IgG4-related diseases: state of the art on clinical practice guidelines

Abstract

Immunoglobulin G4-related diseases (IgG4-RD) are a group of chronic relapsing–remitting inflammatory conditions, characterised by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, fibrosis and a usually favourable response to steroids.In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the European Reference Network ReCONNET, aimed at evaluating existing clinical practice guidelines (CPGs) and recommendations in IgG4-RD. From 167 publications initially obtained from a systematic literature search, only one was identified as a systematic multispecialist, evidence-based, consensus guidance statement on diagnosis and treatment of IgG4-RD, which may be recommended for use as CPG in IgG4-RD.With the recognition of a limited evidence based in this increasingly recognised disease, the group discussion has identified the following unmet needs: lack of shared classification criteria, absence of formal guidelines on diagnosis, no evidence-based therapeutic recommendations and lack of activity and damage indices. Areas of unmet needs include the difficulties in diagnosis, management and monitoring and the scarcity of expert centres.

Effects of a Cloth Panel Containing a Specific Ore Powder on Patients with Japanese Cedar Pollen Allergy During the Pollen Dispersal Season

“…The formula generated in this study also indicated that IgG4 was extracted with a negative coefficient, even though the importance was less than that of the cytokines. Recently, IgG4 has been implicated to play a role in various diseases that have shown elevated serum IgG4, IgG4-positive plasma cell infiltration into affected tissue, and fibrosis [18][19][20]. However, IgG4 was initially considered in relation to the allergic condition.…”

“…Immunoglobulins (Igs): Ig G, A, M, and non-specific Ig E, as well as Ig G4 since Ig G4 is related to food allergy. More recently, Ig G4 has been considered to be associated with IG G4-related diseases, which are systemic diseases characterized by elevated serum IgG4, IgG4-positive plasma cell infiltration into the affected tissue, and fibrosis [18][19][20].…”

Immunoglobulin G4‐related disease in a dog

“…Treatment is expected to include both resolution of mass‐like lesions and a decrease in serum IgG4 concentration, but, serum IgG4 concentrations can remain high after treatment . Recrudescence of disease is expected in affected humans but has not been observed in our case, at the time of writing . Monitoring serum IgE and IgG4 concentrations has been proposed to help predict disease relapse in affected human patients and could be attempted in affected dogs …”

“…Human patients with IgG4‐RD typically are highly responsive to glucocorticoids, if treatment is initiated before the onset of substantial fibrosis . Treatment is expected to include both resolution of mass‐like lesions and a decrease in serum IgG4 concentration, but, serum IgG4 concentrations can remain high after treatment .…”

Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey

“…Since ERN ReCONNET aims at improving the level of adherence to CPGs across European HCPs, by providing practical tools and training to healthcare professionals, many of the initiatives emerging from this survey are being realised under the scope of the ERN ReCONNET, following the suggestions, expectations and points of view of the member HCPs and ePAGs. The ERN ReCONNET has already reviewed the landscape of the existing CPGs in rCTDs [10][11][12][13][14][15][16][17][18][19] and the existence of partially sufficient CPGs for some rCTDs was highlighted, while demonstrating the absence of evidence-based CPGs for other rCTDs. [10][11][12][13][14][15][16][17][18][19] Among the different unmet needs on CPGs, the results raised the issue that more attention should be devoted to the assessment of CPGs adherence in routine clinical practice.…”