the role of extracorporeal photopheresis in scleroderma
TRANSCRIPT
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The Role of Extracorporeal
Photopheresis in Scleroderma
Jaehyuk ChoiAssistant Professor
Department of DermatologyDirector, Extracorporeal Photopheresis Unit
10/15/16
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Outline of the Talk
• Brief introduction to Scleroderma Pathophysiology
• Brief introduction to ECP.• What is it? What is it used for? How does it work?• Lessons from other diseases
• Discussion of clinical studies in scleroderma• Ideal candidates for ECP.• Timing of treatments• Goals of treatment
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Scleroderma• Fibrosing disease.
• Area of High Clinical Need!
• Better insurance coverage for off-label use of promising therapeutics.
• As of summer, 2015, there are no FDA-approved agents for scleroderma.
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Immunopathogenesis of SSc• Clear role of immune system in Scleroderma• Genetics show mutations in innate immune genes and adaptive immune genes
predispose to this disease.• Immunointerventions will likely be critical for effective treatment of this disease!
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Immunopathogenesis of SSc• Two types of treatments.
• Inhibit T helper cells.• Upregulate Treg cells..
https://pharmaceuticalintelligence.com/tag/shrna/
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• Can we selectively increase Treg’s in scleroderma?
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Is there a role for photopheresis?
• Photopheresis• One of the first FDA-
approved immunotherapies• Now utilized world-wide
over 1 million times.• FDA indicated for
scleroderma-like conditions.• FDA-approved for CTCL,
GVHD, and lung/heart transplant rejection.
• Despite promising data, not yet FDA approved for scleroderma.
Choi, et al. Wolverton
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What is photopheresis?
Choi, et al. Wolverton
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ECP-Why use for scleroderma?
• Mechanism of Action• Effective in analogous diseases• Data suggests multiple benefits for patients
with scleroderma
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Mechanisms of Action• Mechanisms of Action
• ECP generates dendritic cells
• ECP generates apoptotic cells.
• ECP generates Tregs• Apoptotic cells and
Tregs dampen inflammation.
Marshall SR (2006) Technology Insight: ECP for the treatment of GvHD—can we offer selective immune control without generalized immunosuppression? Nat Clin Pract Oncol 3: 302–314 doi:10.1038/ncponc0511
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Generation of Tregs• Indirectly through the activation of antigen presenting cells
https://pharmaceuticalintelligence.com/tag/shrna/
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Mechanisms of Action• 8-MOP and UV light induces
apoptosis
• Passage of blood through ECP induces antigen presenting cells.
• Infusion of apoptosis generates Tregs in the graft.
• The combination of apoptotic cells and APCs may be tolerogenic.
Blood. 2008 Aug 15; 112(4): 1515–1521.
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Fig 1. Time course of FEV1 and peripheral blood CD4+CD25high Treg cells in six lung transplant (Tx) recipients treated with ECP. Following the standard protocol used at our institution,8 ECP procedure was initially performed three times weekly and thereafter patients were treated on 2 consecutive days at 2-week intervals for 3 months. Arrows indicate start of ECP treatment. F. Meloni, A. Cascina, S. Miserere, C. Perotti, P. Vitulo, A.M. Fietta
Peripheral CD4+CD25+ TREG Cell Counts and the Response to Extracorporeal Photopheresis in Lung Transplant Recipients
Transplantation Proceedings, Volume 39, Issue 1, 2007, 213–217
http://dx.doi.org/10.1016/j.transproceed.2006.10.227
Triangles- FEV1Squares- CD4+CD25high
In other diseases, when the treatmentIs effective, Treg’s increase.
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Immunopathogenesis of SSc• Increase in Tregs should help disease.
https://pharmaceuticalintelligence.com/tag/shrna/
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ECP Clinical Indications• ECP
• Originally Approved for CTCL• Repurposed for other diseases involving clonal T cells
• Autoimmune diseases and GVHD• Other medicare-approved indications
• GVHD• Heart and lung transplant
• Anecdotal usage• Lichenoid dermatoses• Sclerodermoid dermatoses.• Crohn’s and UC
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Chronic cGVHD
Dermatology 2008;216:287–304
• cGVHD• Heterogeneous disease.• Lichenoid lesions• Poikilodermatous lesions• Morpheaform• Eosinophilic fasciitis
• Morbidity to the patients• Risks associated with systemic
immunotherapy.• Functional deficits
• Ulcers• Special sites- Hands
• Chest- Restrictive lung disease.
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Treatment for GVHD
• Treatment of GVHD Is Similar to Treatment of Scleroderma• Steroids• Systemic Immunosuppression
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What can we learn from our GVHD experience?
• Improvements in disease• Reductions in systemic immunosuppression• Improvements in overall survival
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ECP for acute GVHD
doi:10.1038/ncponc0511
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ECP for chronic GVHD
doi:10.1038/ncponc0511
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What is the data for ECP in GVHD?
• Improvements in disease
• Reductions in systemic immunosuppression
• Improvements in overall survival
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Immunosuppression can be effectively reduced
• Foss, et al.
• 23 patients• Heavily pretreated.• 2 days/2 weeks
• Improvement in 71% of patients• 19/23 had decrease or
cessation of immunosuppressives• 11/23 stopped pred.• 12/23 decreased or stopped
MMF• 5/23 decreased or stopped
prograf.
• Dignan, et al.
• 38 patients with steroid-refractory GVHD
• 27 completed 6 months of ECP
• 19/27 had PR or CR
• 20 had a reduction in immunosuppressive dose.• 17 patients had less steroids
• 5 stopped completely, 4 >75% reduction, 4 >50% reduction, and 4<50% reduction.
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What is the data for ECP in GVHD?
• Improvements in disease• Reductions in systemic immunosuppression
• Improvements in overall survival
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Improvements in overall survival***
• 59 patients• Heavily pretreated.• 1-2 x/week until
improvement• Then 2 days/q2-4 weeks
This figure was published in Haematologica, Volume 91: Greinix et al, The effect of intensified extracorporeal photochemotherapy on long-term survival in patients with severe acute graft-versus-host disease, pp 405 408, Copyright a 2006 Ferrata Storti Foundation.
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ECP- Data suggests earlier utilization of ECP is better for patients
• Why?• Some GVHD is irreversible.• Some immunosuppression
side effects are irreversible• Compression fractures.
• Earlier the better• Couriel, et al. suggests a
better response for the patients who have de novo diagnosis of GVHD
Type No. CR/PRDe Novo 17 13Progressive
16 7
Relapsing 30 17
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ECP- How frequently?Reference Regimen Patient
s (n)CR or PR (skin)
CR or PR (liver)
CR or PR (oral)
Duration of Tx
Besnier 3x/week 5 4/4 1/ - 13-30 tx’s
Smith 0.67-3/wk
18 4/10 3/13 2/7 6-48 tx’s
Child 2x/month
11 10/10 1/6 - -
Salvaneschi 3x/wk 14 10/12 6/9 8/12 1-32 mos.
Apisrnthanarax Varied 32 19/32 - - 12-98
tx’sKanold 3x/wk 63 31/51 24/33 - -Messina 2x/wk 44 20/36 12/20 - -Bisaccia 3x/wk 6 4/6 3/3 2/2 2-13
mos.Rubegni 1x/wk 32 22/27 18/23 23/25 ~35 txFoss 2x/wk 25 15/25 0/6 6/13 3-24
mos.Overall 72% 63% 74%
doi:10.1038/ncponc0511
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ECP-How long? • ECP
• Takes time• Median time to
response.• Steroid
discontinuation• Median time to
discontinuation in Foss study= 84 days
BLOOD, 1 OCTOBER 2008
VOLUME 112, NUMBER 7
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Limitations of the clinical studies
• Limitations:• Small clinical studies• Many are retrospective• Few are randomized or placebo controlled• Almost none are double-blinded
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ECP in sclerosing diseases
• Case Reports• Uncontrolled case series• Controlled studies
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• 49 yo woman
• 10 years ago developed a rash on her skin
• Over next 5 years, the rash spread.
• She developed pruritus and tenderness.
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• Over the past two years, developed arthralgias.
• Increasing limitations to mobility.
• Inability to work a full day.
• Frequent awakening at night with pain and discomfort in her skin and joints.
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• On examination, cobblestoning of the skin.
• Coalescing indurated bound down, reddish purple dyspigmented plaques.
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• Heavily pretreated.• Topical and systemic
steroids.• Azathioprine• MMF• Skin-directed therapy
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• ECP• 2 consecutive days at 2-
week intervals
• Other treatments• Weekly physical therapy
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
The patient's abdominal skin prior to treatment. Indurated and bound-down plaques coalesced to involve most of the abdominal skin. These lesions were tender to palpation and pruritic.The patient's abdominal skin after 15 months of treatment. The indurated plaques had completely resolved, with marked softening noted. A residual reticulated erythema remained.
• Improvements with ECP monotherapy• Softening of skin on her
abdomen and upper and lower extremities.
• Resolution of her rash on her skin.
• Increased range of motion in her shoulders
• Regrowth of hair on her legs.
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Date of download: 12/15/2015 Copyright © 2015 American Medical Association. All rights reserved.
From: Extracorporeal Photochemotherapy for Generalized Deep Morphea
Arch Dermatol. 2009;145(2):127-130. doi:10.1001/archdermatol.2008.547
• Improvements in range of motion.
• By 4 months, treatments were tapered to every three weeks then every 4 weeks 3 months later.
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PatientOlder woman.History of widespread sclerotic plaques and scarsPretreated with light therapy and cyclosporine, MTX, steroids.High degree of pain and disability.
Picture: October 2004
• Treatment• 2003
• -start treatment with mycophenolate mofetil.
• 2004• Start ECP• start 2 days q2 weeks• Tapering every 6 cycles.• After 10 weeks, ulcers
healed.
• Picture: April 2005
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Largest, modern biomarker study to date
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Study design
• 16 patients• 14 female; 2 male• Average age 46.5 years• Mean duration of disease is 3.9 years.
• Treatments• 2 ECP treatments every 6 weeks.
• Assessments• Ultrasound; modified RSS
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Efficacy
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Efficacy
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What is the mechanism?
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Increased levels of Tregs
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Increased Treg function
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Increased Treg cytokines
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What is the mechanism?
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Decreased levels of Th17 cells and cytokines
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Decreased levels of harmful, fibrosis-inducing cytokines
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Single-blind trial• Single-blind comparison of ECP vs.
D-penicillamine• 79 patients
• 2 consecutive days monthly• Results
• 21 of 31 on ECP responded (68%)• 8 of 25 with D-penicillamine.
(32%)
• Improvements in skin severity score, percent skin involvement, and mean oral aperture.
• Significant improvement with ECP at 6 months.
• No improvement at 6 months with D-penicillamine.
D-Penicil-lamine
ECP0
20406080
Response
Respo...
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Larger studies• Double-blind placebo
controlled study
• 64 patients• 16 institutional sites.• Recent onset of disease.• Receive photopheresis or
sham.• Excluded patients with renal,
pulmonary disease.
• ECP 2 days every 4 weeks for 12 months
• Improvements in skin score at 6 months and 12 months.
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Improvements in Skin • Results
• Improvements in skin score
• Statistically significant improvement in skin scores as compared with baseline at 6 and 12 months.
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Improvement in Joints• Results
• Improvements in joints• No statistically
significant differences in joints with contractures in the placebo group.
• Significant decreased number of affected joints in patients treated with ECP.
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State of the field • Photopheresis is a safe immunotherapy effective for
many immune based diseases including lung/heart transplant rejection and treatment of sclerodermoid GVHD.
• ECP appears to increase Tregs• Case reports and small studies support its use in
scleroderma.• Who is most likely to benefit?
• High skin involvement.• Early stage disease (<2 years)• ? Combination treatment
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Future Directions
• FDA and many insurance companies believes the data is not sufficient.
• Need for more studies.• Need biomarkers
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ECP at Northwestern
• Work closely with referring doctors and patients
• Default protocol• 2x/week for 6 weeks and then taper.• Tapering occurs with clinical responses.• Reassess after 3 months
• Assess for changes in disease• Assess for changes in doses of systemic
immunosuppression.
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ECP unit at Northwestern
• Patient oriented:• May adjust schedules to facilitate
• Medical staff at visits• Address clinical issues• Reduce the rate of cancellations.• Decrease time from referral to visits.
• Reduce blood draws • We will work with our referring providers to minimize phlebotomy
for patients.• Access
• Only observed adverse effect is line sepsis• We will try to work with you in the best interests of the patient to ensure
intravenous access.• We would like to minimize ports or catheters.
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ECP Unit at Northwestern
• Data driven• Drs. Galvin, Zhou, and myself• In conjunction with the pediatrics unit• Will try to collect data that will be useful for
future studies.
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ECP team• Rube Walker
• Administrators• Nursing Staff• Physicians