An Oral Disease Severity Score validated for use in oral pemphigus vulgaris

Abstract

This study has validated the ODSS for the assessment of oral PV. It has shown superior inter- and intraobserver reliability to PDAI, ABSIS and PGA and is quick to perform.

British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017

“…Two validated severity scoring systems that have become frontrunners are the Pemphigus Disease Area Index (PDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), each taking 2–5 min to complete . These have also been validated for use in oral PV but are inferior to another system, the Oral Disease Severity Score, which may be combined with ABSIS or PDAI in patients with skin or extraoral mucosal sites …”

“… Measurement of baseline parameters prior to starting treatment.As a minimum this should include ○ Weight ○ Blood pressure and whether there is a clinical history of hypertension ○ Height (children) ○ Blood glucose and HbA1c and whether there is a clinical history of diabetes ○ Pregnancy test (if appropriate) ○ Full blood count, renal and liver function tests. Appropriate investigations to establish diagnosis.As a minimum this should include ○ A lesional skin/mucosal biopsy for routine histopathology ○ Perilesional skin/mucosal biopsy for DIF (alternatively, IIF or desmoglein ELISA if biopsy is not possible). Evidence of appropriate drug monitoring.For patients on corticosteroids, as a minimum this should include regular measurements of or documentation of ○ Blood pressure ○ Weight ○ Blood glucose/HbA1c ○ Height (children) ○ Renal function ○ Evidence that gastric and bone prophylaxis is considered Symptoms suggestive of important side‐effects, for example peptic ulceration or visual decline.Other investigations are dependent on the choice of adjuvant drug but should include documentation of baseline investigations relevant to the drug in question and evidence of appropriate follow‐up monitoring. Adherence to guidelines for prophylaxis and management of steroid‐induced osteoporosis Use of objective disease‐scoring methodologies to assess clinical outcomes, for example PDAI, ABSIS or the Oral Disease Severity Score …”

Mucous membrane pemphigoid and oral blistering diseases

“…The Oral Disease Severity Score (ODSS) is a disease scoring tool that has been validated for MMP, PV and oral lichen planus. 19,20 The MMP Disease Activity Index (MMPDAI) has also been proposed, but awaits validation. 21 Measurement of quality of life (QoL) provides a patient-based measure to assess disease burden and monitor activity.…”

Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation, investigations and management

“…If there is ongoing disease activity at any site, including in the mouth, further dose reductions may result in relapse. Use of an oral disease severity score sheet (ODSS) provides a systematic approach to examining the mouth . Second‐line treatment is instigated if treatment failure occurs, which is defined by international consensus as the lack of disease control after 12 weeks of azathioprine 2.5 mg/kg/day, mycophenolate mofetil 1.5 g twice daily, cyclophosphamide 2 mg/kg/day or methotrexate 20 mg/week alongside corticosteroids.…”