Pancreatic tumor in type 1 autoimmune pancreatitis: a diagnostic challenge.

BACKGROUND: The co-occurrence of type 1 autoimmune pancreatitis (AIP) and pancreatic tumor (PaT) has been previously reported. Pure AIP cases have favorable prognosis and are primarily treated with steroids, while AIP cases with PaT are associated with poor prognosis where the primary management is pancreatic resection. However, it's a challenge to timely identify the concurrent PaT in AIP because of their similar clinical and radiological manifestations. METHODS: We retrospectively reviewed the data in two medical centers from January 2010 to April 2019. The inclusion criteria were as follows: 1) completion of abdominal CT imaging before invasive procedures to the pancreas, 2) a final diagnosis of type 1 AIP using the 2011 international consensus diagnostic criteria, 3) follow-up duration of at least one month unless AIP and PaT were identified simultaneously. The presence of PaT in AIP was made based on histopathological confirmation, and the absence of PaT in AIP was defined as no pathological or radiological evidence of concurrent PaT. Clinical and radiological characteristics including gender, age, surveillance period, serum IgG4 and Ca-199 levels, biopsy, extrapancreatic involvement, CT and MR (if performed) imaging characteristics were compared between AIP with and without PaT. The Fisher's exact test was used for qualitative variables, and nonparametric Mann-Whitney test for quantitative variables. A p value ≤0.05 was considered statistically significant. RESULTS: A total of 74 patients with type 1 AIP were included, of which 5 (6.7%) had the concurrent PaT. The subtypes were pancreatic ductal adenocarcinoma (3/5), solitary extramedullary plasmacytoma in the pancreas (1/5) and cholangiocarcinoma in the pancreatic segment (1/5), respectively. Gender (p = 0.044), the pattern of pancreatic enlargement (p = 0.003), heterogeneity (p = 0.015), low-density (p = 0.004) on CT and rim enhancement on MRI (p = 0.050) differed significantly between AIP with and without PaT. None of the low-density characteristics on CT or other assessed MRI characteristics could significantly differentiate the two groups (p>0.05). CONCLUSIONS: Female, focal pancreatic enlargement, pancreatic heterogeneity, low-density on CT and rim enhancement on MRI are suggestive of the concurrent PaT in type 1 AIP. The characteristics of low-density on CT or other MRI characteristics did not provide further diagnostic values.

Sinomenine hydrochloride protects against polymicrobial sepsis via autophagy.

Sepsis, a systemic inflammatory response to infection, is the major cause of death in intensive care units (ICUs). The mortality rate of sepsis remains high even though the treatment and understanding of sepsis both continue to improve. Sinomenine (SIN) is a natural alkaloid extracted from Chinese medicinal plant Sinomenium acutum, and its hydrochloride salt (Sinomenine hydrochloride, SIN-HCl) is widely used to treat rheumatoid arthritis (RA). However, its role in sepsis remains unclear. In the present study, we investigated the role of SIN-HCl in sepsis induced by cecal ligation and puncture (CLP) in BALB/c mice and the corresponding mechanism. SIN-HCl treatment improved the survival of BALB/c mice that were subjected to CLP and reduced multiple organ dysfunction and the release of systemic inflammatory mediators. Autophagy activities were examined using Western blotting. The results showed that CLP-induced autophagy was elevated, and SIN-HCl treatment further strengthened the autophagy activity. Autophagy blocker 3-methyladenine (3-MA) was used to investigate the mechanism of SIN-HCl in vitro. Autophagy activities were determined by examining the autophagosome formation, which was shown as microtubule-associated protein light chain 3 (LC3) puncta with green immunofluorescence. SIN-HCl reduced lipopolysaccharide (LPS)-induced inflammatory cytokine release and increased autophagy in peritoneal macrophages (PM). 3-MA significantly decreased autophagosome formation induced by LPS and SIN-HCl. The decrease of inflammatory cytokines caused by SIN-HCl was partially aggravated by 3-MA treatment. Taken together, our results indicated that SIN-HCl could improve survival, reduce organ damage, and attenuate the release of inflammatory cytokines induced by CLP, at least in part through regulating autophagy activities.

Case Report: Pemphigus in Young Patients With Thymic Anomalies.

Pemphigus is an autoimmune disease that occurs mainly in elderly individuals. Young patients with pemphigus are rare, and the risk factors are unclear. The thymus is associated with a variety of autoimmune diseases, and there have been reports of pemphigus complicated with thymic diseases. Here, we report three cases of young patients with pemphigus that were associated with thymic anomalies. We suggest that thymic anomalies may be a risk factor for the early onset of pemphigus and may be associated with increased severity of the disease. Interventions for thymic diseases have certain benefits for improving the effect of treatments and prognosis of these patients.

Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity.

BACKGROUND: As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribution and a predilection for superficial location. Those involving deep sites are relatively rare. To the best of our knowledge, only one case of primary intraosseous hybrid peripheral nerve sheath tumours has been documented. In this article, we report another case of hybrid peripheral nerve sheath tumours occurring in bone with different clinical, radiological and pathological features from those in the previously reported cases. CASE PRESENTATION: A 28-year-old female presented with a painful nodule in the right tibia. Radiological examination revealed an oval eccentric osteolytic lesion in the proximal tibia. Histologically, the circumscribed but unencapsulated lesion demonstrated biphasic cellular differentiation. Bland, small epithelioid cells arranged in clusters in the myxoid or collagenous stroma and inconspicuous spindle cells scattered in the hypercellular areas were suggested to originate from Schwann cells according to the detection of S100. Both the elongated spindle cells with thin, wavy nuclei and the spindle cells in fascicular or storiform pattern in hypercellular areas showed a positive immunoreaction for epithelial membrane antigen, indicating perineurial differentiation. Based on histological and immunochemical examinations, the patient was diagnosed with hybrid epithelioid schwannoma/perineurioma. The lesion was resected and has not recurred for 8 months since resection. CONCLUSION: The present case is the second primary intraosseous hybrid peripheral nerve sheath tumour to be reported. This is also the first reported intraosseous tumour composed of epithelioid schwannoma and perineurioma with hypercellularity, indicating diverse involvement sites and a wide range of histological features among hybrid peripheral nerve sheath tumours. Awareness of such diversity is critical for accurate diagnoses. The morphological overlap with other spindle and epithelioid cell neoplasms, especially pure peripheral nerve sheath tumours, requires that immunochemical and molecular examinations be used as objective tools to provide the necessary information for a differential diagnosis.

Interleukin-1 Receptor 2: A New Biomarker for Sepsis Diagnosis and Gram-Negative/Gram-Positive Bacterial Differentiation.

This study was undertaken to explore the value of interleukin-1 receptor 2 (IL1R2) as a novel potential biomarker for diagnosis of sepsis and discrimination of gram-negative (G)/gram-positive (G) bacterial sepsis. The study was performed in Kunming mice and septic patients. Inactive Escherichia coli or Staphylococcus aureus were used to stimulate Kunming mice (10 CFU/kg). In clinical study, septic patients with different pathogen infection were studied, and healthy volunteers and patients with systemic inflammatory response syndrome without definite infection were enrolled as control. IL1R2 transcriptions of human subjects' peripheral leukocytes were measured by real-time quantitative polymerase chain reaction assay. IL1R2 serum concentrations of mice and human subjects were measured by enzyme-linked immunosorbent assay. The value of IL1R2 as a biomarker was compared with procalcitonin (PCT), C-reactive protein (CRP), and Acute Physiology and Chronic Health Evaluation II (APACHE II). The results showed that IL1R2 expression was upregulated in mice treated with inactive Escherichia coli and septic patients. The elevation of serum IL1R2 was more significant in septic patients infected by Escherichia coli or G bacteria than in those infected by Staphylococcus aureus or G bacteria. For sepsis diagnosis and G/G bacterial sepsis discrimination, serum IL1R2 was more sensitive and specific than the traditional biomarkers such as PCT, CRP, and APACHE II as shown by the receiver operating characteristic curves. It was suggested that IL1R2 was a potential biomarker for diagnosis and G/G bacterial differentiation in sepsis.