Objective
IgG4‐related disease (IgG4‐RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. ...This work was undertaken to develop and validate an international set of classification criteria for IgG4‐RD.
Methods
An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included.
Results
A 3‐step classification process was developed. First, it must be demonstrated that a potential IgG4‐RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4‐RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4‐RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval 95% CI 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds.
Conclusion
ACR/EULAR classification criteria for IgG4‐RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.
The diagnosis of pancreatic cystic lesions remains challenging. This study aimed to investigate the diagnostic ability of carcinoembryonic antigen (CEA), cytology, and artificial intelligence (AI) by ...deep learning using cyst fluid in differentiating malignant from benign cystic lesions. We retrospectively reviewed 85 patients who underwent pancreatic cyst fluid analysis of surgical specimens or endoscopic ultrasound-guided fine-needle aspiration specimens. AI using deep learning was used to construct a diagnostic algorithm. CEA, carbohydrate antigen 19-9, carbohydrate antigen 125, amylase in the cyst fluid, sex, cyst location, connection of the pancreatic duct and cyst, type of cyst, and cytology were keyed into the AI algorithm, and the malignant predictive value of the output was calculated. Area under receiver-operating characteristics curves for the diagnostic ability of malignant cystic lesions were 0.719 (CEA), 0.739 (cytology), and 0.966 (AI). In the diagnostic ability of malignant cystic lesions, sensitivity, specificity, and accuracy of AI were 95.7%, 91.9%, and 92.9%, respectively. AI sensitivity was higher than that of CEA (60.9%, p = 0.021) and cytology (47.8%, p = 0.001). AI accuracy was also higher than CEA (71.8%, p < 0.001) and cytology (85.9%, p = 0.210). AI may improve the diagnostic ability in differentiating malignant from benign pancreatic cystic lesions.
Obstructive jaundice is a major symptom of pancreatic head cancer, and although its amelioration is required before scheduling chemotherapy, the decision to perform biliary drainage for resectable ...pancreatic cancer has remained controversial. In recent years, the effectiveness of neoadjuvant therapy for pancreatic cancer has been reported. Preoperative biliary drainage has become increasingly necessary, making the choice of stent an important one; thus, the longer the waiting period extends through neoadjuvant chemotherapy, the more durable stents – such as self‐expandable metallic stents, rather than plastic stents – would be desired as an option. Still, there is insufficient evidence regarding surgical outcomes and long‐term prognosis, and further confirmatory studies are needed. Through this review, we aim to provide an update on the characteristics of biliary stents and preoperative biliary drainage for potentially resectable pancreatic cancer.
Background
The treatment of small (≤ 2 cm), sporadic localized non-functional pancreatic neuroendocrine neoplasms (PNENs) is often controversial. This study aimed to investigate the clinical outcomes ...with observation and surgical resection in small PNENs.
Methods
Seventy-five patients with small localized sporadic non-functional PNENs ≤ 2 cm, who underwent observation or surgical resection, were retrospectively reviewed. Changes in tumor size during follow-up in the observation group were also investigated.
Results
The median age of the cohort was 61 (range 35–81) years. The tumor grades were G1, G2, and unknown, in 61 (81.3%), 8 (10.7%), and 6 (8.0%) patients, respectively. The mean follow-up periods in the observation (
n
= 23) and surgical resection groups (
n
= 52) were 52.3 (range 6.8–133.3) months and 73.0 (range 9.1–179.9) months, respectively. The median overall survival was not reached. During follow-up, no patient died of PNENs, two died of other diseases, three had lymph node metastases, and one experienced recurrence after surgical resection. There was no significant difference in overall survival between the observation and surgical resection groups (hazard ratio: 0.031,
P
= 0.417). The mean change in tumor size in the observation group was + 0.30 mm (range − 1.6 to + 3.0 mm). No deaths, tumor progression, lymph node metastases, distant metastases, or recurrence were noted in patients with PNENs ≤ 1 cm (
n
= 36).
Conclusions
The prognosis of small localized PNENs is good. Observation may be an alternative to surgery in high-risk patients and those with small G1 tumors, particularly those measuring ≤ 1 cm.
Several years have passed since the clinical diagnostic criteria for IgG4‐related sclerosing cholangitis 2012 were published. New findings and knowledge have accumulated since then.
The Research ...Committees for IgG4‐related Diseases and for Intractable Diseases of the Liver and Biliary Tract, in association with the Ministry of Health, Labor, and Welfare of Japan and the Japan Biliary Association, have established a working group consisting of researchers specializing in IgG4‐SC and have drawn up new clinical diagnostic criteria for IgG4‐SC 2020.
The diagnosis of IgG4‐SC is based on a combination of the following six criteria: (a) narrowing of the intra‐ or extrahepatic bile duct; (b) thickening of the bile duct wall; (c) serological findings; (d) pathological findings; (e) other organ involvement; and (f) effectiveness of steroid therapy.
These new diagnostic criteria for IgG4‐SC are useful in practice for general physicians and other non‐specialists.
The Research Committees for IgG4‐related Diseases and for Intractable Diseases of the Liver and Biliary Tract in association with the Ministry of Health, Labour and Welfare of Japan and the Japan Biliary Association created the Clinical Diagnostic Criteria for IgG4‐related Sclerosing Cholangitis 2020 as a revision of the 2012 version.
Background/Purpose
Epidemiological data on immunoglobulin G4‐related sclerosing cholangitis (IgG4‐SC) are scarce.
Method
We randomly selected healthcare facilities from a list of all facilities in ...Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with IgG4‐SC in 2018. Among responding facilities, we further inquired about the clinical characteristics of the patients with IgG4‐SC.
Results
We selected 1180 departments from health centers across Japan. Of them, 532 (45.1%) responded to the questionnaires. The number of reported patients with IgG4‐SC was 1045, and the estimated point prevalence was 2.18 (95% confidence interval, 2.13‐2.23) per 100 000 population. In addition, we enrolled 1096 patients with IgG4‐SC, combining data from previously and newly registered patients. Male patients comprised 79.5% of the population and patients aged 60–80 years had the highest risk for developing the disease. The follow‐up period was 5.1 ± 4.0 years and the 5‐ and 10‐year overall survival rates were 95.3% and 89.0%, respectively.
Conclusions
The point prevalence of IgG4‐SC was 2.18 per 100 000 population. The epidemiological features were similar to those observed in previous studies.
Highlight
Tanaka and colleagues conducted an epidemiological study of immunoglobulin G4‐related sclerosing cholangitis in Japan. The estimated prevalence was 2.18 per 100 000 population. Among 1096 registered patients with the disease, male patients comprised 79.5%, and patients aged 60‐80 years were the most prevalent, confirming the epidemiological findings from previous studies.
Background
Chronic pancreatitis (CP) is a fibro-inflammatory disease of the pancreas. Early diagnosis and intervention, before CP becomes established and irreversible, are essential to improve the ...long-term outcomes. The world’s first diagnostic criteria for early CP were proposed in Japan in 2009, but their clinical utility remains elusive. This study aimed to clarify whether patients with early CP progress to definite CP.
Methods
This is a multicenter, prospective study. Patients diagnosed as having early CP according to the Japanese diagnostic criteria were prospectively followed for 2 years. Clinical profiles including symptoms, drinking and smoking status, laboratory data, imaging findings and treatments were analyzed.
Results
Among the 83 patients who completed the 2-year follow-up period, four (4.8%) patients progressed to definite CP. The diagnosis of 48 (57.8%) patients was unchanged, and that of 31 (37.3%) patients was downgraded. All the four progressive patients were male, alcohol-related, smokers (3 current and 1 ever), and continued drinking. Comparison of the clinical profiles between the progression group (
n
= 4) and non-progression group (
n
= 79) revealed that etiology (alcohol-related), smoking status and presence of acute pancreatitis episodes were associated with the progression to definite CP.
Conclusions
The Japanese diagnostic criteria could identify some patients before the progression to definite CP, while the majority of the patients did not progress. Trial registration number: UMIN000015992.
IgG4‐related sclerosing cholangitis (IgG4‐SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4‐related ...disease. Although clinical diagnostic criteria of IgG4‐SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4‐SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions (CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐SC.
Highlight
IgG4‐related sclerosing cholangitis is recognized as a biliary manifestation of IgG4‐related disease, and differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Kamisawa and colleagues developed consensus guidelines using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐related sclerosing cholangitis.
Retrospective chart audits.
To investigate the optimal timing at which permanent complete cervical spinal cord injury (CSCI) can be confirmed when evaluating paralysis caused by traumatic CSCI.
...Department of Orthopedic Surgery, Spinal Injuries Center, Japan.
Two-hundred and three patients with CSCI that was classified with an American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade A (AIS A) within 72 h of the initial diagnosis of traumatic CSCI were included in the present study. Neurological data from the time of the initial diagnosis to 1 year after the injury were extracted. The number of those with recovery from AIS A and changes of AIS in the recovery were examined.
Thirty-five of 203 (17%) patients whose injuries were initially classified with an AIS A showed recovery from AIS A. Thirty-four of 35 (97%) patients showed recovery from AIS A within 8 weeks after injury.
If CSCI patients with AIS A have not recovered by 8 weeks, the likelihood that they will recover from AIS A is marginal. However, this conversely means that we must consider the possibility that a patient with a traumatic CSCI classified with an AIS A may still show recovery from AIS A within the first 8 weeks after injury.
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