Comprehensive Diagnostic Criteria Research Articles

IgG4-related uveitis. A French cohort and literature review

IntroductionIgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear. Here, we report on a nationwide French multicenter cohort of patients with IgG4-RD and uveitis and conducted a literature review. MethodsPatients with uveitis and a concomitant definite diagnosis of IgG4-RD (Revised Comprehensive Diagnostic criteria, American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, International Consensus Diagnostic Criteria for auto-immune pancreatitis, or diagnostic criteria for IgG4-related hypophysitis), were screened from our national IgG4-RD and systemic fibrosis database. Concomitantly, we conducted a PubMed literature review and selected cases of definite IgG4-RD with uveitis. ResultsWe reported on 16 patients (8 from our database and 8 from the literature) and a total of 30 episodes of uveitis. Uveitis cases represented 3 % of total IgG4-RD patients in the national database on IgG4-RD and systemic fibrosis. Uveitis was inaugural in IgG4-RD in 4/16 cases (25 %) (appearing before any other IgG4-related symptom, at a median of 9 months), occurred concurrently to other IgG4-related symptoms in 9/16 cases (56 %) (at a median of 15 months before IgG4-RD diagnosis), and appeared during follow up in 3/16 patients (19 %) (at a median of 57 months after first IgG4-related symptoms). When uveitis occurred during follow up, it was associated with IgG4-RD manifestations in other organs in 6/9 patients (67 %). Uveitis was bilateral in 8/16 cases (50 %) and granulomatous in 5/10 cases (50 %). It was anterior in 8/13 (62 %), intermediate in 3/13 (23 %), and global (panuveitis) in 4/13 patients (31 %). Median serum IgG4 at diagnosis was 3.2 g/L. Median follow up time was of 6 years, during which 8/16 patients (50 %) experienced at least one relapse of uveitis. Treatment data was available for 29/30 uveitis flares. Steroids were used in 28/29 episodes of uveitis (97 %), leading to remission of uveitis in 16/28 cases (57 %). Methotrexate and rituximab (in combination with systemic steroids) were administered as second- or third-line therapy in 6/29 (21 %) and 5/29 (17 %) episodes of uveitis, respectively, and led to remission of uveitis in 4/6 cases (67 %) and 4/5 cases (80 %), respectively. One third of uveitides required at least two different lines of treatment for remission induction (mainly a combination of both systemic steroids and methotrexate or rituximab). Discussion and conclusionsUveitis may be one of the initial symptoms of IgG4-RD, and IgG4-RD should be considered in the diagnostic workup of uveitis. Its early onset in IgG4-RD may help with early diagnosis and treatment of the disease. Steroid monotherapy may be sufficient to treat IgG4-related uveitis, yet relapses were frequent (50 %) and ultimately a third of patients required at least two lines of treatment. Hence, steroid-sparing agents can be considered at early stages of the disease, particularly for patients with a high risk of relapse or steroid-related complications.

The 2022 revised diagnostic criteria for IgG4-related respiratory diseases

In 2011, the Comprehensive Diagnostic Criteria for IgG4-related disease was published in Japan. Organ-specific diagnostic criteria based on organ-specific findings were proposed and published by each of the related societies, and the diagnostic criteria for IgG4-related respiratory disease was published in 2015. Based on the revisions to the comprehensive diagnostic criteria in 2020 and the publication of the Classification Criteria, new diagnostic criteria for IgG4-related respiratory disease are presented. Emphasis has been placed on evaluating specific pathological findings and excluding other respiratory diseases. It is mentioned in the commentary that in cases with imaging findings suggestive of interstitial pneumonia with chronic fibrosis or poor response to steroid therapy, other possible diseases should be considered.

Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort

BackgroundWe investigated sensitivity of the 2020 Revised Comprehensive Diagnostic Criteria (RCD) and the 2019 ACR/EULAR classification criteria across the four identified IgG4-related disease (IgG4-RD) phenotypes: “Pancreato-Hepato-Biliary”, “Retroperitoneum and Aorta”, “Head and Neck-limited” and “Mikulicz’ and Systemic” in a well-characterized patient cohort.MethodsWe included adult patients diagnosed with IgG4-RD after comprehensive clinical assessment at Oslo University Hospital in Norway. We assigned patients to IgG4-RD phenotypes based on pattern of organ involvement and assessed fulfillment of RCD and 2019 ACR/EULAR classification criteria. Differences between phenotype groups were analyzed using one-way ANOVA for continuous variables, and contingency tables for categorical variables.ResultsThe study cohort included 79 IgG4-RD patients assigned to the “Pancreato-Hepato-Biliary” (22.8%), Retroperitoneum and Aorta” (22.8%) “Head and Neck-limited” (29.1%), and “Mikulicz’ and Systemic” (25.3%) phenotype groups, respectively. While 72/79 (91.1%) patients in total fulfilled the RCD, proportion differed across phenotype groups and was lowest in the “Retroperitoneum and Aorta” group (66.7%, p < 0.001). Among the 57 (72.2%) patients meeting the 2019 ACR/EULAR classification criteria, proportion was again lowest in the “Retroperitoneum and Aorta” group (27.8%, p < 0.001).ConclusionThe results from this study indicate that IgG4-RD patients having the “Retroperitoneum and Aorta” phenotype less often fulfill diagnostic criteria and classification criteria than patients with other IgG4-RD phenotypes. Accordingly, this phenotype is at risk of being systematically selected against in observational studies and randomized clinical trials, with potential implications for patients, caregivers and future definitions of IgG4-RD.

Estimating autoimmune hepatitis in patients with hepatic dysfunction

Autoimmune Hepatitis (AIH) is a chronic inflammatory disease of the liver of unknown aetiology characterized by polyclonal hypergammaglobulinemia and circulating autoantibodies. Objective of the study is to find out the prevalence of autoimmune hepatitis and its causative autoantibody (Anti-nuclear, anti-smooth muscle, anti-liver kidney microsomes-1) following comprehensive diagnostic criteria given by International Autoimmune Hepatitis Group. Material and methods250 patients with raised aminotransferase but, negative for the commonest three clinical conditions viz, viral hepatitis, drug induced liver injury and alcohol induced liver injury were suspected to be a case of autoimmune hepatitis. Recent IAIHG criteria was followed in making diagnosis of autoimmune hepatitis. We did the investigation for viral hepatitis (acute/chronic), drug induced liver injury and alcohol induced liver injury and then measured serum IgG. Indirect Immunofluorescence (IIF) was done for Anti-nuclear antibody and was also done for anti-smooth muscle antibody and anti-liver kidney microsomes-1 antibody simultaneously. ResultAs per IAIHG criteria, out of 250 patients, Ig “G” was elevated in 88 patients with significant titres for Anti-nuclear antibody (50 patients), anti-smooth muscle antibody (65 patients), anti-liver kidney microsomes-1 antibody (7 patients) and Anti-mitochondrial antibody (18 patients) were found. As per International Autoimmune Hepatitis Group Comprehensive scoring system, probable diagnosis was made in 83 patients (33.2%) and 12 were confirmed for autoimmune hepatitis (4.8%). ConclusionFemale preponderance was observed and type 1 Autoimmune Hepatitis was most common whereas type 2 Autoimmune Hepatitis was found in few cases. Most patients had anti-smooth muscle antibody and anti-nuclear antibody positivity indicating the type, but no significant difference was seen in clinical symptoms or biochemical parameters between the different types. 162 patients couldn't be evaluated for autoimmune hepatitis because of serum IgG cut-off criteria >1500 ​mg/l.

Pediatric Migraines: A Comprehensive Review and Perspectives on Diagnosis and Treatment.

Pediatric migraine (PM) is one of the most prevalent neurological disorders in children. It has numerous variants and the sufferers often present to emergency departments with a wide variety of signs and symptoms that make diagnosis difficult. The trend in diagnosing and managing PM cases remain suboptimal despite the comprehensive diagnostic criteria and various therapeutic options. In this review, we discuss PM, provide an approach to the diagnosis, and describe the various available management options. However, the diagnosis of migraine is based on history and physical examination; no specific diagnostic test is available. The main management aspects are acute pain relief, prevention, and identifying triggering factors.

A single-centre retrospective study of factors affecting steroid-free remission of immunoglobulin G4-related disease conducted in South Korea: A notable outcome after complete resection.

Few studies have examined factors affecting steroid-free remission (SFR) in patients with immunoglobulin G4-related disease (IgG4-RD). The aim of this study was to investigate clinical factors affecting SFR in IgG4-RD. The medical records of 68 patients who met the 2020 revised comprehensive diagnostic criteria for IgG4-RD were reviewed retrospectively. SFR was defined as remission maintained for at least 6 months without corticosteroids. Cox regression analysis was performed to examine the associations between SFR and various clinical factors. The relapse rate after SFR was examined using the log-rank test. After a median follow-up of 36 months, 30.9% (21/68) of patients with IgG4-RD achieved SFR. Multivariate Cox regression analysis revealed that IgG4-RD diagnosed by complete resection rather than by common diagnostic procedures was the only factor positively associated with SFR (hazard ratio, 7.41; 95% confidence interval, 2.23-24.60; P = .001). Furthermore, relapse after attainment of SFR was significantly less common in the group that underwent complete resection than in the group that did not undergo complete resection (log-rank P = .006). Patients with IgG4-RD diagnosed by complete resection had a higher likelihood of achieving SFR and a lower rate of relapse after attaining SFR.

The external validation of the 2019 ACR/EULAR classification criteria for IgG4-related disease in a large cohort from China

ObjectiveTo externally validate the performance of the 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease (IgG4-RD) within a cohort from China and to compare the criteria with the 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. MethodsThis study included 875 IgG4-RD and 302 non-IgG4-RD cases (213 mimickers and 89 patients with other diseases). Using expert clinical judgment as the gold standard for diagnosis of IgG4-RD, the performance (sensitivity, specificity, area under the curve (AUC) of the 2019 ACR/EULAR criteria for IgG4-RD was evaluated. We also compared it with the 2020 RCD criteria. ResultsThe 2019 ACR/EULAR classification criteria had a sensitivity of 76.6% (95% CI: 73.8% to 79.4%) and a specificity of 98.0% (96.0%-99.4%), an AUC of 0.873 (0.857–0.889) in the overall cohort. Those false negative cases under the 2019 ACR/EULAR classification criteria had significantly lower levels of serum IgG4, and fewer had pathological information, with a higher frequency in the involvement of those uncommon organs compared with the true positive cases. The cases judged as negative by the 2019 ACR/EULAR classification criteria yet judged as “definite” by the 2020 RCD criteria had more involvement of uncommon organs. ConclusionsThe 2019 ACR/EULAR classification criteria for IgG4-RD show outstanding specificity and good sensitivity in real-world clinical practice. The 2020 RCD criteria are helpful for the diagnosis of IgG4-RD in clinical scenarios where IgG4-RD presents as involving an isolated organ, especially the unusual sites.

Association of IgG4-related disease and systemic rheumatic disorders

PurposeAutoimmune disorders can occur together especially in genetically predisposed individuals. We here aimed to assess the occurrence of IgG4-related disease (IgG4-RD) in association with other systemic immune-mediated conditions. MethodsWe retrospectively analyzed the clinical records of patients with IgG4-RD followed at the IgG4-RD Clinic of San Raffaele Hospital (Milan, Italy) for pre-existing or concomitant immune-mediated disorders. IgG4-RD was diagnosed based on histological findings and on the 2011 Comprehensive Diagnostic criteria. Associated immune-mediated disorders were diagnosed based on available classification and/or diagnostic criteria. ResultsTwo-hundred and thirty-four patients with a definitive diagnosis of IgG4-RD were included in this study. A pre-existing immune-mediated connective tissue disease was reported in 6/234 patients (3%): one case each of sarcoidosis, Takayasu arteritis (TA), eosinophilic granulomatosis with polyangitis (EGPA), and rheumatoid arthritis; and two cases of granulomatosis with polyangitis (GPA). Organs involved by IgG4-RD included the lungs, the pancreas, the peritoneum, lacrimal glands, meninges and orbits. Sarcoidosis, EGPA, and TA preceded the onset of IgG4-RD. GPA preceded IgG4-RD onset in one case and occurred simultaneously in the other case. Rheumatoid arthritis occurred together with IgG4-RD in one case. ConclusionOur observation suggests that “secondary” IgG4-RD can present in the context of pre-existing systemic immune-mediated disorders and complicate systemic autoimmune diseases as well as chronic granulomatous conditions. Further studies are needed to define whether this peculiar clinical scenario is associated with different genetic backgrounds, pathological bases, and long-term outcomes.

Performance of classification and diagnostic criteria for IgG4-related disease and comparison of patients with and without IgG4-related disease

IgG4-related disease (IgG4-RD) was recently described in Japan. It is characterised by extensive organ involvement with tissue fibrosis. We assessed the performance of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria as well as differences between patients with and without IgG4-RD. In this retrospective, single-centre study of 50 patients admitted with suspected IgG4-RD, we evaluated the sensitivity and specificity of both criteria. We also compared clinical characteristics and laboratory data of patients with IgG4-RD (n = 42) and patients without IgG4-RD (n = 8). The ACR/EULAR classification criteria had 88.1% sensitivity and 87.5% specificity for IgG4-RD diagnosis. The RCD criteria had 100% sensitivity and 50% specificity. Patients with IgG4-RD had significantly more affected organs (p = 0.002). Patients with a single affected organ and IgG4-RD had significantly higher serum IgG4/IgG ratios (p = 0.027), lower serum C-reactive protein levels (p = 0.020), and lower total haemolytic complement activity (p = 0.044) than those without IgG4-RD. The ACR/EULAR classification criteria have high specificity and the RCD criteria have high sensitivity for diagnosing IgG4-RD. The number of affected organs is important for diagnosing IgG4-RD.

Tear levels of IL-7, IL-1α, and IL-1β may differentiate between IgG4-related disease and Sjögren's syndrome.

We aim to assess and compare a cytokine and chemokine profile in tears from patients with IgG4-related disease (IgG4-RD) and Sjögren's syndrome (SS), and to see if this profile could aid in differentiating these two diseases. We included 10 patients with IgG4-RD who met the Comprehensive Diagnostic Criteria for IgG4-RD and 17 patients who met the AECG criteria for primary SS. The Schirmer-I test was carried out using two standardized sterile tear strips, which were then immediately frozen at - 86°C until assayed. The tears were extracted from the strips after they had been defrosted using a buffer containing 0.5M NaCl and 0.5% Tween-20. The amounts (pg/ml) of the following cytokines and chemokines were then measured using luminometry: IFN-γ, TNF-α, G-CSF, IL-1-α, IL-1β, IL-4, IL-7, IL-12p40, IL-12p70, IL-13, IL-17A, CCL2, CCL3, CCL4, CCL11, and CXCL10. In the IgG4-RD group, seven patients had lacrimal gland involvement, five had dry eye symptoms, and six had a positive Schirmer-I test. In the SS group, 16 (94.1%) had dry eyes and all had a positive Schirmer-I test. We were able to differentiate between both diseases using levels of IL-7, IL-1α, and IL-1β; in particular, the IL-7/IL-1α and IL-7/IL-1β ratios had the best discriminatory potential, with cut-off values of 0.32 (AUC: 0.93, sensitivity: 94%, specificity: 80%, p = 0.0003) and 12.55 (AUC: 0.96, sensitivity: 94%, specificity: 90%, p = 0.0001), respectively. Our results suggest that IL-7, IL-1α, and IL-1β tear levels could help differentiate IgG4-RD from SS. Key Points • The lacrimal gland is frequently involved in IgG4-RD and SS. This characteristic makes both diseases mimics of one another. • Patients with IgG4-RD and SS have different profiles of tear cytokines and chemokines. • Tear IL-7, IL-1α, and IL-1β levels may serve as helpful biomarkers in separating IgG4-RD from SS.

Factors Influencing Vocational Education and Training Teachers’ Professional Competence Based on a Large-Scale Diagnostic Method: A Decade of Data from China

China has the world’s most extensive vocational education and training (VET) system, and VET teachers are central to training high-quality technical and skilled workers. This study aimed to explore the factors influencing VET teachers’ professional competence based on empirical research. We used a large-scale diagnostic method based on the competence measurement of VET, the teachers’ professional competence model. Comprehensive diagnostic tasks, assessment criteria, and test protocols jointly developed by Chinese and German researchers were used to diagnose professional competence. Background questionnaires were collected from 601 VET teachers in 39 vocational colleges and schools in China over ten years. Path analysis was used to explore factors influencing the professional competence of VET teachers. The study found that skill awards, highest academic degree, course type (practical over theoretical), highest level of professional qualification, and enterprise support positively influenced professional competence. School support negatively influenced VET teachers’ professional competence. Organizational identification mediated the relationship between enterprise support, school support, and professional competence. However, professional identity or learning strategy aren’t mediated the relationship between enterprise support, school support, and professional competence. This study provides an empirical basis for enhancing VET teachers’ professional competence and facilitating further development of theories regarding the professional competence of VET teachers.

Advantages of an alternate-day glucocorticoid treatment strategy for the treatment of IgG4-related disease: A preliminary retrospective cohort study.

Alternate-day glucocorticoid (GC) therapy is a treatment option that can reduce GC-associated adverse events. We investigated the safety and efficacy of alternate-day GC therapy in patients with immunoglobulin G4-related disease (IgG4-RD).Medical records of patients with IgG4-RD who were followed for at least one year at St. Luke’s International Hospital, Tokyo, Japan, from 2004 to 2020 were reviewed. Patients who fulfilled comprehensive IgG4-RD diagnostic criteria were divided into alternate-day or daily GC treatment groups based on their treatment protocol. The effect of alternate-day GC therapy on glucocorticoid toxicity index (GTI) score was evaluated using multilinear analysis with adjustments for cumulative GC doses until each assessment point and propensity scores (PS) for alternate-day GC therapy. Kaplan–Meier curves and Cox proportional hazard models were used to assess the efficacy of alternate-day GC therapy for disease control.Among the 67 patients with IgG4-RD, patients with alternate-day (n = 13) and daily (n = 31) GC treatments were analyzed after excluding 23 ineligible patients. The median (interquartile range) age was 64 (60–70) years, 29 (65.9%) were male patients, 26 (59.1%) patients had positive biopsy results, and the median follow-up period was 1643 days. Significantly more patients with alternate-day GC treatment used concomitant immunosuppressants (11 [84.6%] vs 11 [35.5%]; P = .007). The alternate-day strategy significantly lowered the GTI score after adjusting for cumulative GC dose until the assessment and PS (adjusted coefficient: −29.5 [−54.3, −4.8], P = .021 at 12 months; −20.0 [−39.8, −0.1], P = .049 at 24 months). Serious infections were numerically less frequent in the alternate-day group (incidence ratio [95% confidence interval [CI]: 0.45 [0.05, 3.63], P = .45). Most patients (92.3%) in the alternate-day GC treatment group and all patients in the daily GC treatment group showed treatment responses in the remission induction therapy. The PS-adjusted hazard ratio of alternate-day GC treatment for disease flares was not significant (1.55 [0.53, 4.51]; P = .43).The alternate-day treatment strategy significantly reduced GC-related adverse events regardless of the cumulative GC dose. Alternate-day GC treatment is a feasible option for patients with IgG4-RD, without a significant increase in disease flares particularly when combined with immunosuppressants.

Proposing a Framework to Understand the Role of Imaging in Degenerative Cervical Myelopathy: Enhancement of MRI Protocols Needed for Accurate Diagnosis and Evaluation.

Proposing a Framework to Understand the Role of Imaging in Degenerative Cervical Myelopathy: Enhancement of MRI Protocols Needed for Accurate Diagnosis and Evaluation.

Prevalence and Correlates of Sexual Aversion: A Canadian Community-Based Study

BackgroundSexual aversion (SA) is a chronic difficulty impacting sexual, relational and psychological wellbeing. Yet, there is a dearth of studies exploring its prevalence and associated factors. AimTo estimate the prevalence of SA and examine its correlates among a community sample of Canadian adults. MethodsA large web-based sample of the Quebec (Canada) adult population (n = 1,935) completed an online survey on sexual wellbeing. Prevalence rates were estimated for SA and other sexual difficulties. Multivariate logistic regression analyses were used to identify correlates of SA. OutcomesDemographics (eg, gender, employment status), self-reported experiences of sexual difficulties (low sexual desire and arousal, vaginal dryness, pain during sexual intercourse, erectile difficulties, premature or delayed ejaculation, and orgasm difficulties), and markers of psychosexual wellbeing (eg, psychological distress, performance anxiety) according to the presence or absence of SA were assessed. ResultsThe prevalence of SA was 9.7% (95% CI: 8.5–11.2) in the present sample (6.9% [95% CI: 5.1–8.9] in men, 11.3% [95% CI: 9.4–13.4] in women and 17.1% [95% CI: 9.4–27.4] in nonbinary and/or trans individuals). The multivariate logistic regression model explained 31% of the likelihood of experiencing SA. SA was related to psychological distress (aOR: 1.77, 95% CI: 1.33–2.38), sexual satisfaction (aOR: .59, 95% CI:.49–0.70), sexual performance anxiety (aOR: 2.08, 95% CI: 1.45–2.98), and discomfort with sex-related information (aOR: 1.02, 95% CI: 1.01–1.04) Clinical implicationsSeveral psychosexual correlates of SA were documented and could be targeted by practitioners during the assessment and treatment of individuals living with SA. Strengths and limitationsThe study's strengths include its large, gender diverse sample and use of comprehensive diagnostic criteria for SA. Probability-based sampling methods and longitudinal studies should be conducted to address the current study's limitations. ConclusionSA research is critical to document its prevalence in different sociodemographic groups, explore additional intrapersonal and interpersonal mechanisms involved in SA etiology, and ensure that the needs of people living with SA are met with tailored interventions.David Lafortune, Éliane Dussault, Mathieu Philibert, Natacha Godbout. Prevalence and Correlates of Sexual Aversion: A Canadian Community-Based Study. J Sex Med 2022;19:1269–1280.

Isolated IgG4-related cholecystitis with localized gallbladder wall thickening mimicking gallbladder cancer: a case report and literature review

BackgroundIgG4-related cholecystitis, which is a manifestation of IgG4-related disease in the gallbladder, is associated with autoimmune pancreatitis or IgG4-related sclerosing cholangitis in most cases; isolated gallbladder lesions without systemic manifestations are very rare. Gallbladder wall thickening is often diffuse, but sometimes localized, in which case, differentiation from gallbladder cancer becomes difficult. The characteristic features of IgG4-related cholecystitis on imaging that would enable differentiation from gallbladder cancer remain poorly described.Case presentationWe present a rare case of isolated IgG4-related cholecystitis with localized gallbladder wall thickening that was clinically difficult to distinguish from malignancy before resection. An 82-year-old man was referred to our hospital because of gallbladder wall thickening on abdominal ultrasonography without any symptoms. Dynamic computed tomography of the abdomen showed localized wall thickening from the body to the fundus of the gallbladder that was enhanced from an early stage with a prolonged contrast effect. There were no other findings, such as pancreatic enlargement and bile duct dilatation. Magnetic resonance cholangiopancreatography revealed neither dilatation nor stenosis of the bile duct and pancreatic duct. Endoscopic ultrasonography (EUS) showed a smooth layered thickening of the gallbladder wall with a maximum thickness of 6 mm and a well-preserved outermost hyperechoic layer in the same area. Laparoscopic cholecystectomy was performed because malignancy could not be completely ruled out. Pathological examination of a resected specimen revealed IgG4-positive plasma cell infiltration, fibrosis, and phlebitis. Although the serum IgG4 level measured after resection was normal, the condition was ultimately diagnosed as probable IgG4-related cholecystitis according to the 2020 revised comprehensive diagnostic criteria for IgG4-related disease. The EUS images reflected the pathological findings, in which lymphocytic infiltration was distributed in a laminar fashion in the gallbladder wall.ConclusionsAlthough rare, isolated IgG4-related cholecystitis with localized wall thickening mimicking gallbladder cancer remains a clinical problem. A smooth laminar thickening of the gallbladder wall on EUS imaging could be one of the most informative characteristics for differentiating IgG4-related cholecystitis from gallbladder cancer.

Rapidly progressive osteoarthritis of hip: establishing and validating diagnostic criteria in the Southeast Asian population

BackgroundThe study aimed to establish quantitative diagnostic criteria for rapidly progressive osteoarthritis (RPOA) of the hip and to compare the criteria with those for other pathological hip entities in the Asian population.MethodsFrom July 2011 to September 2019, 126 patients who had undergone hip replacement were retrospectively recruited from a fast-track joint replacement list. Patient demographics and radiological parameters were evaluated. Diagnosis of hip RPOA was established based on Lequesne et al’s criteria. The patients with RPOA, hip dysplasia, avascular necrosis, and primary osteoarthritis were allocated to the corresponding groups separately and compared. The diagnostic criteria of RPOA were established and validated in the sample population.ResultsDiagnosis of hip RPOA was confirmed in 18 patients. Their mean age at surgery (72 years) was significantly higher in this group than in the dysplasia and avascular necrosis groups. The mean pelvic tilt parameter (0.485) of RPOA group was significantly lower than those of other groups. The mean initial Tonnis angle (8.35°) of RPOA group was significantly higher than those of avascular necrosis and osteoarthritis groups. The differences were statistically significant between RPOA and non-RPOA groups in limb shortening rate, superior joint space narrowing, acetabular destruction, and head destruction (P < 0.05). Tonnis angle and lateral subluxation also increased significantly during the disease progression.ConclusionPosterior pelvic tilt and increased Tonnis angle may contribute to the pathogenesis of RPOA, leading to progressive acquired acetabular obliquity and lateral subluxation. We propose the modern comprehensive diagnostic criteria be based on the existing literature and the current findings. Further external validation is recommended.

Thoracic involvement and imaging patterns in IgG4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) is a rare orphan disease. Lung, pleura, pericardium, mediastinum, aorta and lymph node involvement has been reported with variable frequency and mostly in Asian studies. The objective of this study was to describe thoracic involvement assessed by high-resolution thoracic computed tomography (CT) in Caucasian patients with IgG4-RD. Thoracic CT scans before treatment were retrospectively collected through the French case registry of IgG4-RD and a single tertiary referral centre. CT scans were reviewed by two experts in thoracic imagery blinded from clinical data. 48 IgG4-RD patients with thoracic involvement were analysed. All had American College of Rheumatology/European League Against Rheumatism classification scores ≥20 and comprehensive diagnostic criteria for IgG4-RD. CT scan findings showed heterogeneous lesions. Seven patterns were observed: peribronchovascular involvement (56%), lymph node enlargement (31%), nodular disease (25%), interstitial disease (25%), ground-glass opacities (10%), pleural disease (8%) and retromediastinal fibrosis (4%). In 37% of cases two or more patterns were associated. Asthma was significantly associated with peribronchovascular involvement (p=0.04). Among eight patients evaluated by CT scan before and after treatments, only two patients with interstitial disease displayed no improvement. Thoracic involvement of IgG4-RD is heterogeneous and likely underestimated. The main thoracic CT scan patterns are peribronchovascular thickening and thoracic lymph nodes.

Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype.

The classification of vasculitis according to a schema with universal acceptance is challenging, given the heterogeneous and protean nature of these diseases. Formal nomenclature and classification criteria for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have suffered several changes since their first description; none provides comprehensive diagnostic and classification criteria. Different factors account for the difficulties in the classification of vasculitis, including the incomplete understanding of the pathogenesis, the multisystemic nature of the disease, the non-specific patterns of vascular involvement, the overlap between entities, and the presence of various classification systems. The present article reviews the classification of AAV considering different points of view, including clinical, serologic, pathogenetic, organ predilection, therapeutic, and prognostic factors, and provides perspectives on future challenges in the understanding of AAV. There is an unmet need for a unifying view of the disease spectrum that considers the constantly evolving paradigms.

IL-6 expression helps distinguish Castleman\u2019s disease from IgG4-related disease in the lung

BackgroundIt is difficult to distinguish between multicentric Castleman’s disease (MCD) and IgG4-related lung disease (IgG4-LD), an IgG4-related disease (IgG4-RD) in the lung.MethodsWe focused on IL-6, which is elevated in MCD, to distinguish between MCD and IgG4-LD by RNAscope, a highly sensitive RNA in situ method. Six cases of MCD and four cases of IgG4-LD were selected.ResultsIn all cases of MCD and IgG4-LD, 10 or more IgG4-positive cells were found in one high-power field. All MCD cases were inconsistent with the pathological IgG4-related comprehensive diagnostic criteria, but 2 of 6 cases had an IgG4/IgG ratio greater than 40%. In all IgG4-LD cases, histological features were consistent with the pathological IgG4-RD comprehensive diagnostic criteria. IL-6 expression was observed in all MCD and IgG4-LD cases except for one IgG4-LD biopsy. IL-6-expressing cells were mainly identified in the stroma. Sites of IL-6 expression were not characteristic and were sparse. IL-6 expression tended to be higher in MCD compared with IgG4-LD. A positive correlation was found between the IL-6 H-score and serum IL-6 level.ConclusionDifferences in IL-6 expression may help distinguish between MCD and IgG4-LD. In addition, the presence of high IL-6 levels may help elucidate the pathological mechanisms of IgG4-LD.

The 2020 Revised Comprehensive Diagnostic Criteria for IgG4-Related Disease. The Research Program for Intractable Disease by the Ministry of Health, Labour and Welfare (MHLW) Japan

The 2020 Revised Comprehensive Diagnostic Criteria for IgG4-Related Disease. The Research Program for Intractable Disease by the Ministry of Health, Labour and Welfare (MHLW) Japan