Mucosal Involvement in Bullous Pemphigoid Is Mostly Associated with Disease Severity and to Absence of Anti-BP230 Autoantibody

Clapé, A.; Muller, C.; Gatouillat, Grégory; Jan, Sébastien Le; Barbe, Coralie; Pham, Bach-Nga; Antonicelli, Frank; Bernard, Philippe

doi:10.3389/fimmu.2018.00479

Cited by 33 publications

(66 citation statements)

References 48 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Such data suggest that the extent of mucosal involvement may be a severity marker [10]. On the basis of previous literature, a total BPDAI score 56 could denote "severe BP" [11]. Hence, BPDAI can be considered as an intuitive method for reflecting the severity of the clinical presentation of BP patients.…”

Section: Bullous Pemphigoid Disease Area Index (Bpdai)mentioning

confidence: 95%

Factors associated with the activity and severity of bullous pemphigoid: a review

et al. 2020

View full text Add to dashboard Cite

Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. It usually affects people older than 70 years of age. The two main autoantigens are BP180 and BP230, both of which are components of hemidesmosomes. Immunoglobulin (Ig)G and IgE autoantibodies to BP180 detected by the enzyme-linked immunoassay (ELISA) show close associations with the activity and severity of BP. In addition, inflammatory cells (eosinophils, neutrophils and mast cells) and cytokines (e.g. interleukins and CC chemokine ligands) play an important part in the pathogenesis, activity and severity of BP. We summarized the potential contribution of each factor postulated to be associated with the activity and severity of BP, and provide guidance for clinicians to pay timely and close attention to such parameters. This review may also promote the development of novel therapies for BP. KEY MESSAGES Bullous Pemphigoid Disease Area Index (BPDAI) is a scoring system which can reflect the extent of clinical involvement of BP patients. The titres of IgE autoantibodies and IgG autoantibodies against the NC16A domain of BP180 are closely correlated with the activity and severity of BP. Many inflammatory cells and molecules, such as eosinophils and interleukins, can also reflect the activity and severity of BP.

show abstract

Section: Bullous Pemphigoid Disease Area Index (Bpdai)mentioning

confidence: 95%

Factors associated with the activity and severity of bullous pemphigoid: a review

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Non-bullous lesions also usually develop during a prodromal stage that may last for several months. Mucosal lesions, which occur in 15–20% of the BP patients, are associated with high disease severity and with absence of anti-BP230 antibodies ( 108 ).…”

Section: Diagnostically Relevant Clinical and Immunopathological Charmentioning

confidence: 99%

Diagnosis of Autoimmune Blistering Diseases

2018

View full text Add to dashboard Cite

Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.

show abstract

“…Indeed, it has been shown that IL-17B is an anti-inflammatory cytokine (46). Given that IL-17RB is expressed by mucosal epithelial cells, such effects could be involved in the regulation of mucosal inflammation that occurs in certain patients with BP (47). However, why a patient would express a higher IL-17B than another patient is still unknown, and whether this level varies according to disease extent for a specific patient still needs to be investigated.…”

Section: Discussionmentioning

confidence: 99%

Characteristic Pattern of IL-17RA, IL-17RB, and IL-17RC in Monocytes/Macrophages and Mast Cells From Patients With Bullous Pemphigoid

et al. 2019

Self Cite

View full text Add to dashboard Cite

Inflammation is largely implicated in bullous pemphigoid (BP), the most frequent skin auto-immune blistering disease. IL-17, essentially IL-17A/F, has been involved in blister formation through regulation of protease production, and its specific serum profile within BP was related to disease outcome. However, relationships between IL-17 family ligands and receptors are quite complex with six different IL-17 isoforms, and five different receptors. We here aimed at clarifying the contribution of the IL-17 axis in BP by characterizing not only the expression of IL-17 receptor (IL-17R) members within immune cells isolated from BP patients (PMNs, n = 9; T-lymphocytes, n = 10; and monocytes, n = 10) but also the expression of IL-17 isoforms in sera (n = 83), and blister fluid (n = 31) of BP patients. We showed that at diagnosis, IL-17RA and IL-17RC expression were significantly increased in monocytes isolated from BP patients as compared to those from control subjects (p = 0.006 and p = 0.016, respectively). Notably, both IL-17RA and IL-17RC mRNA expression remained elevated in BP monocytes at time of relapse. We further demonstrated a significant increase of all IL-17 isoforms tested in BP blister fluid compared with BP serum (IL-17A, p < 0.0001; IL-17A/F, p < 0.0001; IL-17B, p = 0.0023; IL-17C, p = 0.0022; IL-17E, p < 0.0001). Among all, IL-17B was the only cytokine for which a significant decreased concentration within blister fluid was observed in BP patients with severe disease compared to patients with moderate disease (p = 0.012). We further evidenced a significant negative correlation between IL-17B levels and blister/erosion BPDAI subscore (r = −0.52, p = 0.003). We finally identified mast cells as a potential target of IL-17B in lesional skin of BP patients. In conclusion, we showed here that IL-17RA and IL-17RC expression in monocyte was associated with disease activity and evidenced in situ a negative correlation between BP disease activity and IL-17B, whose effects could be mediated by IL-17RB expressed by mast cell in BP lesional skin.

show abstract

Mucosal Involvement in Bullous Pemphigoid Is Mostly Associated with Disease Severity and to Absence of Anti-BP230 Autoantibody

Cited by 33 publications

References 48 publications

Factors associated with the activity and severity of bullous pemphigoid: a review

Factors associated with the activity and severity of bullous pemphigoid: a review

Diagnosis of Autoimmune Blistering Diseases

Characteristic Pattern of IL-17RA, IL-17RB, and IL-17RC in Monocytes/Macrophages and Mast Cells From Patients With Bullous Pemphigoid

Contact Info

Product

Resources

About