positivity rate was significantly higher in female patients (22.83%) than in male patients (3.03%) (χ2=5.210, P=0.014). There was no statistically significant difference in the positivity rate of TgAb between patients with OLP (7.2%) and the control group (3.2%) (P>0.05). Patients with erosive lesions had a significantly higher TPOAb positivity rate (25.0%, 17/68) compared to those with non-erosive lesions (8.77%, 5/57), and the difference was statistically significant (χ2=4.831, P=0.028). Logistic regression analysis revealed that female patients with OLP had an 8.935-fold higher risk of being TPOAb positive compared to males (OR=8.935, 95%CI: 1.134-70.388, P=0.038). Patients with erosive OLP lesions had a 3.199-fold higher risk of TPOAb positivity compared to those with non-erosive lesions (OR=3.199, 95%CI: 1.064-9.618, P=0.038). Conclusion The prevalence of HT is higher in patients with OLP, with higher positivity rates of anti-thyroid antibodies observed in female patients and those with erosive OLP lesions. This suggests that thyroid disease screening should be incorporated into the clinical management of patients with OLP, especially for women and patients who present with erosive lesions.

Objective:To analyze the clinical characteristics, imaging, myopathology and outcomes of patients with COVID-19 related autoimmune myopathy.Methods:The clinical features, serum creatine kinase (CK), myositis antibodies, muscle magnetic resonance imaging, myopathology and therapy of 5 patients with COVID-19 related autoimmune myopathy diagnosed in Peking University First Hospital from December 2022 to April 2023 were collected. The effects of the therapy after a short term follow up were analyzed.Results:Among the 5 patients, there were 3 males and 2 females, with onset age of 42-86 years. All patients presented with proximal muscle weakness in the recovery term of COVID-19. Myalgia was noted in 3 cases, dysphagia in 1, skin damage in 2, interstitial lung disease in 1. The serum CK of the 5 patients was 1 663-16 000 IU/L, 1 patient had anti-3-hydroxy-3-methylglutaryl-CoA reductase autoantibodies and 1 patient had anti-signal recognition particle autoantibodies. The electromyography showed myogenic lesions in all patients. Muscle magnetic resonance imaging showed diffuse muscle edema in all patients, myofascial edema in 3 and subcutaneous-tissue edema in 3. The muscle biopsies in 4 patients revealed necrotic myopathy,with high P62 expression in muscle fibers. The electromicroscopy of 2 patients revealed vacuolated mitochondria and intranuclear tubulofilamentous inclusions in muscle fibers. Four patients were treated with glucocorticoids, of whom 2 patients combined with intravenous immunoglobulin, tacrolimus or cyclophosphamide. One case had close monitoring without drug therapy. They showed significant improvement, but the CK was still abnormal in 4 patients.Conclusions:COVID-19 leads to immune mediated myopathy. The manifestation of patients is characterized by proximal predominant weakness and high creatine kinase level. Muscle magnetic resonance imaging shows diffuse muscle edema. The muscle biopsies reveal necrotic myopathy. The effectiveness of immunosuppression needs to be further studied.

Objective:To evaluate the risk factors for the complexity and surgical prognosis in patients with proliferative diabetic retinopathy (PDR).Methods:A historical cohort study of the CONCEPT trial, including 97 patients (97 eyes) who were diagnosed with PDR and requiring three-channel 23-gauge transconjunctival pars plana vitrectomy (PPV) from June 2017 to January 2018 at the First Affiliated Hospital of Nanjing Medical University.All patients received preoperative intravitreal injection of 0.5 mg conbercpet.Based on the PDR complexity score, patients were divided into >3 group or ≤3 group, and the systematic risk factors were compared between the two groups.The influence of sex, age, hypertension, renal insufficiency, duration of diabetes mellitus, and hemoglobin A1c level on the PDR complexity score was evaluated by multivariate logistic regression analysis.Based on age, patients were divided into <45 years group, 45-<60 years group, and ≥60 years group, and the differences in mean operative time, incidence of intraoperative hemorrhage, surgically induced lacrimation and silicone oil filling, and incidence of hemorrhage on color fundus photos and macular edema by optical coherence tomography at postoperative months 1 and 6 were analyzed among different age groups.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of The First Affiliated Hospital of Nanjing Medical University (No.2017-SR-283).Written informed consent was obtained from each subject.Results:The age of patients with PDR complexity score >3 was 46.5(36.0, 51.8) years, which was less than 54.0(45.5, 61.5) years for PDR complexity score ≤3, and the difference was statistically significant ( Z=1.835, P=0.002).Among the factors predicting PDR complexity score >3, logistic regression analysis indicated that only age was statistically significant ( P=0.005).For each 1-year increase in age, the risk of PDR complexity score >3 would increase by 7.4%( OR: 0.929, 95% CI: 0.883-0.977).Among the systemic factors, there were significant differences in age, history of diabetes, proportion of patients with hypertension and renal insufficiency among the three age groups (all at P<0.05).Among the ocular factors, there were significant differences in the proportion of patients with history of retinal laser treatment, fibrovascular membrane and complexity score >3 among the three groups (all at P<0.05).The proportion of patients with fibrovascular membrane and complexity score >3 in the <45 years group was significantly higher than that in the 45-<60 and ≥60 years groups (all at P<0.05).There were significant differences in the proportion of patients with intraoperative bleeding and silicone oil filling in the three age groups (all at P<0.017).The proportion of intraoperative bleeding and silicone oil filling in <45 years group was significantly higher than that in 45-<60 and ≥60 years groups (all at P<0.05).The macular edema on postoperative month 1 in the <45 years old group was significantly higher than that in the 45-<60 and ≥60 years groups (both at P<0.05). Conclusions:Among systemic factors, age has a significant impact on the increased PDR complexity and contributes to the poor prognosis of patients.There is a higher percentage of intraoperative complications and early postoperative macular edema in patients in the younger age group compared to the older age group.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is one of the subtypes of immune-mediated necrotizing myopathy. Anti-HMGCR antibodies induce complement activation,subsequently resulting in myofiber necrosis,regeneration with autophagy abnormalities and mitochondrial changes. The age of onset is from children to adulthood. Some patients have a history of exposure to statins. Most patients are subacute onset. The patients with chronic progressive process, are more like muscular dystrophy. The main symptoms are proximal symmetrical weakness of limbs and usually accompanied with extra-muscle symptoms. The MRI showed muscle edema in all patients and fatty infiltrates in some patients. Myositis-specific auto-antibodies and muscle biopsies play key roles in diagnosis of HMGCR myopathy. Corticosteroids and immunosuppressants were first line therapy. Pediatric patients or patients with chronic course are usually refractory, and the efficacy of different combinations of immunosuppressants needs to be further investigated.

Objective This study aimed to investigate the MRI features of forearm muscles from myotonic dystrophy type 1(DM1)and to evaluate the correlation between clinical factors and muscle MRI.Methods We retrospectively reviewed the medical records of genetic-confirmed DM1 patients including the clinical manifestations,the electromyography and the muscle pathology results.MRI scan was used to examine forearm muscles.Analysis of T1weighed images and short inversion time inversion recovery images were used to evaluate muscle fatty infiltration and muscle edema,respectively.The correlation between the fatty infiltration and age,disease duration and muscle strength were investigated.Results Twenty-three DM1 patients from different family were enrolled.The onset age was 17～60 year-old,mean age was(34.3±12.2)year-old,and the mean course of disease was 5(3,9)years.Their main symptoms were hand weakness and stiffness,accompanying with alopecia and excessive daytime sleepiness.The median fatty infiltration scores were recorded as follows:flexor digitorum profundus(FDP)4.0(3.0,4.0),flexor pollicis longus(FPL)4.0(3.0,4.0),extensor pollicis brevis 2.0(1.0,2.5),abductor pollicis longus 2.0(0.5,2.5),extensor pollicis longus 2.0(1.0,2.0),flexor digitorum superficialis 2.0(0.5,2.5),supinator(S)2.0(1.0,2.0).Other muscles scores were less than 1 of which the pronator teres(PT)was 1.0(0.0,1.0).Atrophy of FDP was detected in nine,atrophy of FPL in six and hypertrophy of PT in 11 patients.The muscle edema was evident in FDP,FPL,and the flexor digitorum superficialis,while brachioradialis and pronator teres were normal.There was no significant correlation between mean muscle fat infiltration score and age or course of disease.The mean muscle fat infiltration was significantly correlated with muscle strength score(r =-0.590,P<0.01).The mean fatty infiltration scores of FDP and FPL were not correlated with age nor course of disease,but correlated with grip strength(r =-0.754,P<0.01 and r =-0.699,P<0.01).Conclusion The forearm MRI in DM1 patients exhibit a relatively specific distribution of selective muscle involvement and can be used to assess muscle weakness.

Objective:To summarize the characteristics of neuralgia in Fabry disease and explore the effects of genders and alpha-galactosidase A (GLA) gene mutation types on neuralgia.Methods:Questionnaires and Brief Pain Inventory evaluations were conducted on the recruited patients diagnosed as Fabry disease in Department of Neurology, Peking University First Hospital from January 2001 to April 2020. The characteristics of the neuralgia were summarized, and the portrait of neuralgia between male and female patients, and the patient groups carrying truncated mutations and non-truncated mutations of GLA gene was compared.Results:A total of 93 patients with Fabry disease were enrolled. The incidence of neuralgia was 91.4% (85/93),and the average onset age of pain was 9 years. The average remission age was 20 years with the remission incidence of 22.8% (18/79). Pain attack on extremities [96.5%(82/85)] was the most common form. The neuralgia relieving rate of male patients [17.5%(11/63)] was lower than that of females (7/16, χ2=5.01, P=0.025).Brief Pain Inventory scores showed that the degree of most severe pain attack within 24 hours of male patients (4.16±3.20) was higher than that of females (2.07±2.02, t=3.03, P=0.004),and the impact of pain on daily life [male 4 (7) vs female 0 (4), Z=-2.33, P=0.020], walking ability [male 5 (8) vs female 0 (2), Z=-2.87, P=0.004], daily work [male 5 (8) vs female 0 (2), Z=-3.10, P=0.002], relationship [male 2 (6) vs female 0 (3), Z=-2.67, P=0.008] and interests [male 4 (8) vs female 0 (3), Z=-2.81, P=0.005] of male patients was also higher than female patients. The truncated mutation group [1 (2)] only showed higher score on the current pain level than the non-truncated mutation group [0(0), Z=-2.89, P=0.003]. Conclusions:The neuralgia in Chinese patients with Fabry disease showed high incidence and early onset. Male patients presented more severe pain than female which led to a greater impact on life, while the type of GLA gene mutation had less impact on neuralgia.

Objective:To summarize the clinical, pathological and muscle magnetic resonance imaging (MRI) features of human immunodeficiency virus (HIV)-associated nemaline myopathy (NM; HIV-NM).Methods:The present patient was a 23-year-old man with HIV infection who developed progressive proximal weakness and atrophy for more than 10 months. He was admitted to the Department of Neurology of Beijing Ditan Hospital in early June 2021. Electromyography showed myogenic findings. The serum creatine kinase was 202.4 U/L. CD 4+ count was 585×10 6/L. Serum monoclonal immunoglobulin (M protein) was negative. The patient underwent MRI examination of bilateral thigh muscles, biopsy of left biceps brachii and gene detection. The clinical, pathological and muscle MRI changes of HIV-NM were summarized based on the literature review. Results:MRI examination of bilateral thigh muscles showed edema changes. Muscle biopsy showed nemaline structures in some muscle fibers, accompanied by fiber atrophy and regeneration. No gene mutation related to clinical phenotype was found by second-generation sequencing. After intravenous immunoglobulin combined with prednisone, the patient′s weakness symptoms were significantly improved. A total of 17 cases of HIV-NM (including the present case) were identified in the literature, who were aged (33.7±9.1) years. Fifteen were males and two were females. All patients developed proximal limb weakness. Creatine kinase was normal or slightly elevated. Serum monoclonal protein was positive in 3 cases (3/7). Immunosuppressive therapy was effective.Conclusions:The main clinical characteristics of HIV-NM are progressive proximal limb weakness and muscle atrophy. The muscle pathology shows a large number of nemaline structures in atrophic muscle fibers. Muscle edema can be seen on muscle MRI. This is the first report of HIV-NM in China, which may be a special subtype of immune myopathy.

OBJECTIVES: To evaluate the effectiveness of an acellular dermal matrix or a connective tissue autograft in the treatment of multiple adjacent gingival recessions through Meta-analysis. METHODS: Randomized controlled trials were screened in four electronic databases in English according to the inclusion and exclusion criteria until April 20, 2022. The main outcome indicators were keratinized gingival tissue width, recession depth, probing depth, clinical attachment level, complete root coverage, and root coverage esthetic score. RESULTS: Seven randomized controlled trials were included. After 12 months, the connective tissue graft in the control group could increase the keratinized gingival tissue width [mean difference (MD)=-0.28 (-0.47, -0.08), P=0.006], reduce the gingival recession depth [MD=0.23 (0.12, 0.35), P<0.000 1], and improve the complete root coverage [risk ratio=0.80, 95% confidence interval (0.69, 0.93), P=0.003] compared with the acellular dermal matrix in the experimental group. No significant difference was found in probing depth, clinical attachment level, and root coverage esthetic score between groups. CONCLUSIONS Connective tissue grafts have advantages in increasing the keratinized gingival tissue width, reducing the gingival recession depth, and improving the complete root coverage in surgeries for treating multiple adjacent gingival recessions. Acellular dermal matrices also have some clinical value in terms of operation simplicity and similar effectiveness.
Humans ; Gingival Recession/surgery* ; Acellular Dermis ; Surgical Flaps ; Esthetics, Dental ; Connective Tissue/transplantation*

Objective:To determine the clinical, pathological and imaging phenotypes of pediatric patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy to explore its diagnostic strategies.Methods:The clinical features of 10 pediatric patients with anti-HMGCR myopathy in the Department of Neurology, Peking University First Hospital from July 2014 to July 2021 were collected. Muscle biopsies were performed in all patients, with histological, enzymatic histochemical and immunohistochemical staining.Results:The male to female ratio was 6∶4, the age of onset was 3-16 (8.3±3.7) years, 2 cases had subacute onset, and 8 cases experienced chronic progressive onset. All patients presented with neck and proximal muscular weakness of all limbs. Skin rash was observed in 2 cases. Serum creatine kinase was 998-27 981 U/L. The electromyography results were available from 6 cases, who experienced myogenic changes. The muscle magnetic resonance imaging was performed in 5 cases and revealed muscle edema predominantly in posterior compartment of thigh, with mild fatty infiltrate in 2 cases. An initial diagnosis was limb-girdle muscular dystrophy in 7 cases, but with subsequently negative genetic testing. Muscle biopsies revealed scattered necrotic fibers and regenerating fibers, complement deposition in sarcolemma basement-membrane areas of non-necrotic fibers and a few of lymphocyte infiltrate in all specimens. Moreover, a high frequency of major histocompatibility complex Ⅰ expression in muscle fibers was observed in 9 cases, proliferation of connective tissue of endomysium in 8 cases, muscle fiber hypertrophy in 4 cases and vacuoles in 2 cases.Conclusions:Pediatric anti-HMGCR myopathy is frequently misdiagnosed as muscular dystrophy. Systematic consideration of anti-HMGCR myopathy and testing for myositis specific antibody in children with genetically unconfirmed muscular dystrophy may help the differential diagnosis.

The vacuole in muscle fibers is a non-specific myopathological change. As a myopathological term, it occurs in several rare disorders. The vacuoles are mostly related to the autophagy of glycogen, lipids, abnormal proteins, and organelles, and a few are caused by glycogen or lipids deposition or sarcoplasmic reticulum luminal vacuolization. The vacuoles impair the structure of muscle fibers, being one of the myopathological features in various diseases. In this review, clinical features and myopathological changes of various diseases with vacuolar muscle fibers were introduced and the diagnostic value of intrafibral vacuoles was focused on. Knowing the underlying pathogenesis is required to understand these myopathological changes.