Clinical immunology Immunoglobulin G4 is prevailing over immunoglobulin G1 in autoimmunity of pemphigus and bullous pemphigoid: analysis of tissue-bound antibodies in active diseases

A Comparative Analysis of CD32A and CD16A Polymorphisms in Relation to Autoimmune Responses in Pemphigus Diseases and Subepithelial Autoimmune Blistering Disorders

“…Furthermore, the balance between SNPs involved in activating and inhibitory Fcγ receptor signaling may partially affect the development and progression of autoimmune diseases through the regulation of Th1 and Th2 cytokine levels. Our previous studies [5,28,29] demonstrated the role of IgG subclasses in a temporal analysis of humoral response in ABDs, indicating that CD16A polymorphisms (rs396991) are probably linked to the IgG1 subclass in pemphigus, and may therefore serve as a marker of chronic disease or remission.…”

Dew drops on spider web appearance: a newly named pattern of IgG4 deposition in pemphigus with direct immunofluorescence

“…This is particularly true, if tissue examined is fragmented and/or crushed as it happens with mucosal specimens. Regarding DIF patterns in pemphigus, while evaluating deposits of IgG4 using a single-step approach [ 5 ], we noticed that IgG4 deposition is dotty under magnification in the range of 400–600. This dotty pattern resembles dew drops on spider web [ 6 ], so we propose calling this dew drops on spider web appearance ( Figure 1 ) in opposition to the textbook pattern of IgG deposition known as a fish net or chicken wire appearance.…”

“…Putting aside detailed pathogenesis, we suggest that this dew drops on spider web appearance of IgG4 pemphigus deposits as newly described in depth here, that has already been mentioned just incidentally by us in a PubMed accessible reference [ 14 ], should be known by both laboratory workers and dermatology clinicians dealing with pemphigus. The reason for being familiar with this appearance is that evaluating IgG4 deposition in a cost-effective manner with traditional DIF is of diagnostic value, especially in undiagnosed clinically active smoldering pemphigus cases despite this treated with inadequate immunosuppression and thus having equivocal results of serum testing with ELISA techniques and DIF testing for just IgG and C3 deposition, as it enables the detection of Th2-mediated phenomena characteristic for the active stage of pemphigus [ 5 ]. It is interesting in the context of IgG4-mediated autoimmune processes in pemphigus that some patients with IgG4-related disease experience a relapse following rituximab [ 15 ].…”

Clinical immunology Serum trypsin and TCR as novel markers for predicting disease activity in IgG4-related disease

“…Kamisawa for the first time put forward the concept of IgG4-RD in 2003. After that, the disease gradually attracted extensive attention and recognition all over the world [ 6 – 11 ]. In 2010, a review titled “a new syndrome: clinical disease spectrum was related to IgG4” announced the unified name [ 8 ].…”

“…There may be blocked, compression symptoms or organ atrophy, and there may also be a result of cell infiltration or fibrosis resulting in organ failure [ 1 – 5 ]. Therefore, IgG4-RD includes a wide variety of diseases, including autoimmune pancreatitis, Mikulicz's disease, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor [ 6 , 7 ]. Trypsin and T-cell receptor (TCR) are thought to mediate the regulation of B cell maturation, survival and antibody production [ 8 , 9 ].…”