New Recommendations on How to Stage Hidradenitis Suppurativa - PowerPoint Presentation

New Recommendations on How to Stage Hidradenitis Suppurativa Faculty Alexandra B. Kimball, MD, MPH Professor of Dermatology Harvard Medical School Director Clinical Unit of Research Trials in Skin Beth Israel Deaconess Medical Center Boston, Massachusetts

This program will include a discussion of off-label treatment and investigational agents not approved by the FDA for use in the United States.

Kimball AB, et al. Br J Dermatol . 2014;171:1434-1442. Introduction to HS Systemic, chronic inflammatory disease that affects 1% of population Signs and symptoms Abscesses, nodules, fistulas, and scarring Pain and odor Psychosocial burden Typical Locations Affected by HS How do we assess disease severity? Saunte DML et al. Advances in Diagnosis and Treatment. JAMA. 2017;318:2019-2032. Copyright © 2017 American Medical Association. All rights reserved. Figure No Longer Available

a. Gulliver W, et al. Rev Endocr Metab Disord . 2016;17:343-351.b. Zouboulis CC, et al. Br J Dermatol. 2017;177:1401-1409. Tools to Assess Disease Severity Current grading and staging tools [a,b] Hurley staging: oldest and most commonly used Sartorius score HiSCRHS-PGAHS Severity Index

Images courtesy of Alexa B. Kimball, MD. Kimball AB, et al. Brit J Derm . 2014;171:1434-1442.  Hurley Staging System Limitation: assesses only severity of skin condition, not disease activity Abscesses No sinus tractsAbscesses Sinus tractsScarring Multiple, interconnected tracts and abscesses Stage 1 Stage 2 Stage 3

Kimball AB, et al. Brit J Derm . 2014;171:1434-1442.HS-PGA Scoring System Stage: S core Abscesses Draining Fistulas Inflammatory Nodules Noninflammatory Nodules Clear: 0 0 0 0 0 Minimal: 1 0 0 0 Some present Mild: 2 0 0 1-4 – 1 0 – Moderate: 3 0 0 ≥5–1≥1–2-5<10–Severe: 42-5≥10–Very Severe: 5>5–– Used in clinical trials to assess disease severity

Kimball AB, et al. J Eur Acad Dermatol Venereol. 2016;30:989-994. HiSCR Assessment Identifies areas that can show improvement in disease activity S implifies scoring process and increases sensitivity to detect HS-specific lesionsHiSCR Assessment Relative to baseline> 50% reduction in the total number or inflammatory nodules No increase in abscess count No increase in draining fistula count

Practical Use of Assessments A combination of tools provides a strategy to assess and monitor patients for best outcomes Hurley staging + HS-PGA General pain assessment DLQI

a. Jiménez-Gallo D, et al. Mediators Inflamm. 2017;2017:2450401; b. Dufour DN, et al. Postgrad Med J. 2014;90:216-221; c. Newman TM, et al. Radiol Case Rep . 2013;8:737. Additional Testing Bloodwork can help evaluate treatment efficacyCRP and ESR: patients with HS showed ↑ levels of serum proinflammatory cytokines, CRP, and ESR compared with healthy controls[a]Hematocrit: anemia may be present[b]ImagingUS may be useful in visualizing tracts[c]Subcutaneous mass noted in the right axillary region Color Doppler US shows evidence of flow at the periphery of the mass Reprinted from Radiol Case Rep., 8, Newman TM, et al., Hidradenitis suppurativa: Mammographic and sonographic manifestations in two cases, 737, Copyright 2013, with permission from Elsevier. Figure No Longer Available

Long-Term Approach to Treatment Remember: HS is a chronic diseaseMay schedule visits 3 to 4 months apart Manage patient expectations Patient education is crucial regarding the time needed for treatments to become effective Use bloodwork to monitor patients who may be unable to be seen in-office

a. Wolkenstein P, et al. J Am Acad Dermatol. 2007;56:621-623. b. Onderdijk AJ, et al. J Eur Acad Dermatol Venereol. 2013;27:473-478. Psychosocial Burden QoL Effects Assess QoL [a,b] Embarrassment Depression Lack of self-confidence Social functioning Personal relationships Work life QoL should be considered in patient treatment plan

Gulliver W, et al. Rev Endocr Metab Disord. 2016;17:343-351. First-Step Recommendations General recommendations Pain management Weight loss Tobacco cessation Counsel regarding hygiene strategiesTreating physician should be aware of Disease assessment toolsDLQIPain assessment tools

Zouboulis CC, et al. J Eur Acad Dermatol Venereol. 2015;29:619-644. European Guidelines for Treatments Lifestyle modifications and infection/pain management Hygiene and wound care Wide local excisions Biologics AdalimumabInfliximabTopical AntibioticClindamycinDeroofingLasersLocal excisionsSystemic Antibiotics and RetinoidsClindamycin + rifampicine/tetracyclineAcitretin Mild HS SevereHS ModerateHS Surgical Treatment Pharmaceutical Treatment

Zouboulis CC, et al. J Eur Acad Dermatol Venereol . 2015;29:619-644. Treatment by Disease Severity Mild HS Hurley Stage: 1 HS-PGA: Clear→Minimal or Mild Topical antibiotics Systemic antibioticsAdjunctive therapy Hormones (female pts)Anti-inflammatory agents Retinoids Severe HS Hurley Stage: 2 or 3 HS-PGA: Moderate→Very Severe Systemic antibiotics Clindamycin+rifampicin Biologics Adalimumab Infliximab Anti-inflammatory agents Dapsone (stage 2) Cyclosporine A Body wash can be helpful for mild/moderate disease

Concluding Remarks Early diagnosis and treatment of HS offers the best opportunity to limit irreversible damageUse of disease severity assessments as well as patient feedback can help guide treatment decisions As there is no universal treatment available, therapy should be tailored for individual patients Clinicians must reassess and adjust therapies until an effective combination is found for each patient Patient education is crucial regarding realistic timeframes for improvement

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