Systemic Sclerosis (SSc)

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Statistically significant improvement in skin score after 6 months1*

For patients with early-onset, diffuse systemic sclerosis1
THERAKOS® Photopheresis harnesses the patient’s immune system to help treat systemic sclerosis2

*No significant improvement in skin score of ECP compared to D-pen-treated patients was observed at 10 months.1
†A change in the skin score during treatment was considered to be clinically relevant if it differed by at least 15% from baseline.1

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Significant improvement compared to baseline in oral aperture after 6 months and both oral aperture and right/left-hand closure after 10 months1

*No significant improvement in oral aperture of D-pen-treated patients compared to baseline was observed.1

†No significant improvement in hand closure of ECP-treated patients compared to baseline was observed at 6 months. No significant improvement in hand closure of D-Pen-treated patients compared to baseline was observed.1

Rook 1992 Trial Design (N=56): A 10-month randomized, single-blind, controlled trial in patients with recent onset systemic sclerosis. Outcomes included skin score assessed by rating the thickness of the skin on a 0 to 3 scale in 15 areas of the body with possible skin severity scores ranging from 0 to 45 and joints assessed by standardized measurement of the change in oral aperture and right and left-hand closure. All patients had recent onset systemic sclerosis (<4 years duration) and ≥30% progression in site of cutaneous involvement. Patients were randomized 1:1 to receive ECP, 2 consecutive days, every 4 weeks (n=31) or D-penicillamine (n=25), 750 mg/day (the dose was increased by 250 mg every two months until a daily dose of 750 mg/day was reached). Patients were assessed monthly for a minimum duration of 6 months. Collagen production-reducing pharmacologic agents like steroids, colchicine, potassium aminobenzoate, and griseofulvin were not permitted during the study. For treatment of Raynaud’s phenomenon, only stable doses of calcium channel blockers were permitted.1

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Statistically significant skin improvement from baseline at 6 and 12 months3

*Comparison of skin scores between the two study arms did not achieve statistical significance because of the small sample size of the study arms.3

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Statistically significant improvement from baseline in a greater number of joints and had fewer new joints become involved after 6 and 12 months3

Knobler 2006 Trial Design (N=57): A 12-month randomized, double-blind, placebo-controlled trial in patients with diffuse systemic sclerosis. The primary endpoint was decrease in skin involvement evaluated by skin score in 22 regions with the modified scleroderma skin scoring method recommended by Kahaleh et al, Clin Exp Rheumatol 1986;4:367-369. The secondary endpoint was change in joint involvement as measured with a goniometer. All patients had diffuse systemic sclerosis and were <2 years from disease onset. Patients were randomized 1:1 to receive Photopheresis, 2 consecutive days, every 4 weeks (n=27) or Sham Photopheresis, 2 consecutive days, every 4 weeks (n=30). Patients were assessed monthly for 12 months. Collagen production-reducing pharmacologic agents, including corticosteroids, colchicine, griseofulvin, and D-pen were not permitted.3

Well-tolerated treatment modality that can help manage systemic sclerosis3

An established tolerability profile3

  • No serious adverse events and no significant difference in overall adverse events between study arms were reported

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References: 1. Rook AH, et al. Arch Dermatol. 1992;128:337-346. 2. Hart JW, et al. Ther Adv Hematol. 2013;4:320-334. 3. Knobler R, et al. J Am Acad Dermatol. 2006;54:793-799

Important Safety Information for THERAKOS® CELLEX® Photopheresis Procedure

INDICATION

The THERAKOS® CELLEX® Photopheresis System is indicated for use in the ultraviolet-A (UVA) irradiation, in the presence of the photoactive drug 8-methoxypsoralen (8-MOP), of extracorporeally circulating leukocyte-enriched blood, in the palliative treatment of the skin manifestations of cutaneous T-cell lymphoma (CTCL) and systemic sclerosis (SSc).

CONTRAINDICATIONS

Certain underlying medical conditions contraindicate THERAKOS CELLEX Photopheresis, including:

  • Patients who cannot tolerate extracorporeal volume loss during the leukocyte enrichment phase

  • Patients exhibiting idiosyncratic or hypersensitivity reactions to 8-methoxypsoralen/psoralen compounds

  • Patients with coagulation disorders or who have had previous splenectomy

WARNINGS & PRECAUTIONS

THERAKOS CELLEX Photopheresis treatments should always be performed in locations where standard medical emergency equipment is available. Volume replacement fluids and/or volume expanders should be readily available throughout the procedure.

  • MR-Unsafe: Do not expose the device to a magnetic resonance (MR) environment. The device may present a risk of projective injury, and thermal injury and burns may occur. The device may generate artifacts in the MR image, or may not function properly.

  • Thromboembolic Events: Thromboembolic events, including pulmonary embolism and deep vein thrombosis, have been reported in the treatment of Graft versus Host Disease (GvHD), an indication not approved in Canada. Special attention to adequate anticoagulation is advised when treating patients with GvHD.

  • Concomitant Therapy: When prescribing and administering THERAKOS CELLEX Photopheresis for patients receiving concomitant therapy, exercise caution when changing treatment schedules to avoid increased disease activity that may be caused by abrupt withdrawal of previous therapy.

ADVERSE REACTIONS

Hypotension may occur during any treatment involving extracorporeal circulation. Monitor the patient closely during the entire treatment.

Transient pyretic reactions, 37.7-38.9 °C (100-102 °F), have been observed in some patients within 6-8 hours of reinfusion of the photoactivated leukocyte-enriched blood. A temporary increase in erythroderma may accompany the pyretic reaction.

Treatment frequency exceeding labeling recommendations may result in anemia.

Venous access carries a small risk of infection and pain.

Important Safety Information for Methoxsalen Sterile Solution Used in Conjunction with THERAKOS® CELLEX® Photopheresis System

CONTRAINDICATIONS

Methoxsalen Sterile Solution is contraindicated in:

  • Patients exhibiting idiosyncratic reactions to psoralen compounds

  • Patients with aphakia

  • Patients possessing a specific history of a light-sensitive disease state

SERIOUS WARNINGS & PRECAUTIONS

  • Concomitant Therapy: Exercise care in treating patients who are receiving concomitant therapy (either topically or systemically) with known photosensitizing agents.

  • Carcinogenicity: Oral administration of methoxsalen followed by cutaneous UVA exposure (PUVA therapy) is carcinogenic. Patients exhibiting multiple basal cell carcinomas or having a history of basal cell carcinoma should be diligently observed and treated.

  • Teratogenicity: Methoxsalen may cause fetal harm when given to a pregnant woman. Women undergoing photopheresis should be advised to avoid becoming pregnant.

  • Cataractogenicity: Patients should be told emphatically to wear UVA absorbing, wrap-around sunglasses for twenty-four (24) hours after methoxsalen treatment, any time they are exposed to direct or indirect sunlight and whether they are outdoors or exposed through a window.

  • Safety in children has not been established.

FOR MORE INFORMATION

See product monograph for methoxsalen sterile solution (if used in conjunction with the THERAKOS CELLEX Photopheresis System) and the Operator’s Manual for the CELLEX system at health-products.canada.ca/dpd-bdpp or by calling 1-833-223-4ECP (1-833-223-4327).

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Important Safety Information for THERAKOS® CELLEX® Photopheresis Procedure
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INDICATION

The THERAKOS® CELLEX® Photopheresis System is indicated for use in the ultraviolet-A (UVA) irradiation, in the presence of the photoactive drug 8-methoxypsoralen (8-MOP), of extracorporeally circulating leukocyte-enriched blood, in the palliative treatment of the skin manifestations of cutaneous T-cell lymphoma (CTCL) and systemic sclerosis (SSc).