Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

Lévy-Sitbon, C.; Barbe, Coralie; Plée, Julie; Goeldel, A.-L.; Antonicelli, Frank; Réguiai, Z.; Jolly, Damien; Grange, Florent; Bernard, Philippe

doi:10.1159/000362717

Cited by 29 publications

(20 citation statements)

References 33 publications

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“…Anti-BP180, but not anti-BP230, titres has been established to correlate well with disease activity (12)(13)(14). Another study, conducted in France (15), found good correlation of BPDAI with clinical parameters of disease activity and proposed a cut-off value of 56 for severe BP.…”

mentioning

confidence: 99%

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

Wijayanti¹,

Zhao²,

Boettiger³

et al. 2017

Acta Derm Venerol

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A significant obstacle in guiding evidence-based management of bullous pemphigoid (BP) is the lack of a standardised, validated scoring system for the condition. The aim of this study is to evaluate the suitability of the Bullous Pemphigoid Disease Area Index (BPDAI) and the Autoimmune Bullous Skin disorder Intensity Score (ABSIS) as outcome measures for BP in clinical trials. Thirty-two BP patients were repeatedly assessed over four years using Physician Global Assessment (PGA), anti-BP180 ELISA titres, BPDAI, ABSIS, BPDAI-Pruritus, Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The reliability, validity, responsiveness, and minimal clinically important differences (MCIDs) were calculated. For inter-rater reliability, the intraclass correlation coefficients (95% CI) were: BPDAI 0.957 (0.901-0.982) and ABSIS 0.881 (0.736-0.949). Compared to ABSIS, BPDAI was better correlated with PGA(r = 0.875, p < 0.001), BPDAI-Pruritus (r=0.632, p = 0.004), ABQOL (r = 0.521, p = 0.011) and TABQOL (r=0.538, p = 0.008). MCIDs for BPDAI were 4-points for assessing clinical improvement and 3-points for deterioration. ABSIS demonstrated less responsiveness with MCIDs at 8.6-points for improvement and 4-points for deterioration. These results indicate that BPDAI demonstrated excellent reliability, validity and responsiveness, while ABSIS had moderate to good reliability, validity and responsiveness.

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confidence: 99%

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

Wijayanti¹,

Zhao²,

Boettiger³

et al. 2017

Acta Derm Venerol

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“…Baseline severity data were available for 67.17% (352 of 524) of cases. There have been several studies considering objective scoring systems for bullous diseases including BP, but there is no published standardized severity scoring system available . Therefore, a measure of severity was suggested as part of the audit as a means of standardizing interpretation across centres; this was primarily based on the number of blisters.…”

Section: Resultsmentioning

confidence: 99%

“…There have been several studies considering objective scoring systems for bullous diseases including BP, but there is no published standardized severity scoring system available. 5,6 Therefore, a measure of severity was suggested as part of the audit as a means of standardizing interpretation across centres; this was primarily based on the number of blisters. Of the 352 patients, 63.9% (225 of 352) had a recorded baseline severity of very mild (< 3 blisters), 21.9% (77 of 352) mild (3-9 blisters), 8.8% (31 of 352) moderate (10-30 blisters) and 5.4% (19 of 352) severe (> 30 blisters).…”

Section: Patient Populationmentioning

confidence: 99%

National audit on the management of bullous pemphigoid

2019

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Summary Background Bullous pemphigoid (BP) is an autoimmune, subepidermal, blistering condition that typically affects elderly people. Aim To undertake a national clinical audit based on standards derived from the British Association of Dermatologists (BAD) clinical guidelines on the management of BP. Methods In 2018, BAD members were invited to submit data for five consecutive adults with BP per centre, who had been under hospital supervision for at least 12 months, in a national audit over an 11‐week period. Results In total, 123 responders from 120 hospitals provided data for 524 cases. Diagnosis was made either clinically (10.7%; 56 of 524) or through histology with direct immunofluorescence (41.6%; 218 of 524), indirect immunofluorescence (10.3%; 54 of 524) or both (37.4%; 196 of 524). Most patients had very mild baseline disease (63.9%; 225 of 352) with 21.9% (77 of 352) considered mild, 9.8% (31 of 352) moderate and 5.4% (19 of 352) severe. Documentation of diabetes, glycated haemoglobin (HbA1c), blood pressure and hypertension was available for 54.1% (283 of 523), 51% (267 of 524), 44.2% (231 of 522) and 61.5% (321 of 522) of cases, respectively. Oral corticosteroids were commenced in 85.5% (448 of 524) of patients, with 38.4% (172 of 448) of these having documented risk of osteoporosis; data regarding prescription of bone‐protection therapies were available for 99.7% (447 of 448) of cases, with 75.6% (338 of 447) of these having a bone‐protection prescription. Patient satisfaction was documented in 59.3% (310 of 523) of cases. Systemic treatment was commenced in 95.9% (502 of 524) of cases during the 12‐month assessment period, with baseline blood test and follow‐up data available for 96.6% (485 of 502) and 95.6% (480 of 502), respectively. Documentation of baseline blood tests was available for 87.4% (424 of 485) of cases, with follow‐up tests recorded in 69.8% (335 of 480). Conclusion Overall, compliance with elements of documentation was moderate or low, whereas standards pertaining to direct care were high.

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“…ABSIS = Autoimmune Bullous Skin Disorder Intensity Score; BPDAI = Bullous Pemphigoid Disease Area Index; CDASI = Cutaneous Dermatomyositis Disease Area and Severity Index; CLASI = Cutaneous Lupus Erythematosus Disease Area and Severity Index; EBDASI = Epidermolysis Bullosa Disease Activity and Scarring Index; EASI = Eczema Acvitity and Scarring Index; MCID = minimal clinically-important difference; PASI = Psoriasis Area and Severity Index; PDAI = Pemphigus Disease Area Index; SCORAD = Scoring Atopic Dermatitis. 1 Rahbar et al, 2014; 2 Murrell et al, 2008; 3 Boulard et al, 2016; 4 Shimizu et al, 2014, 5 Pfutze et al, 2007; 6 Gourraud et al, 2012; 7 Puzenat et al, 2010; 8 Tofte et al, 1998; 9 Hanifin et al, 2001; 10 Breuer et al, 2004; 11 Schram et al, 2012; 12 Sartorius et al, 2010; 13 Loh et al, 2014; 14 Jain et al, 2016; 15 Wijayanti et al, 2014, Wijayanti et al, 2016; 16 Murrell et al, 2012; 17 Patsatsi et al, 2012; 18 Lévy-Sitbon et al, 2014; 19 Bonilla-Martinez et al, 2008; 20 Albrecht et al, 2005; 21 Klein et al, 2011; 22 Goreshi et al, 2012; 23 Anyanwu et al, 2015; 24 Stalder et al, 1993; 25 Schram et al, 2012; 26 Angelova-Fischer et al, 2005; 27 Langley and Ellis, 2004; 28 Zhao et al, 2015.…”

Section: Figmentioning

confidence: 99%

Validation studies of outcome measures in pemphigus

Hanna

Kim

Murrell

2016

International Journal of Women's Dermatology

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Pemphigus is a group of rare and potentially fatal autoimmune blistering diseases that are associated with auto-antibodies that target intercellular adhesion molecules. Incidence of pemphigus varies among populations, with the lowest incidence in Switzerland and Finland at 0.6–0.76 per million per year and the highest in Jewish communities at 16.1–32 per million per year. Pemphigus is associated with devastating morbidity and despite advancements in our understanding of the disease and a widening array of therapeutic options, no cure exists. The delay in the development of a cure may in part be attributed to the absence of a standardized and completely validated severity outcome measures to allow for high-quality multicenter control studies. Such a tool is necessary to define the best practice in clinical studies, allow for accurate comparisons between study results, justify drug use within the clinical setting, and reduce the cost burden that is associated with the use of ineffective therapies. Utilizing outcome measures that are not validated provides an opportunity to synthesize outcome measures with the intent to favor particular treatments and thus produce false conclusions. According to the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) group, a validation of these measurement instruments requires investigating their responsiveness, reliability, and validity. More than 116 outcome measures exist to assess pemphigus severity, of which the Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS) are the most comprehensively corroborated measures. With regard to validity and reliability, PDAI was unsurpassed by ABSIS and PVAS. Data indicate that ABSIS is more reliable than PVAS, but PVAS seems to have greater validity although the results are not consistent. PDAI, ABSIS, and PVAS have not yet had their responsiveness analyzed, which should be the next step to completely validate the outcome measures and conclusively determine which measure is superior.

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Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

Cited by 29 publications

References 33 publications

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

National audit on the management of bullous pemphigoid

Validation studies of outcome measures in pemphigus

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