Immunoglobulin G4-related disease associated with cutaneous vasculitis.
TL;DR: Two patients with IgG4related disease associated with cutaneous vasculitis are examined, and the only subclass of human IgG that is unable to activate complement by the classical pathway is examined.
Abstract: © 2014 The Authors. doi: 10.2340/00015555-1707 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Immunoglobulin G (IgG) is composed of 4 Ig isotypes. A new entity called IgG4-related disease has recently been established (1). This disorder is characterised by an elevated serum level of IgG4 and an infiltration of IgG4+ plasma cells, as well as lymphocyte infiltration of various organs. IgG4 is the only subclass of human IgG that is unable to activate complement by the classical pathway. In the present report, we examine two patients with IgG4related disease associated with cutaneous vasculitis.
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TL;DR: The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone and the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed.
Abstract: An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only. Nevertheless, the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed. The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone.
8 citations
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TL;DR: Choroby IgG4-zależne to stosunkowo nowa grupa schorzeń o niewyjaśnionej dotychczas etiologii w surowicy i naciekami tkankowymi z komórek IgG 4-dodatnich z typowym włóknieniem zajętych narzÂdów.
Abstract: Choroby IgG4-zależne to stosunkowo nowa grupa schorzeń o niewyjaśnionej dotychczas etiologii. Charakteryzują się one zwiększonym stężeniem podklasy IgG4 immunoglobulin w surowicy i naciekami tkankowymi z komórek IgG4-dodatnich z typowym włóknieniem zajętych narządów. Zwiększone stężenie IgG4 może występować w wielu innych chorobach przebiegających z przewlekłym stanem zapalnym. W ostatnich latach zwraca się uwagę, że może to dotyczyć również chorych na układowe zapalenia naczyń, szczególnie ANCA-dodatnie. Celem niniejszego opracowania jest chęć zwrócenia uwagi na fakt, iż w niektórych przypadkach zarówno objawy kliniczne, jak i obraz histopatologiczny chorób IgG4-zależnych i układowych zapaleń naczyń mogą być podobne. Znaczenie zwiększonego stężenia IgG4 u chorych z AAV (ANCA-associated vasculitis) jest niejasne i wymaga dalszych badań.
Cites background from "Immunoglobulin G4-related disease a..."
...Publications have described cases of IgG4-related disease with concomitant cutaneous leukocytoclastic vasculitis [20], Henoch-Schönlein purpura [21], or allergic vasculitis with hypocomplementaemia [22]....
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01 Jan 2014
TL;DR: The aim of this study is to draw attention to the fact that in some cases, both clinical presentation and histopathological findings in IgG4-related diseases and systemic vasculitis may be similar.
Abstract: Summary IgG4-related disease is a relatively new group of diseases of still unknown etiology. It is characterized by elevated serum levels of subclass IgG4 immunoglobulin and by abundant infiltration of IgG4+ plasma cells with typical fibrosis of the affected organs. Elevated concentration of IgG4 may be present in many other conditions associated with chronic inflammation. In recent years, it is noted that this may also apply to patients with systemic vasculitis, in particular antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The aim of this study is to draw attention to the fact that in some cases, both clinical presentation and histopathological findings in IgG4-related diseases and systemic vasculitis may be similar. The importance of elevated serum IgG4 immunoglobulin in patients with ANCA-associated vasculitis (AAV) is unclear and requires further research.
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TL;DR: Clinicians should develop ideas and raise awareness of autoimmune-related diseases and Autoimmune hemolytic anemia, also a relatively rare disease that caused by autoreactive erythrocyte antibodies, which rarely overlap.
Abstract: IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.
References
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TL;DR: The IgG4 anti‐proteinase 3 antibodies are able to stimulate neutrophils to undergo a pro‐inflammatory response and may play a role in the pathogenesis of small vessel vasculitis.
Abstract: Anti-proteinase 3 antibodies are implicated in the pathogenesis of small vessel vasculitis. These are primarily immunoglobulin G (IgG), with different subclasses predominating at different stages of disease. However, little is known of their respective roles in pathogenesis. We have previously shown that patient IgG4 was able to induce superoxide release from human neutrophils. To circumvent difficulties in separating the subclasses and additional differences in polyclonal patient antibodies we have generated monoclonal mouse/human IgG1 and IgG4 anti-proteinase 3 antibodies. Using these antibodies we have compared effects of IgG1 and IgG4 on human neutrophils in terms of superoxide release, cytokine production, degranulation and adhesion. Additionally we have investigated the interaction of the subclasses with Fc receptors expressed by the neutrophil. Chimeric antibodies were generated using human constant regions of each subclass and a variable region taken from a monoclonal antibody directed against proteinase 3. Superoxide release from neutrophils was measured by the reduction of ferricytochrome C, degranulation by the conversion of a synthetic colour substrate, cytokine release by interleukin-8 enzyme-linked immunosorbent assay, and adhesion by a flow-based adhesion assay. Fc receptor binding was assessed using blocking antibodies. The IgG4 anti-proteinase 3 was able to induce a dose-dependent release of superoxide, degranulation and adhesion. The antibody was not able to stimulate the secretion of interleukin-8. Fc receptors were essential for neutrophil stimulation and the constitutive Fc receptors were necessary for different stimulatory pathways. The IgG4 anti-proteinase 3 antibodies are able to stimulate neutrophils to undergo a pro-inflammatory response and may play a role in the pathogenesis of small vessel vasculitis.
40 citations
"Immunoglobulin G4-related disease a..." refers background in this paper
...(3) suggested that IgG4 could stimulate neutrophils via Fc receptors and may play a role in the pathogenesis of small vessel vasculitis....
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TL;DR: Here is defined a novel heterophilic antibody interaction and is established the universality of the unique Fc–Fc binding, both involving IgG4.
Abstract: IgG4 has been implicated in a diverse set of complex pathologies – eg autoimmune pancreatitis (AIP), idiopathic membranous nephropathy – and carries unique features including lack of activation of the classical complement pathway and a dynamic Fab-arm exchange We recently showed that the rheumatoid factor (RF)-like activity of IgG4 is achieved through a hitherto unknown, Fc–Fc (and not Fab–Fc as is the case in classical RF; CRF) interaction; hence the name, novel RF (NRF) Here, we further explore the resemblance/difference between CRF and NRF As heterophilic interactions of human IgM RF (CRF) are well known, we checked whether this is the case for IgG4 Human IgG4 showed variable reactivity to animal IgGs: reacting intensely with rabbit and mouse IgGs, but weakly with others The binding to rabbit IgG was not through the Fab (as in CRF) but via the Fc piece, as was recently shown for human IgG (NRF) This binding correlates with the IgG4 concentration per se and could therefore be of diagnostic usage and incidentally explain some observed interferences in biological assays In conclusion, here is defined a novel heterophilic antibody interaction and is established the universality of the unique Fc–Fc binding, both involving IgG4
22 citations
"Immunoglobulin G4-related disease a..." refers background in this paper
...Compared with the other IgG subclasses, IgG4 has a negligible ability to activate the classic complement pathway (1, 4)....
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