IgA pemphigus: A systematic review

Kridin, Khalaf; Patel, Payal M.; Jones, Virginia; Cordova, Adriana; Amber, Kyle T.

doi:10.1016/j.jaad.2019.11.059

Cited by 49 publications

(85 citation statements)

References 21 publications

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“…In our review, a total of 17 (18%) neoplasms (mostly hematological) were described in all groups except in patients with exclusively Dsc IgG autoantibodies. Inflammatory bowel disease and other autoimmune diseases have been reported as associated with IgA pemphigus (78), but this association was infrequent in our study.…”

Section: Discussioncontrasting

confidence: 69%

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Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

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Section: Discussioncontrasting

confidence: 69%

“…Lymphoproliferative disorders and solid malignancies have been reported previously in 18% of patients with IgA pemphigus (78). In our review, a total of 17 (18%) neoplasms (mostly hematological) were described in all groups except in patients with exclusively Dsc IgG autoantibodies.…”

Section: Discussionmentioning

confidence: 59%

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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“…Desmogleins and desmocollins are glycoproteins that belong to a superfamily of cadherin molecules. The subcorneal pustular dermatosis subtype exhibits IgA autoantibodies targeting the transmembrane glycoprotein Dsc1, while the antigen of the intraepidermal neutrophilic dermatosis has been found to interact with both Dsg1 and Dsg3 ( 90 , 91 ).…”

Section: Iga Pemphigusmentioning

confidence: 99%

“…In IgA pemphigus, autoantibodies bind to sites containing the monocyte/granulocyte IgA-Fc receptor (CD89), causing a massive inflammatory reaction and neutrophil infiltration of the epidermis, which clinically presents as blistering and pustule ( 4 , 7 , 12 , 90 ). Although the targets of IgA antibodies have been identified, the direct pathogenic effects of the IgA autoantibodies and the exact mechanism of blister formation have not been established, thus a clinical picture of IgA pemphigus is not well known and requires additional investigations ( 90 ).…”

Section: Iga Pemphigusmentioning

confidence: 99%

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

et al. 2021

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Pemphigus represents a group of chronic inflammatory disorders characterized by autoantibodies that target components of desmosomes, leading to the loss of intercellular adhesion between keratinocytes and causing intraepithelial blistering. The pemphigus group consists of four main clinical types with several variants: pemphigus vulgaris (with pemphigus vegetans and pemphigus herpetiformis as variants), pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus (with two clinical variants: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis). Genetic factors are involved in the pathogenesis, with HLA-DR4 (DRB1*0402) and HLA-DRw6 (DQB1*0503) allele more common in patients with pemphigus vulgaris, HLA class II DRB1*0344 and HLA Cw*1445 correlated with paraneoplastic pemphigus, and HLA-DRB1*04:01, HLA-DRB1*04:06, HLA-DRB1*01:01, HLA-DRB1*14, associated with a higher risk of developing pemphigus foliaceus. Autoantibodies are conducted against structural desmosomal proteins in the skin and mucous membranes, mainly desmogleins, desmocollins and plakins. Cell-mediated immunity may also play a role, especially in paraneoplastic pemphigus. Patients may present erythema, blisters, erosions, and ulcers that may affect the skin, as well as mucosal surfaces of the oral cavity, eyes, nose, leading to severe complaints including pain, dysphagia, and fetor. Oral mucosal postbullous erosive lesions are frequently the first sign of disease in pemphigus vulgaris and in paraneoplastic pemphigus, without skin involvement, making the diagnosis difficult. Treatment options classically include immunosuppressive agents, such as corticosteroids and corticosteroid-sparing agents such as azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate or dapsone. Newer therapies focus on blocking cell signaling events induced by pathogenic autoantibodies and/or targeting specific autoantibodies. The disease evolution is conditioned by the treatment with maximum doses of corticosteroids and the side effects associated with long-term immunosuppressive therapy, which is why patients need a multidisciplinary approach in following the treatment. In this review, we provide a comprehensive overview of the epidemiology, pathophysiology, clinical aspect, diagnosis and management of the main intraepidermal blistering diseases from the pemphigus group.

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“…7depicts the frequency of GPIHBP1 autoantibodies in different immunoglobulin classes, organized according to titer grade, revealing that IgA and IgG4 GPIHBP1 autoantibodies were predominant and tended to have the highest titer grade.IgA and IgG4 autoantibodies are known to cause human disease. IgA autoantibodies have been observed in systemic lupus erythematosus(57)(58)(59)(60), pemphigus vulgaris(61,62), and celiac disease(63). IgG4 autoantibodies have been documented in pemphigus vulgaris (61), membranous nephropathy(64), and thrombotic thrombocytopenic purpura(65).…”

mentioning

confidence: 99%

Chylomicronemia from GPIHBP1 autoantibodies

Miyashita

Lutz²,

Hudgins

et al. 2020

Journal of Lipid Research

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Some cases of chylomicronemia are caused by autoantibodies against GPIHBP1, an endothelial cell protein that shuttles lipoprotein lipase (LPL) to the capillary lumen. GPIHBP1 autoantibodies prevent binding and transport of LPL by GPIHBP1, thereby disrupting the lipolytic processing of triglyceride-rich lipoproteins. Here, we review the “GPIHBP1 autoantibody syndrome” and summarize clinical and laboratory findings in 22 patients. All patients had GPIHBP1 autoantibodies and chylomicronemia, but we did not find a correlation between triglyceride levels and autoantibody levels. Many of the patients had a history of pancreatitis, and most had clinical and/or serological evidence of autoimmune disease. IgA autoantibodies were present in all patients, and IgG4 autoantibodies were present in 19 of 22 patients. Patients with GPIHBP1 autoantibodies had low plasma LPL levels, consistent with impaired delivery of LPL into capillaries. Plasma levels of GPIHBP1, measured with a monoclonal antibody–based ELISA, were very low in 17 patients, reflecting the inability of the ELISA to detect GPIHBP1 in the presence of autoantibodies (immunoassay interference). However, GPIHBP1 levels were very high in 5 patients, indicating little capacity of their autoantibodies to interfere with the ELISA. Recently, several GPIHBP1 autoantibody syndrome patients were treated successfully with rituximab, resulting in the disappearance of GPIHBP1 autoantibodies and normalization of both plasma triglyceride and LPL levels. The GPIHBP1 autoantibody syndrome should be considered in any patient with newly acquired and unexplained chylomicronemia.

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IgA pemphigus: A systematic review

Cited by 49 publications

References 21 publications

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

Chylomicronemia from GPIHBP1 autoantibodies

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