Abstract
BackgroundThe aim of this study was to identify fibrosis-related serological surrogate outcome measures in patients with immunoglobulin G4-related disease (IgG4-RD).MethodsThis was a clinical observational study of 72 patients with untreated IgG4-RD from four institutions in Japan. The serum concentrations of growth differentiation factor 15 (GDF-15), CCL2, hyaluronic acid (HA), amino-terminal propeptide of type III procollagen (PIIINP), and tissue inhibitor of metalloproteinases 1 (TIMP-1) were measured by enzyme-linked immunosorbent assays. The enhanced liver fibrosis (ELF) score was calculated from the TIMP-1, PIIINP, and HA values. We evaluated associations between the values of these biomarkers and laboratory data, the IgG4-RD responder index (IgG4-RD RI) score, and organ involvements.ResultsCompared with the 44 healthy controls, the patients with IgG4-RD showed significantly elevated serum concentrations of GDF-15, MCP-1, HA, PIIINP, and TIMP-1 and ELF scores. The patients’ serum concentrations of GDF-15, CCL2, HA, and TIMP-1 (but not PIIINP) were positively correlated with each other. Among them, serum GDF-15 most efficiently distinguished patients with IgG4-RD from healthy controls. Serum GDF-15 was not associated with the IgG4-RD RI score or the number of organ involvements but was independently associated with the presence of retroperitoneal fibrosis and with parotid gland involvement.ConclusionsWe observed increased serological surrogate outcome measures of fibrosis in IgG4-RD. GDF-15 may precisely reflect the fibrotic degree in patients with IgG4-RD.
Highlights
The aim of this study was to identify fibrosis-related serological surrogate outcome measures in patients with immunoglobulin G4-related disease (IgG4-RD)
We investigated the organ involvements associated with high growth differentiation factor 15 (GDF-15) by performing a multivariate logistic regression analysis
The enhanced liver fibrosis (ELF) score was found to be a clinically useful indicator of active fibrosis and the extent of IgG4-RD [13]. These data indicate the importance of serum biomarkers reflecting the fibrotic process in patients with IgG4-RD and we focused on the significance of GDF-15 and CCL2 in our present investigation
Summary
The aim of this study was to identify fibrosis-related serological surrogate outcome measures in patients with immunoglobulin G4-related disease (IgG4-RD). Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory condition generally characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and (often but not always) elevated serum IgG4 concentrations [1]. Fibrosis and the infiltration of IgG4-positive plasma cells are predominant histopathological features of IgG4-RD. Histopathological findings are the key to the diagnosis of IgG4-RD, a tissue biopsy may not be anatomically available depending on the affected organ(s) (for example, the retroperitoneal space, bile duct, and pancreas). The extent of fibrosis can predict poor responsiveness to immunosuppressive therapies [4]. Fibrosis is an important element of pathogenesis in IgG4-RD. Transforming growth factor beta (TGFβ) and IL-10, which are Foxp3-positive regulatory
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