scholarly journals Validation of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD) 2011, and proposal of a new 2020 version

2021 ◽  
Author(s):  
Takako Saeki ◽  
Mitsuhiro Kawano ◽  
Tasuku Nagasawa ◽  
Yoshifumi Ubara ◽  
Yoshinori Taniguchi ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Diagnostic Criteria ◽  
Test Performance ◽  
Japanese Patients ◽  
Clinical Findings ◽  
Serum Igg4 ◽  
Parotid Swelling ◽  
Type 1 Autoimmune Pancreatitis ◽  
Immunohistological Staining

Abstract Background In 2011, the IgG4-related kidney disease (IgG4-RKD) working group of the Japanese Society of Nephrology proposed diagnostic criteria for IgG4-RKD. The aim of the present study was to validate those criteria and develop a revised version. Methods Between April 2012 and May 2019, we retrospectively collected Japanese patients with kidney disease, for whom data on serum IgG4 values and/or immunohistological staining for IgG4 in renal biopsy samples were available. These patients were classified as IgG4-RKD or non-IgG4-RKD based on the diagnostic criteria for IgG4-RKD 2011, and the results were evaluated by expert opinion. Accordingly, we developed some revised versions of the criteria, and the version showing the best performance in the present cohort was proposed as the IgG4-RKD criteria for 2020. Results Of 105 included patients, the expert panel diagnosed 55 as having true IgG4-RKD and 50 as mimickers. The diagnostic criteria for IgG4-RKD 2011 had a sensitivity of 72.7% and a specificity of 90.0% in this cohort. Of the 15 patients with true IgG4-RKD who were classified as non-IgG4-RKD, all lacked biopsy-proven extra-renal lesions, although many had clinical findings highly suggestive of IgG4-RD. The revised version to which “bilateral lacrimal, submandibular or parotid swelling, imaging findings compatible with type 1 autoimmune pancreatitis or retroperitoneal fibrosis” was added as an item pertaining to extra-renal organ(s) improved the sensitivity to 90.9% while the specificity remained at 90.0%. Conclusion The revised version has considerably improved test performance after addition of the new extra-renal organ item (imaging and clinical findings).

Clinical Evaluation of International Consensus Diagnostic Criteria for Type 1 Autoimmune Pancreatitis in Comparison With Japanese Diagnostic Criteria 2011

Pancreas ◽  
2013 ◽  
Vol 42 (8) ◽  
pp. 1238-1244 ◽  
Author(s):  
Itaru Naitoh ◽  
Takahiro Nakazawa ◽  
Kazuki Hayashi ◽  
Katsuyuki Miyabe ◽  
Shuya Shimizu ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Evaluation ◽  
Autoimmune Pancreatitis ◽  
International Consensus ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals Investigation of Susceptibility Genes Triggering Lachrymal/Salivary Gland Lesion Complications in Japanese Patients with Type 1 Autoimmune Pancreatitis

PLoS ONE ◽  
2015 ◽  
Vol 10 (5) ◽  
pp. e0127078 ◽  
Author(s):  
Takaya Oguchi ◽  
Masao Ota ◽  
Tetsuya Ito ◽  
Hideaki Hamano ◽  
Norikazu Arakura ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Autoimmune Pancreatitis ◽  
Japanese Patients ◽  
Susceptibility Genes ◽  
Type 1 Autoimmune Pancreatitis

Immunostaining With Immunoglobulin G Subclass Antibody Cocktail for Diagnosis of Type 1 Autoimmune Pancreatitis

2020 ◽  
Vol 28 (8) ◽  
pp. 844-849
Author(s):  
Rie Nakata ◽  
Takeshi Uehara ◽  
Mai Iwaya ◽  
Shiho Asaka ◽  
Shota Kobayashi ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Tumor Formation ◽  
Mean Values ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis ◽  
Immunoglobulin G Subclass ◽  
Antibody Cocktail ◽  
Focal Tumor

Background. Immunoglobulin (Ig) G4-related diseases (RDs) are systemic diseases in which serum IgG4 levels are frequently elevated. They can cause diffuse or focal tumor formation, organ swelling, and tissue thickening in organs infiltrated by IgG4 + plasma cells. The diagnostic criteria for IgG4-RDs include an IgG4/IgG ratio >40%, but counting IgG+ cells can be difficult because of the weakness of IgG staining density. We hypothesized that an antibody cocktail of mixed IgG1, IgG2, IgG3, and IgG4 (AC-IgG) might give immunohistochemistry results comparable with those of IgG in IgG4-RD. Methods. We compared AC-IgG reactivity with IgG expression in type 1 autoimmune pancreatitis (AIP), a representative IgG4-RD. We compared immunohistochemistry results using AC-IgG and IgG-only in 10 cases of AIP. The coefficient of variation (Cv) was used to analyze differences between AC-IgG and IgG findings in AIP by 13 board-certified pathologists. Results. Although mean values for IgG+ cells did not significantly differ between AC-IgG (34.3; range = 27.4-37.1) and IgG (30.0; range = 23.0-45.6; P = .6254), Cv was lower for AC-IgG (33.4%) than for IgG (51.4%; regression equation; y[IgG] = 0.988 x + 0.982; correlation coefficient = 0.907). The data showed that the results of both methods were largely consistent. Conclusion. AC-IgG could replace IgG to count IgG+ cells because of its lower Cv.

scholarly journals Evaluation of the Clinical Profile and Malignancies in Children With Neurofibromatosis Type 1

2021 ◽  
Author(s):  
Nihal Şahin ◽  
Ugur Demirsoy ◽  
Funda Corapcioglu
Keyword(s):  
Risk Factor ◽  
Diagnostic Criteria ◽  
Neurofibromatosis Type 1 ◽  
Bone Dysplasia ◽  
Female Gender ◽  
Neurofibromatosis Type ◽  
Clinical Findings ◽  
Degree Relative ◽  
Risk Of Malignancy

Abstract Purpose: Neurofibromatosis type 1 (NF 1) is a significant disease as it is one of the most common autosomal dominant disorders in childhood. Several systems are affected due to significant progression. This study aimed to analyze the clinical findings in children with NF 1 and investigate the characteristics of those with malignancy. Methods: Medical records of 55 children with NF 1 that were followed up for ten years (2004-2015) in our center were analyzed. We assessed clinical and demographical characteristics of patients, presence NF 1 diagnostic criteria, NF 1 related complications, and malignancies. The patients without malignancy are classified in group 1 while patients with malignancy are in group 2. Results: The mean age was 7.68 ± 4.65 years. Female gender was dominant in both groups. Café au lait spots were present in all patients. Axillary-inguinal freckling was observed in 76.4% of patients, followed by neurofibromas in 30.9%, Lisch nodules in 29.1%, bone dysplasia in 14.5%, optic gliomas (OG) in 23.6%, and a history of first degree relative with NF 1 in 63.6%. Central nervous system (CNS) tumors were present in 40%. Tumors beyond CNS were acute myeloid leukemia and schwannoma. None of the diagnostic criteria was a risk factor for malignancy. Having >3 criteria was the risk factor for malignancy in NF-1 (OR:5.891, CI 95%: 1.676-20.705, p=0.006).Conclusions: The major problem is malignancies in NF -1 patients. There are no clearly defined risk factors predicting malignancies in NF-1 at present. However, we found the risk of malignancy higher in patients with more diagnostic criteria.

scholarly journals Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kosuke Minaga ◽  
Tomohiro Watanabe ◽  
Akane Hara ◽  
Ken Kamata ◽  
Shunsuke Omoto ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Normal Serum ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

IgG4-related disease responder index but not serum IgG4 correlates with disease activity and the risk of 1-year relapse in type 1 autoimmune pancreatitis

Pancreatology ◽  
2019 ◽  
Vol 19 ◽  
pp. S135
Author(s):  
Filippo Vieceli ◽  
Giulia De Marchi ◽  
Antonio Amodio ◽  
Lorenzo Brozzi ◽  
Pietro Campagnola ◽  
...  
Keyword(s):  
Disease Activity ◽  
Autoimmune Pancreatitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis
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