Performance of the 2019 ACR/EULAR Classification Criteria for IgG4-Related Disease in a Large Chinese Cohort.

OBJECTIVE: The purpose of this research was to ascertain the effectiveness of the newly established criteria for classifying IgG4-related disease (IgG4-RD), as applied to a large Chinese cohort in real-world clinical settings. METHODS: Patient data were procured from the digital health records of 4 prominent academic hospitals. The criterion standard for identifying IgG4-RD patients was from a seasoned rheumatologist. The control group consisted of individuals with other ailments such as cancer, other forms of pancreatitis, infectious diseases, and illnesses that mimic IgG4-RD. RESULTS: A total of 605 IgG4-RD patients and 760 mimickers were available for analysis. The 2019 EULAR/ACR criteria have a sensitivity of 69.1% and a specificity of 90.9% in this large Chinese cohort. IgG4-RD had a greater proportion of males (55.89% vs 36.25%, p < 0.001), an older average age at diagnosis (54.91 ± 13.44 vs 48.91 ± 15.71, p < 0.001), more pancreatic (29.59% vs 6.12%, p < 0.001) and salivary gland (63.30% vs 27.50%, p < 0.001) involvement, and a larger number of organ involvement (3.431 ± 2.054 vs 2.062 ± 1.748, p < 0.001) compared with mimickers. CONCLUSIONS: The 2019 EULAR/ACR criteria are effective in classifying IgG4-RD in Chinese patients, demonstrating high specificity and moderate sensitivity.

Intraoperative slow-release dexamethasone intravitreal implant (Ozurdex) in epiretinal membrane peeling surgery: a prospective randomized controlled trial.

Purpose: This study aimed to determine the efficacy of the dexamethasone (DEX) intravitreal implant for the regression of macular edema and the improvement of best-corrected visual acuity (BCVA) after the removal of idiopathic epiretinal membrane (ERM). Methods: This prospective randomized controlled trial recruited 81 patients with idiopathic ERM. These patients all underwent 25-gauge pars plana vitrectomy combined with ERM and internal limiting membrane peeling surgery. Among them, 41 eyes in the DEX group received additional DEX implants and 40 in the non-DEX group did not. Outcomes including central retinal thickness (CRT), BCVA, and intraocular pressure were measured 1 and 3 months after surgery. Results: The DEX group had thinner CRTs compared to the non-DEX group at 1 month postoperatively (p <0.05), but did not differ significantly at the 1-week and 3-month follow-up visits (p = 0.109 and p = 0.417, respectively). There were no statistical differences with respect to BCVA (p = 0.499, 0.309, 0.246, and 0.517, respectively) and intraocular pressure (p = 0.556, 0.639, 0.741, and 0.517, respectively) between the two groups at each point of follow-up visits. Conclusion: DEX accelerated the reduction of CRT at 1 month after surgery. However, no evidence of further anatomical (CRT) or functional (BCVA) benefits using DEX was observed at 3 months. Clinical Trial Registration: https://clinicaltrials.gov/, identifier NCT05416827.

Clinical profiles differ in IgG4-related disease with and without allergy: a large case-control study in China.

OBJECTIVES: This study aimed to elucidate different clinical profiles in IgG4-related disease (IgG4-RD) with and without allergy. METHODS: Four hundred and thirty-four patients diagnosed with IgG4-RD at Peking University People's Hospital were included. Clinical and treatment options-based relapse data were collected and compared between IgG4-RD patients with and without allergy. RESULTS: Among these patients, 214 (49.3%) had allergic diseases. Most of the IgG4-RD patients with allergy had initial involved organs directly exposed to ambient air and their allergic symptoms occurred mostly before or at IgG4-RD disease onset. Compared with IgG4-RD patients without allergy, allergic patients had almost equal sex ratio, more organ involvement, earlier ages of disease onset and diagnosis, longer disease duration, higher incidence of dacryoadenitis, sialadenitis, lymphadenopathy, paranasal sinus and lung lesions. Higher serum IgG4, IgE and IgG4/IgG ratio, lower serum C3 complement 3 (C3) and C4, and higher incidence of eosinophilia were also found in IgG4-RD patients with allergy. Furthermore, allergy may increase relapse rate and shorten relapse-free survival time in IgG4-RD patients treated with glucocorticoids only, whereas combination therapy of glucocorticoids and immunosuppressants could improve treatment outcome. CONCLUSIONS: Allergy leads to disparities in clinical profiles in IgG4-RD patients. Allergy could result in higher relapse rate and shorten relapse-free survival time in patients receiving glucocorticoids only.

Quantitative analysis of choroidal vasculature in central serous chorioretinopathy using ultra-widefield swept-source optical coherence tomography angiography.

We aimed to quantitatively compare the choroid blood flow and choroid thickness at the periphery among eyes with central serous chorioretinopathy (CSC), fellow eyes and healthy eyes using ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA). Retrospective analysis of 49 patients with CSC (98 eyes, including unaffected fellow eyes) and 49 age and sex matched controls were included. We obtained 3-dimensional data of vertical 20 mm × horizontal 24 mm × scan depth 6 mm, comprising 9 subfields (superotemporal, upper, superonasal, temporal, central, nasal, inferotemporal, lower, inferonasal regions). CSC eyes presented with greater density of large-vessel choroidal layer in all the 9 subfields compared with controls. Compared with normal eyes, CSC eyes had greater choroidal thickness (superotemporal, upper, superonasal, temporal, central, nasal, inferotemporal, and inferonasal subfields) and choroidal volume (superotemporal, upper, superonasal, temporal, central, and nasal subfields). Compared with control eyes, the choriocapillaris density in the superotemporal, inferotemporal and inferonasal subfields was greater in patients with CSC. Our study may provide further evidence for the congestion of vortex vein in the pathogenesis of CSC. UWF SS-OCTA can be used to evaluate the abnormalities of the choroidal structures even at the periphery in eyes with CSC.

Comparison of 24×20 mm2 swept-source OCTA and fluorescein angiography for the evaluation of lesions in diabetic retinopathy.

AIM: To compare ultra-widefield (24×20 mm2) swept-source optical coherence tomography angiography (SS-OCTA) and fluorescein angiography (FA) in the evaluation of diabetic retinopathy (DR) lesions. METHODS: Forty-six eyes of 23 patients with treatment-naïve DR were included at Peking University People's Hospital from September 1, 2021, until December 31, 2021, as well as 23 age and gender matched healthy controls. Quantitative assessments of DR lesions on FA and SS-OCTA (superficial capillary plexus, SCP, 24×20 mm2) were performed. RESULTS: Area of fovea avascular zone (FAZ) was larger in DR cases than controls (0.34±0.069 mm2 vs 0.287±0.108 mm2, P=0.006). In DR eyes, the mean FAZ area was 0.34±0.069 and 0.334±0.087 mm2 on SS-OCTA and FA, respectively (P=0.428), while the median FAZ perimeter was 2.382 (IQR, 2.201-2.59) and 2.333 (IQR, 2.138-2.6) mm on SS-OCTA and FA images (P=0.733). There was no significant difference in the size of the non-perfusion area (NPA) between the images on SS-OCTA and FA (12.389, IQR 4.96-28.3 and 11.125, IQR 5-28.31 mm2, P=0.197). The median total microaneurysm (MA) count was 35 (IQR, 19-46) and 73 (IQR, 43-93) on SS-OCTA and FA (P<0.001), respectively. No significant difference in intra-retinal microvascular abnormality (IRMA) and neovascularization (NV) count was found between the two techniques. The intraclass coefficient (ICCs) of all the parameters above indicated stable repeatability. CONCLUSION: Ultra-widefield SS-OCTA represents a reliable, noninvasive, and quantitative imaging technique in the assessment of microvasculature in DR, which offers a potential substitute for FA in DR evaluation.

Three-dimensional choroidal vascularity index in central serous chorioretinopathy using ultra-widefield swept-source optical coherence tomography angiography.

Background: To map and compare the three-dimensional choroidal vascularity index (3D-CVI) in eyes with unilateral central serous chorioretinopathy (CSC), fellow eyes and control eyes using ultra-widefield swept source optical coherence tomography (UWF SS-OCTA). Methods: In this prospective observational study, the 3D-CVIs were measured in 9 subfields or 1 × 1 mm grids by the UWF SS-OCTA with a viewing angle of horizontal 24 × vertical 20 mm. The proportions of vortex vein anastomoses and their corresponding CVI in the central regions were compared among the CSC, fellow and control eyes. Correlations of CVI and vascular density of the large choroidal vessel layer/choriocapillaris layer/choroidal thickness (CT) were also assessed. Results: Thirty-two eyes in 32 patients with unilateral CSC and 32 normal eyes were included in the study. The mean CVI in the eyes with CSC was significantly greater than that in the fellow eyes of CSC and control eyes (41.99 ± 3.56% vs. 40.38 ± 3.855%, P = 0.003; 41.99 ± 3.56% vs. 38.93 ± 4.067%, P = 0.004, respectively). The CVIs in superotemporal, inferotemporal and inferonasal regions were significantly higher in CSC eyes than control eyes (P = 0.03, P = 0.02, P = 0.008). In CSC eyes, there was a linear positive correlation between 3D-CVI and vascular density of the large choroidal vessel layer and CT in all subfields. The proportion of vortex vein anastomoses in CSC was 25/32 (78.1%), and significantly higher in fellow and control eyes (P < 0.001). The average central CVI was significantly higher in CSC eyes with anastomoses than in CSC eyes without anastomoses (42.8 ± 5.1% vs. 38.4 ± 2.7%, P = 0.039). CVIs in superior, central, inferior, superonasal, nasal and inferonasal regions were significantly correlated with vortex vein anastomoses (P < 0.05), regardless of CSC, fellow or healthy eyes. In addition, whether there were vortex vein anastomoses, CVI in superotemporal region was significantly higher in eyes with CSC (P = 0.002) and fellow eyes (P = 0.014), compared to control eyes. No significant correlation was found between hypertension and CVIs in the three groups. Conclusion: Remodeling of choroidal drainage routes by venous anastomosis between superior and inferior vortex veins may be common in CSC. The 3D-CVI could be a comprehensive parameter to evaluate the choroid vasculature and help understand the pathogenesis of pachychoroid spectrum disease.

Comparison of swept-source OCTA and indocyanine green angiography in central serous chorioretinopathy.

BACKGROUND: To compare swept-source optical coherence tomography angiography (SS-OCTA) and indocyanine green angiography (ICGA) in patients with central serous chorioretinopathy (CSC). METHODS: SS-OCTA and ICGA images of 39 eyes with symptomatic CSC were collected and aligned. Spatial overlap of the annotations of the coarse granulated high reflective area on choriocapillary OCTA and the hyperfluorescence area on mid-phase ICGA was calculated according to the Jaccard index (JI). SS-OCTA findings of fellow eyes and changes in SS-OCTA abnormalities during the follow-up were also analyzed. RESULTS: Three main types of abnormalities in choriocapillaris SS-OCTA images were found: type A, coarse granulated high reflective area (39 eyes [100%]); type B, roundish dark halo around Type A (32 eyes [82.1%]); and type C, coarse granulated low reflective area (39 eyes [100%]). The mean JI of type A on SS-OCTA and the hyperfluorescence area on ICGA were 0.55 ± 0.15 for grader 1 and 0.49 ± 0.15 for grader 2. The mean area of type A abnormalities on SS-OCTA and hyperfluorescence on ICGA was 3.976 (IQR, 2.139-8.168) and 3.043 (IQR, 1.408-4.909) mm2 (P = 0.199). The areas of type A, B and C abnormalities on SS-OCTA after laser treatment or observation were 3.36mm2 (IQR, 2.399-9.312), 2.9mm2 (IQR, 2.15-3.7), and 0.19mm2 (IQR, 0.08-0.23), respectively, which was smaller than those in the baseline (7.311mm2 (IQR 3.788-11.209), P < 0.001; 4.3mm2 (IQR, 2.8-9.8), P = 0.002;0.33mm2 (IQR, 0.23-0.38), P < 0.001). The change in the type A, B or C area was not significantly different between the two groups (P = 0.679, 0.732, and 0.892). CONCLUSION: The coarse granulated high reflective area in SS-OCTA corresponded well with the hyperpermeability area in ICGA. SS-OCTA promotes noninvasive visualization and follow-up quantifications of the choroidal vasculature in CSC patients.

All-trans retinoic acid plus low-dose rituximab vs low-dose rituximab in corticosteroid-resistant or relapsed ITP.

The study aimed to compare the efficacy and safety of all-trans retinoic acid (ATRA) plus low-dose rituximab (LD-RTX) with LD-RTX monotherapy in corticosteroid-resistant or relapsed immune thrombocytopenia (ITP) patients. Recruited patients were randomized at a ratio of 2:1 into 2 groups: 112 patients received LD-RTX plus ATRA, and 56 patients received LD-RTX monotherapy. Overall response (OR), defined as achieving a platelet count of ≥30 × 109/L confirmed on ≥2 separate occasions (≥7 days apart), at least a doubling of the baseline platelet count without any other ITP-specific treatment, and the absence of bleeding within 1 year after enrollment, was observed in more patients in the LD-RTX plus ATRA group (80%) than in the LD-RTX monotherapy group (59%) (between-group difference, 0.22; 95% CI, 0.07-0.36). Sustained response (SR), defined as maintenance of a platelet count >30 × 109/L, an absence of bleeding, and no requirement for any other ITP-specific treatment for 6 consecutive months after achievement of OR during 1 year following enrollment, was achieved by 68 (61%) patients in the combination group and 23 (41%) patients in the monotherapy group (between-group difference, 0.20; 95% CI, 0.04-0.35). The 2 most common adverse events (AEs) for the combination group were dry skin and headache or dizziness. Our findings demonstrated that ATRA plus LD-RTX significantly increased the overall and sustained response, indicating a promising treatment option for corticosteroid-resistant or relapsed adult ITP. This study is registered at www.clinicaltrials.gov as #NCT03304288.

Clinical characteristics of IgG4-related respiratory disease patients: a large Chinese cohort study.

OBJECTIVES: IgG4-related disease (IgG4-RD) is an autoimmune disorder and frequently involves multiple organs. The respiratory tract is one of the most frequently affected sites. In this study, we aimed to compare the demographic and clinical characteristics between IgG4 related respiratory disease (IgG4-RRD+) and extra-thoracic IgG4-related disease (IgG4-RRD-) in a large cohort. METHODS: A total of 448 cases of IgG4-RD (104 IgG4-RRD+ patients and 344 IgG4-RRD- patients) diagnosed at Peking University People's Hospital during 2003 to 2020 were included in our study. Patients' demographic data, clinical characteristics, laboratory parameters and imaging features were analysed. RESULTS: IgG4-RRD+ patients had an older age at disease onset and diagnosis. Multiorgan involvement and hypocomplementaemia were more common in IgG4-RRD+. Besides, the level of ESR, IgG and IgG4 were higher in IgG4-RRD+ patients. In IgG4-RRD+ group, salivary gland, lacrimal gland, lymph nodes, biliary system and kidney were more commonly involved than those in the IgG4-RRD- group. Also, more organ involvement eosinophilia and biliary involvement were independent risk factors for the development of IgG4-RRD+. CONCLUSIONS: Our study revealed demographic, clinical and laboratory differences between the two phenotypes, in addition to describing the imaging features of IgG4-RRD+, which will be helpful for understanding of the disease.

Comparison between Fovea-Sparing and Complete Internal Limiting Membrane Peeling for the Treatment of Myopic Traction Maculopathy: A Systemic Review and Meta-Analysis.

INTRODUCTION: Myopic traction maculopathy (MTM) is a major cause of impaired vision in eyes with high myopia, which is characterized by retinal thickening, retinoschisis, lamellar macular hole (MH), and foveal retinal detachment. Pars plana vitrectomy (PPV) with fovea-sparing internal limiting membrane peeling (ILMP) has been developed to theoretically prevent postoperative MH formation and improve best-corrected visual acuity (BCVA) gain for MTM compared with the complete ILMP. However, in previous studies, the anatomic and visual outcomes still remain uncertain and controversial. OBJECTIVES: The aim of this study was to evaluate the anatomic and visual outcomes of vitrectomy with fovea-sparing ILMP for the treatment of MTM compared with complete ILMP. METHODS: Articles from PubMed, EMBASE, Web of Science, and Cochrane Library were systematically retrieved. The main outcomes were the rate of a postoperative MH and visual improvement of BCVA (converted to logarithm of the minimum angle of resolution [logMAR]). The secondary outcomes were the proportion of patients with visual improvement, the proportion of anatomic success, preoperative and postoperative BCVA, preoperative and postoperative central fovea thickness, and time to anatomic resolution. RESULTS: There was a higher rate of postoperative MH formation (odds ratio [OR] 5.64; 95% confidence interval [CI]: 1.72-18.44; p = 0.004) and less improvement of BCVA in logMAR (mean difference [MD] -0.09; 95% CI: -0.18 to 0.00; p = 0.04) in the complete ILMP group. However, postoperative BCVA (MD 0.14; 95% CI: 0.00-0.27; p = 0.05), the proportion of patients with visual improvement (OR 0.39; 95% CI: 0.15-1.02; p = 0.05), postoperative central foveal thickness (MD -10.02; 95% CI: -24.4 to 4.36; p = 0.17), the rate of anatomic success (MD 0.39; 95% CI: 0.15-1.03; p = 0.06), and time to resolution (MD -1.65; 95% CI: -3.66 to 0.36; p = 0.11) showed no significant differences. CONCLUSION: PPV combined with the fovea-sparing ILMP could contribute to a lower MH formation rate and more improvement of BCVA in logMAR than PPV combined with complete ILMP.

Development and validation of a nomogram to predict cancer-specific survival of uveal melanoma.

BACKGROUND: Uveal melanoma (UM) is a rare but aggressive cancer, which is the most common primary intraocular malignancy in adults. We aimed to develop and validate a competing risk nomogram to predict cancer-specific survival (CSS) of patients with UM, as well as compare its prognostic value with that of the American Joint Committee on Cancer (AJCC) staging system. METHODS: Data of patients diagnosed with UM from 2010 to 2015 were identified from the Surveillance, Epidemiology, and End Results (SEER) database. We extracted and integrated significant prognostic factors based on competing risk regression to build a nomogram. The nomogram with an online prediction version was also created. The performance of the nomogram was evaluated using Harrell's concordance index (C-index) and calibration plots. Receiver operating characteristic (ROC) curve was carried out to estimate clinical applicability of the model. Improvements in the predictive accuracy of our new model compared with AJCC staging system were estimated by calculating the relative integrated discrimination improvement (IDI) and the net reclassification improvement (NRI). RESULTS: A total of 839 eligible patients with primary UM were randomly assigned to a training cohort (588, 70%) and a validation cohort (251, 30%). Age, histological type, T stage and M stage were independent prognostic factors to predict CSS of UM and were incorporated in the nomogram. The calibration plots indicated that the 3- and 5-year CSS probabilities were consistent between the nomogram prediction and the actual observation. The C-index for this model was 0.778 (95% CI:0.756-0.800) and 0.786 (95% CI: 0.749-0.816) in the training cohort and validation cohort. Areas under the curve (AUCs) were 0.814, 0.771, and 0.792 in the training cohort, 0.788, 0.781 and 0.804 in the validation cohort, respectively. The NRI value in AJCC staging system was - 0.153 (95% CI -0.29 - - 0.041) for 3 years of follow-up and - 0.276 (95% CI -0.415 - - 0.132) for 5 years of follow-up. The IDI values for 3 and 5 years of follow-up in the AJCC staging system were - 0.021 (P = 0.076) and - 0.045 (P = 0.004), respectively. CONCLUSIONS: We have developed and validated a competing risk nomogram to reliably predict cancer-specific survival of patients with UM. This convenient tool may be useful for evaluating cancer-specific prognosis.

Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case-control study based on 450 patients.

We aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD-) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People's Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD- were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China.

BACKGROUND: IgG4-related ophthalmic disease (IgG4-ROD) is one of the phenotypes of IgG4-related disease (IgG4-RD), and its lesions are mainly located in the ocular. Currently, there are few studies on IgG4-ROD and no study has compared the phenotypic differences between IgG4-ROD and non IgG4-ROD (nIgG4-ROD). Thus, it is difficult to establish the optimal treatment strategy for IgG4-ROD. The aim of this study was to identify the disparities between the two groups and to clarify the risk factors for IgG4-ROD relapse. METHODS: 434 IgG4-RD patients met comprehensive diagnostic criteria and diagnosed at Peking University People's Hospital between January 2009 and January 2020 were recruited in this study. Patients were divided into IgG4-ROD and nIgG4-ROD group according to the ophthalmic involvement. Demographic, clinical, and laboratory data of two groups were collected and compared. Cox regression analysis was used to identify the independent risk factors for IgG4-ROD relapse. RESULTS: 255 IgG4-ROD patients were identified in this study. IgG4-ROD group had almost equal sex ratio, younger age of disease onset and diagnosis comparing with nIgG4-ROD patients. As compared to nIgG4-ROD group, higher percentage of IgG4-ROD patients met the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) for IgG4-RD; moreover, IgG4-ROD patients had higher AECC scores and IgG4-RD responder index (RI). Allergic diseases and multiorgan involvement were more common in IgG4-ROD group. IgG4-ROD was frequently associated with salivary gland, paranasal sinus, lung, and lymph node involvement, while retroperitoneal fibrosis and biliary system lesions were more common in nIgG4-ROD. IgG4-ROD patients had higher serum IgG4 levels, IgG4/IgG ratio, IgE levels, and lower CRP levels. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse. IgG4-ROD patients treated with initial glucocorticoid plus immunosuppressant had longer relapse-free survival time than patients treated with initial glucocorticoid monotherapy. CONCLUSIONS: IgG4-ROD patients had distinctive clinical features compared with nIgG4-ROD patients. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse, which could prolong the relapse-free survival time of IgG4-ROD patients. These findings may have important implications for understanding and management of IgG4-ROD.

Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People's Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.

Clinical features of IgG4-related retroperitoneal fibrosis among 407 patients with IgG4-related disease: a retrospective study.

OBJECTIVES: IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF-) in a large cohort. METHODS: We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People's Hospital between March 2009 and May 2019. RESULTS: Among 407 patients, 58 had retroperitoneum affected. As compared with IgG4-RD RPF- patients, IgG4-RD RPF+ patients showed older age at disease onset and diagnosis. IgG4-RD RPF+ group involved more male patients. In terms of organ involvement, IgG4-RD RPF+ group was more frequently presented with kidney involvement, while salivary gland, lacrimal gland and pancreas were more prominent in the IgG4-RD RPF- group. In addition, the CRP, ESR level and creatinine level were significantly higher in IgG4-RD RPF+ patients, and hypocomplementemia were more common in this group. CONCLUSION: We have revealed demographic, clinical and laboratory differences between IgG4-RD RPF+ and RPF- patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

A risk score for predicting hospitalization for community-acquired pneumonia in ITP using nationally representative data.

Infection is one of the primary causes of death from immune thrombocytopenia (ITP), and the lungs are the most common site of infection. We identified the factors associated with hospitalization for community-acquired pneumonia (CAP) in nonsplenectomized adults with ITP and established the [corrected] (ACPA) prediction model to predict the incidence of hospitalization for CAP. This was a retrospective study of nonsplenectomized adult patients with ITP from 10 large medical centers in China. The derivation cohort included 145 ITP inpatients with CAP and 1360 inpatients without CAP from 5 medical centers, and the validation cohort included the remaining 63 ITP inpatients with CAP and 526 inpatients without CAP from the other 5 centers. The 4-item ACPA model, which included age, Charlson Comorbidity Index score, initial platelet count, and initial absolute lymphocyte count, was established by multivariable analysis of the derivation cohort. Internal and external validation were conducted to assess the performance of the model. The ACPA model had an area under the curve of 0.853 (95% confidence interval [CI], 0.818-0.889) in the derivation cohort and 0.862 (95% CI, 0.807-0.916) in the validation cohort, which indicated the good discrimination power of the model. Calibration plots showed high agreement between the estimated and observed probabilities. Decision curve analysis indicated that ITP patients could benefit from the clinical application of the ACPA model. To summarize, the ACPA model was developed and validated to predict the occurrence of hospitalization for CAP, which might help identify ITP patients with a high risk of hospitalization for CAP.

Outcomes of symptomatic venous thromboembolism after haploidentical donor hematopoietic stem cell transplantation and comparison with human leukocyte antigen-identical sibling transplantation.

BACKGROUND: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is regarded as a curative therapy for majority of hematologic malignancies and some non-malignant hematologic diseases. Venous thromboembolism (VTE) has become increasingly recognized as a severe complication following allogeneic hematopoietic stem cell transplantation (allo-HSCT). OBJECTIVES: To show the characteristics of VTE after haploidentical donor hematopoietic stem cell transplantation (HID-HSCT) and make comparisons with matched related donor HSCT (MRD-HSCT). PATIENTS/METHODS: A retrospective nested case-control study design was used, cases with VTE and matched controls were selected, with 3534 patients underwent HID-HSCT and 1289 underwent MRD-HSCT. RESULTS: During follow-up, 114 patients with VTE were identified. The incidence of VTE in HID-HSCT group was similar to that of MRD-HSCT group (2.4% versus 2.3%, P = 0.92). In HID-HSCT group, VTE occurred at a median time of 92.5 days, which was earlier than MRD-HSCT group (243.5 days). For HID-HSCT, advanced disease status, cardiovascular risk factors, acute graft-versus-host disease (aGVHD), and relapse were the independent risk factors for VTE. For MRD-HSCT, cardiovascular risk factors, aGVHD, and relapse were associated with VTE. Overall survival (OS) of patients following HID-HSCT and MRD-HSCT were similar, but the OS in patients with VTE was significantly lower than patients without VTE. CONCLUSIONS: There was no statistical difference in the incidence of VTE after HID-HSCT compared with MRD-HSCT. The development of VTE adversely impacted the OS after allo-HSCT.