OBJECTIVE: Autoimmune pancreatitis (AIP) is a diagnosis-challenging disease that often mimics pancreatic malignancy. Pancreatic resection is considered to be a curative treatment for pancreatic ductal adenocarcinoma (PDAC). This meta-analysis aims to study the incidence of AIP in patients who have undergone pancreatic resection for clinical manifestation of cancer.
METHODS: A comprehensive search was conducted in three databases, PubMed, Embase and the Cochrane Library, using the terms \'autoimmune pancreatitis\' and \'pancreatic resection\' and supplemented by manual checks of reference lists in all retrieved articles.
RESULTS: Ten articles were included in the final analysis. 8917 pancreatic resections were performed because of a clinical suspicion of pancreatic cancer. AIP accounted for 140 cases (1.6%). Type 1 AIP comprised the majority of cases, representing 94% (132 cases), while type 2 AIP made up the remaining 6% (eight cases) after further classification. AIP accounted for almost 26% of all cases of benign diseases involving unnecessary surgery and was overrepresented in males in 70% of cases compared to 30% in females. The mean age for AIP patients was 59 years. Serum CA 19 - 9 levels were elevated in 23 out of 47 (49%) AIP patients, where higher levels were detected more frequently in patients with type 1 AIP (51%, 22 out of 43) than in those with type 2 AIP (25%, 1 out of 4). The sensitivity of IgG4 levels in type 1 AIP was low (43%, 21/49 patients).
CONCLUSIONS: Even with modern diagnostic methods, distinguishing between AIP and PDAC can still be challenging, thus potentially resulting in unnecessary surgical procedures in some cases. Serum CA 19 - 9 levels are not useful in distinguishing between AIP and PDAC. Work must thus be done to improve diagnostic methods and avoid unnecessary complicated surgery.

IgG4-related diseases (IgG-RDs) are a group of fibroinflammatory diseases that affect a variety of tissues, resulting in tumour-like effects and/or organ dysfunction. Monoclonal gammopathies (MGPs) are a group of disorders characterized by clonal proliferation of plasma cells or lymphoid cells resulting in the secretion of a monoclonal immunoglobulin. Cases of MGPs in IgG4-RDs coexisting with plasma cell dyscrasias and lymphoid neoplasms have been reported over the past few years. Therefore, the results of examinations of M protein in IgG4-RD patients should be interpreted with caution. Herein, we report the case of a 58-year-old male with a history of type 2 diabetes who presented with submandibular masses, anosmia, swollen lymph nodes, proteinuria, and renal impairment. Laboratory tests revealed hyperglobulinemia and elevated levels of IgG4 (124 g/L) and serum-free light chains (sFLCs). Serum protein electrophoresis (SPEP) revealed an M spike of 5.6 g/dL, and immunofixation electrophoresis (IPE) revealed biclonal IgG-κ and IgG-λ. The patient underwent bone marrow, lymph node, and kidney biopsy, which ruled out plasma cell disorders and lymphoma. He was finally diagnosed with an IgG4-RD comorbid with diabetic nephropathy. The findings in this case highlight that significant activation of B cells in IgG4-RD patients, especially those with multiorgan involvement can lead to significant hyperglobulinemia and high sFLC and IgG4 levels, which are more pronounced in the setting of renal impairment. Relatively high concentrations of polyclonal IgG4 can give rise to a focal band bridging the β and γ fractions, which may mimic the appearance of a monoclonal band on SPEP and monoclonal gammaglobulinemia in IFE. The patient experienced considerable improvement in his symptoms after rituximab combined with glucocorticoid therapy, and a monoclonal immunoglobulin was not detected.

Coronavirus disease 19 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is affecting the world with a surge in cases. A variety of autoimmune diseases occur after SARS-CoV-2 infection or vaccination, of which IgG4-related disease (IgG4-RD) is an important type. IgG4-RD can involve multiple organs of the body. The ocular manifestation of IgG4-RD is called IgG4-related ophthalmic disease (IgG4-ROD). We herein report a patient diagnosed with IgG4-ROD. The patient developed ptosis and vision loss after SARS-CoV-2 vaccination, and the symptoms worsened after SARS-CoV-2 infection. After excluding other diseases like myasthenia gravis and Eaton-Lambert syndrome that may cause ptosis, the diagnosis of IgG4-ROD was confirmed by pathological examination. We discussed the predisposing factors, diagnosis and treatment of this patient to provide a more empirical and theoretical basis for clinical diagnosis and treatment. We conducted a literature review of previously reported cases of IgG4-RD following SARS-CoV-2 infection or vaccination. We retrieved a total of 9 cases, of which 5 developed symptoms after vaccination and 4 after infection. Demographic and clinical characteristics were summarized. In conclusion, our case represents the first case of proven IgG4-ROD after COVID-19 vaccination. We believe that IgG4-ROD and SARS-CoV-2 infection or vaccination are closely related, and the immune system disorder caused by SARS-CoV-2 infection or vaccination may be a key factor in the pathogenesis of IgG4-RD. But for now, there is no direct evidence that there is a causal relationship between SARS-CoV-2 infection or vaccination and IgG4-ROD, which still needs more research and exploration to confirm.

We present a case of a patient with recurrent prostate cancer after treatment for favorable intermediate risk cancer. There was an exceptionally steep increase in PSA from <0.5 to 130ng/mL in 27 months accompanied with the development of bone metastasis. The PSA increase was unexpected. We suspect that this unusual development of metastases must have been caused by an impairment of the immune system caused by his IgG4 disease, and this may have allowed residual prostate cancer cells in the prostate to spread quickly. The influence of IgG4 on cancer is debated.

An increasing number of cases have reported that coronavirus disease 2019 (COVID-19) can lead to immune system dysregulation and induce autoimmune diseases, but the mechanism is unclear. We treated a patient who presented with an unknown fever after infection with the novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in 2023. After excluding the possibility of infection, malignancy, and other connective tissue diseases, we considered the diagnosis of IgG4-related disease in consideration of the patient\'s pathology and clinical findings. In this article, we summarize our diagnostic experience and summarize the case reports of IgG4-related diseases associated with SARS-CoV-2 infections since the COVID-19 pandemic.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated disease that can affect multiple organs, including the orbits, salivary glands, thyroid gland, lungs, aorta, pancreas, bile ducts, lymph nodes, and retroperitoneum. While timely diagnosis is particularly important given the efficacy of glucocorticoid treatment for IgG4-RD, accurate recognition can prove a challenge given the overlap between the imaging features of this disease and other entities.
After a review of the epidemiology, pathophysiology, and clinical considerations (including treatment) associated with IgG4-RD, this pictorial review will showcase the variable imaging manifestations of this disease in the abdomen and pelvis. Post-treatment imaging appearance of these entities will be reviewed and mimickers of this disease in the abdomen and pelvis will be presented.
The presence of mass-like soft tissue with radiographic characteristics of fibrosis affecting multiple organs should raise suspicion for IgG4-RD, although definite diagnosis can only be made with appropriate clinical, serological, and pathologic data.

Immunoglobulin G4-related disease (IgG4-RD)-associated hypercalcemia has rarely been reported. We report a case of IgG4-RD that presented as severe symptomatic hypercalcemia. A 50-year-old woman with a history of sustained bilateral periorbital swelling and proptosis for more than 5 years presented to our hospital complaining of a 3-day history of significant and progressive nausea, vomiting, loss of appetite, fatigue, and pruritus. She denied a long history of medication. On admission, laboratory tests showed severe hypercalcemia with serum adjusted calcium elevated to 4.34 mmol/L and renal dysfunction with serum creatinine elevated to 206 μmol/L. Urinary calcium excretion was increased. The serum IgG4 subclass was markedly elevated to 22.4 g/L with polyclonal hypergammaglobulinemia. Tests of autoantibodies were all negative. Bone metabolism markers that reflect the activity of osteoblasts and osteoclasts were all significantly elevated. However, the levels of intact parathyroid hormone and 25(OH) vitamin D3 were decreased. B-ultrasonography showed chronic inflammation of bilateral submandibular glands. Neither bone marrow biopsy nor positron emission tomography - computed tomography examination showed evidence of neoplastic diseases. The patient was treated with intravenous saline infusion, loop diuretics, salmon calcitonin, glucocorticoids, and hemodialysis with a good response.

UNASSIGNED: Hypertrophic pachymeningitis (HP) is a rare inflammatory disorder characterized by local or diffuse thickening of the intracranial or spinal dura mater. The most frequent cause of HP is antineutrophil cytoplasmic antibodies (ANCA), followed by IgG4. However, few cases of IgG4-HP coexpressing ANCA have been reported. Herein, we present three cases of IgG4-HP coexpressing ANCA and review the relevant literature to document the overlap of these two HP causes as a potential clinical pattern.
UNASSIGNED: We retrospectively analyzed three patients with IgG4-HP coexpressing ANCA in our center and consulted the PubMed database to find other relevant cases reported in English from 1976 to April 2022. We used the following keywords: pachymeningitis, meningitis, dura, antineutrophil cytoplasmic antibody, myeloperoxidase, and proteinase-3. We analyzed the clinical, serological, radiological, and pathological characteristics of the obtained cases based on the ACR and Chapel Hill criteria and the exponential moving average (EMA) algorism for ANCA-associated vasculitis (AAV) and the IgG4-RD Comprehensive Diagnostic Criteria.
UNASSIGNED: We analyzed a total of 10 cases: seven literature reports and our three patients (52- and 61-year-old women and a 65-year-old man). The IgG4-related disease (IgG4-RD) diagnoses were definitive in four cases, and probable and possible in three cases. Eight patients had ANCA against myeloperoxidase (MPO), and two had ANCA against proteinase-3 (PR3). Two patients had both IgG4-RD and AAV, while the others only had ANCA seropositivity without additional clinical or pathological markers of AAV.
UNASSIGNED: With regard to HP, we reconfirmed the existence of the IgG4-RD and AAV overlap syndrome. Meanwhile, our review does not support the hypothesis that ANCA positivity in IgG4-RD results from an excessive B-cell response. We speculate that IgG4-RD and AAV have similar or associated pathogeneses, although uncovering the role of IgG4 and ANCA in these pathophysiological processes requires further investigation.

We report an extremely rare case of hypertrophic pachymeningitis in which a 71-year-old man presented with an intractable recurrent headache for >1 year. During this period, he became positive for immunoglobulin G4 and proteinase 3-antineutrophil cytoplasmic antibodies. Contrast-enhanced magnetic resonance imaging showed characteristic diffuse thickening of the dura. Symptoms were improved by intravenous methylprednisolone (500 mg per day for 5 days) and cyclophosphamide pulse therapy during corticosteroid withdrawal; he remained symptom-free during 1-year follow-up. This case suggests that this disease can be treated by corticosteroids combined with immunosuppressive agents.

The effective conduction of action potential in the peripheral nervous system depends on the structural and functional integrity of the node of Ranvier and paranode. Neurofascin (NF) plays an important role in the conduction of action potential in a saltatory manner. Two subtypes of NF, NF186, and NF155, are involved in the structure of the node of Ranvier. In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), anti-NF antibodies are produced when immunomodulatory dysfunction occurs, which interferes with the conduction of action potential and is considered the main pathogenic factor of CIDP. In this study, we describe the assembling mechanism and anatomical structure of the node of Ranvier and the necessary cell adhesion molecules for its physiological function. The main points of this study are that we summarized the recent studies on the role of anti-NF antibodies in the changes in the node of Ranvier function and its impact on clinical manifestations and analyzed the possible mechanisms underlying the pathogenesis of CIDP.