Serum BAFF and APRIL levels in patients with IgG4-related disease and their clinical significance

Kiyama, Kazuhiro; Kawabata, Daisuke; Hosono, Yuji; Kitagori, Koji; Yukawa, Naoichiro; Yoshifuji, Hajime; Omura, Koichiro; Fujii, Tomoyuki; Mimori, Tsuneyo

doi:10.1186/ar3810

Cited by 65 publications

(36 citation statements)

References 47 publications

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“…4A). Consistent with previous reports (41,42), this elevation correlated with elevated serum BAFF levels (Fig. 4A).…”

Section: Pancreatic Infiltration Of Ifn-a Producing Pdcs In Patients supporting

confidence: 81%

“…The latter possibility is suggested by the fact that both pathways result in enhancement of BAFF production by innate cells and the subsequent production of IgG4 by B cells (16,17). Clinical observations showing that serum BAFF levels are significantly higher in patients with IgG4-related AIP than in normal individuals (41,42) support the importance of this cytokine in the immunopathogenesis of this disease.…”

Section: Discussionmentioning

confidence: 61%

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Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis

Arai

Yamashita

Kuriyama

et al. 2015

The Journal of Immunology

132

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The abnormal immune response accompanying IgG4-related autoimmune pancreatitis (AIP) is presently unclear. In this study, we examined the role of plasmacytoid dendritic cell (pDC) activation and IFN-α production in this disease as well as in a murine model of AIP (MRL/Mp mice treated with polyinosinic-polycytidylic acid). We found that the development of AIP in treated MRL/Mp mice occurred in parallel with pancreatic accumulation of pDCs producing IFN-α, and with pDC depletion and IFN-α-blocking studies, we showed that such accumulation was necessary for AIP induction. In addition, we found that the pancreas of treated MRL/Mp mice contained neutrophil extracellular traps (NETs) shown previously to stimulate pDCs to produce IFN-α. Consistent with these findings, we found that patients with IgG4-related AIP also exhibited pancreatic tissue localization of IFN-α–expressing pDCs and had significantly higher serum IFN-α levels than healthy controls. In addition, the inflamed pancreas of these patients but not controls also contained NETs that were shown to be capable of pDC activation. More importantly, patient pDCs cultured in the presence of NETs produced greatly increased levels of IFN-α and induced control B cells to produce IgG4 (but not IgG1) as compared with control pDCs. These data suggest that pDC activation and production of IFN-α is a major cause of murine AIP; in addition, the increased pDC production of IFN-α and its relation to IgG4 production observed in IgG4-related AIP suggest that this mechanism also plays a role in the human disease.

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“…4A). Consistent with previous reports (41,42), this elevation correlated with elevated serum BAFF levels (Fig. 4A).…”

Section: Pancreatic Infiltration Of Ifn-a Producing Pdcs In Patients supporting

confidence: 81%

Section: Discussionmentioning

confidence: 61%

Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis

Arai

Yamashita

Kuriyama

et al. 2015

The Journal of Immunology

132

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“…Overproduction of BAFF in transgenic mice has been shown to lead to pathological findings resembling those observed in pSS (12) . On the other hand, higher levels of BAFF that have been detected either in the serum or saliva of pSS patients may support this association in humans (13,14) . Besides playing a crucial role in salivary gland inflammation and in the pathophysiological mechanisms of pSS, the increased expression of BAFF may also participate in ocular surface disorders that are frequently observed in pSS patients.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome

Arıkan¹,

Gökmen²,

Çömez³

et al. 2015

Arquivos Brasileiros De Oftalmologia

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“…In the 6 studies in which the ratio was detailed, 3 used a minimum ratio of 30%, 1 used 40%, and 2 used 50%. 6,8,44,[57][58][59] The mean IgG4þ plasma cell/total IgGþ plasma cell ratio per HPF specified in 139 cases was 58%, ranging from 0% to 100% (Table 2). A major example of the utility of the IgG4þ plasma cell/total IgGþ plasma cell ratio is in retroperitoneal fibrosis, a condition in which the fibrotic nature of the disease is frequently so dominant at the time of diagnosis that the lymphoplasmacytic infiltrate present early in this condition is sparse by the time the patient comes to medical attention.…”

Section: Morphological Datamentioning

confidence: 99%

Diagnostic Approach to the Complexity of IgG4-Related Disease

Stone

Brito-Zerón

Bosch

et al. 2015

Mayo Clinic Proceedings

153

224

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IgG4-related disease (IgG4-RD) is a systemic disease characterized by the infiltration of IgG4-bearing plasma cells and, more importantly, distinctive histopathological features: storiform fibrosis, obliterative phlebitis, a lymphoplasmacytic infiltrate, and mild-to-moderate tissue eosinophilia. The diagnostic approach is complex and relies on the coexistence of various clinical, laboratory, and histopathological findings, none of which is pathognomonic in and of itself. IgG4-related disease should be suspected in patients presenting with unexplained enlargement or swelling of 1 or more organs or tissue organs. Four laboratory abnormalities often provide initial clues to the diagnosis of IgG4-RD: peripheral eosinophilia, hypergammaglobulinemia, elevated serum IgE levels, and hypocomplementemia. Elevated serum IgG4 levels provided critical information in identifying the first cases of IgG4-RD, but recent studies have reported substantial limitations to the measurement of serum IgG4 concentrations, precluding reliance on serum IgG4 concentrations for diagnostic purposes. In contrast, new studies have suggested a promising role of flow cytometry studies in the diagnosis and longitudinal management of IgG4-RD. Demonstration of the classic histopathological features of IgG4-RD remains crucial to diagnosis in most cases, and biopsy proof is preferred strongly by most disease experts before the initiation of treatment. Of note, the multiorgan nature of IgG4-RD was first established in 2003. This review intends to provide most recent knowledge about the clinical, laboratory, radiological, and pathological characteristics of IgG4-RD that may guide the physician to establish an early diagnosis. We searched PubMed and MEDLINE for relevant articles published between January 1, 2000, and November 1, 2014, using the search terms IgG4 and IgG4-related.

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Serum BAFF and APRIL levels in patients with IgG4-related disease and their clinical significance

Cited by 65 publications

References 47 publications

Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis

Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis

Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome

Diagnostic Approach to the Complexity of IgG4-Related Disease

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