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Journal ArticleDOI

Immunoglobulin G4-related disease associated with cutaneous vasculitis.

01 May 2014-Acta Dermato-venereologica (Acta Derm Venereol)-Vol. 94, Iss: 3, pp 327-328

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Journal ArticleDOI
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.

7 citations


Journal ArticleDOI
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.

Journal ArticleDOI
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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Journal ArticleDOI
TL;DR: IgG4-RSD is an underrecognized condition about which knowledge is now growing rapidly, yet there remain many unknowns with regard to its cause, pathogenesis, various clinical presentations, approach to treatment, disease monitoring, and long-term outcomes.
Abstract: Purpose of reviewTo summarize the existing knowledge of various clinical presentations of IgG4-related systemic disease (IgG4-RSD) and to review the evolving list of organs affected by IgG4-RSD.Recent findingsThe term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirel

270 citations


"Immunoglobulin G4-related disease a..." refers background in this paper

  • ...A new entity called IgG4-related disease has recently been established (1)....

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  • ...Compared with the other IgG subclasses, IgG4 has a negligible ability to activate the classic complement pathway (1, 4)....

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Journal ArticleDOI
TL;DR: It is proposed that the IgG4-IgG4 Fc interaction resembles an intermediate of the Fab-arm (half-molecule) exchange reaction that is stabilized because one of the Igg4 molecules is coupled to a solid phase.
Abstract: The Fc fragment of IgG4 can interact with the Fc fragment of another IgG molecule. This interaction is a confounding factor when measuring IgG4 rheumatoid factor levels. Recently, we demonstrated that half-molecules of IgG4 can exchange to form a bispecific Ab. We expected these two phenomena to be related and investigated the physicochemical aspects of IgG4 Fc-Fc interactions. We found that IgG4 is >99% monomeric by size-exclusion chromatography; therefore, IgG4 Fc-Fc interactions in the fluid phase (if any) would be short-lived. However, 125I-labeled IgG4 does bind to IgG1 and IgG4 coupled to a solid phase. By contrast, IgG1 does not bind to coupled IgG4. Furthermore, conditions that induce partial unfolding/dissociation of the CH3 domains enhance IgG4 Fc binding, suggesting that Fc binding is primarily CH3 mediated. IgG4 slowly associates with both IgG4 and IgG1 coupled to a biosensor chip. Remarkably, subsequent dissociation was much faster for IgG4 than for IgG1. Moreover, after binding of an IgG4 mAb to Sepharose-coupled Ag, we observed additional binding of IgG4 with irrelevant specificity, whereas similar binding was not observed with Ag-bound IgG1. We propose that the IgG4-IgG4 Fc interaction resembles an intermediate of the Fab-arm (half-molecule) exchange reaction that is stabilized because one of the IgG4 molecules is coupled to a solid phase. By contrast, IgG4 Fc recognizes IgG1 only after a conformational change that renders CH3(IgG1) accessible to an interaction with the CH3(IgG4). Such Fc interactions may enhance Ag binding of IgG4 in vivo.

108 citations


"Immunoglobulin G4-related disease a..." refers background in this paper

  • ...Moreover, IgG4 has the tendency to interact with other immunoglobulins, such as rheumatoid factor, and reveals an intrinsic affinity for IgG when coated to a solid phase (6, 7)....

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Journal ArticleDOI
TL;DR: In conclusion, patients with IgG4-related skin disease had uniform clinicopathology, and lesions were frequently present on the skin of the periauricular, cheek, and mandible regions, and were frequently accompanied by IgG 4-related lymphadenopathy.
Abstract: IgG4-related disease is a recently recognized systemic syndrome characterized by mass-forming lesions with lymphoplasmacytic infiltration, increase in the number of IgG4(+) cells in affected tissues and elevation of serum IgG4 levels. In 2009, we were the first to report skin lesions in patients with IgG4-related disease, but no large case series has been reported and clinicopathological findings remain unclear. To clarify these features, we herein report 10 patients (9 men and 1 woman; median age, 64 years; age range, 46-81 years) with IgG4-related skin disease. All patients had erythematous and itchy plaques or subcutaneous nodules on the skin of the head and neck, particularly in the periauricular, cheek, and mandible regions, except for one patient, whose forearm and waist skin were affected. In addition, eight patients had extracutaneous lesions: these were found on the lymph nodes in six patients, the lacrimal glands in three patients, the parotid glands in three patients, and the kidney in one patient. Histologically examined extracutaneous lesions were consistent with IgG4-related disease; five of six lymph node lesions showed progressively transformed germinal centers-type IgG4-related lymphadenopathy. Cases of IgG4-related skin disease were classified into two histological patterns: those exhibiting a nodular dermatitis pattern and those with a subcutaneous nodule pattern. The infiltrate was rich in plasma cells, small lymphocytes, and eosinophils; the majority of the plasma cells were IgG4(+). The IgG4(+) cell count was 49-396 per high-power field (mean±s.d., 172±129), with an IgG4(+)/IgG(+) cell ratio ranging from 62 to 92%. Serum IgG4 levels were elevated in all examined patients. In conclusion, patients with IgG4-related skin disease had uniform clinicopathology. Lesions were frequently present on the skin of the periauricular, cheek, and mandible regions, and were frequently accompanied by IgG4-related lymphadenopathy.

77 citations


"Immunoglobulin G4-related disease a..." refers background in this paper

  • ...(2) reported that skin infiltratation was rich in plasma cells with IgG4 positive in patients with IgG4-related skin disease....

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Journal ArticleDOI
TL;DR: A novel role for non‐complement‐activating alloantibodies and MBL in humoral rejection is suggested, as demonstrated in mice reconstituted with monoclonal antibodies to MHC class I antigens.
Abstract: The role of non-complement-activating alloantibodies in humoral graft rejection is unclear. We hypothesized that the non-complement-activating alloantibodies synergistically activate complement in combination with complement-activating antibodies. B10.A hearts were transplanted into immunoglobulin knock out (Ig-KO) mice reconstituted with monoclonal antibodies to MHC class I antigens. In allografts of unreconstituted Ig-KO recipients, no C4d was detected. Similarly, reconstitution with IgG1 or low dose IgG2b alloantibodies did not induce C4d deposition. However, mice administered with a low dose of IgG2b combined with IgG1 had heavy linear deposits of C4d on vascular endothelium. C4d deposits correlated with decreased graft survival. To replicate this synergy in vitro, mononuclear cells from B10.A mice were incubated with antibodies to MHC class I antigens followed by incubation in normal mouse serum. Flow cytometry revealed that both IgG2a and IgG2b synergized with IgG1 to deposit C4d. This synergy was significantly decreased in mouse serum deficient in mannose binding lectin (MBL) and in serum deficient in C1q. Reconstitution of MBL-A/C knock out (MBL-KO) serum with C1q-knock out (C1q-KO) serum reestablished the synergistic activity. This suggests a novel role for non-complement-activating alloantibodies and MBL in humoral rejection.

67 citations


"Immunoglobulin G4-related disease a..." refers background in this paper

  • ...However, it has been shown in mice that non-complement-activating subclasses of antibodies may synergise with other IgG subclasses to activate complement through the lectin pathway (5)....

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Journal Article
TL;DR: These studies demonstrate an example of RF-like Fc-binding reactivity that is conferred by the gamma-4 constant region rather than the classic Ag binding site and suggest that increased production of IgG4 may contribute to the pathogenesis of RA.
Abstract: The majority of plasma cells in rheumatoid arthritis (RA) synovium produce rheumatoid factors (RF). IgG RF predominate in the immune complexes found in RA synovial fluid and have been implicated in the pathogenesis of RA. IgG4 RF are a major component of IgG RF produced in serum and synovium of RA patients, even though this subclass comprises only 4% of the serum IgG. We produced an IgG4 mAb, hRF-1, with RF reactivity from the synovial tissue of a patient with RA. mAb hRF-1 had binding specificity for mammalian IgG similar to Staphylococcus aureus protein A, which is characteristic of RF from patients with RA. To determine the molecular basis of this particular RF reactivity, the heavy and light chain genes of mAb hRF-1 were amplified by PCR, cloned, and ligated into the pSG5 plasmid for expression in COS-7 cells. Chain recombination experiments localized the Fc-binding reactivity to the hRF-1 heavy chain. Using a series of chimeric Ab sequences, the Fc-binding reactivity was mapped to the constant region of IgG4 rather than the variable region involved in classic RF reactivity. Multiple domains, including Hinge, CH2, and CH3 of the IgG4 constant region were required for Fc binding. Our studies demonstrate an example of RF-like Fc-binding reactivity that is conferred by the gamma-4 constant region rather than the classic Ag binding site and suggest that increased production of IgG4 may contribute to the pathogenesis of RA.

56 citations


"Immunoglobulin G4-related disease a..." refers background in this paper

  • ...Moreover, IgG4 has the tendency to interact with other immunoglobulins, such as rheumatoid factor, and reveals an intrinsic affinity for IgG when coated to a solid phase (6, 7)....

    [...]


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