Laquinimod Polman, C. et al. Neurology 2005;64:987-991

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Laquinimod Polman, C. et al. Neurology 2005;64:987-991 Multicenter, double-blind, randomized trial, patients with RR MS received 0.1 mg or 0.3 mg laquinimod or placebo as three daily tablets for 24 weeks Gadolinium-enhanced brain MRI scans were performed at screening, every eighth week during treatment, and 8 weeks after end of treatment The primary efficacy variable was the cumulative number of active lesions over 24 weeks

Figure 2. Cumulative number of active lesions (mean {+/-} SE) by visit (primary endpoint) Polman, C. et al. Neurology 2005;64:987-991 No statistically significant differences in the primary endpoint of active scans but there was a strong trend (37% active in placebo, 31% in 0.1 and 21% in 0.3 group).

Table 3 Statistical evaluation of the reduction in the cumulative number of active lesions at week 24 Polman, C. et al. Neurology 2005;64:987-991

Phase III ALLEGRO Trial 2 year randomized, double blind, placebo controlled with 1106 RRMS participants 0.6 mg qday Primary outcome - # confirmed relapses showed a 26% reduction (p=0.0024) 36% decrease in risk of progression by EDSS (p=0.0122) 33% reduction in brain atrophy (p=0.0001)

Phase III BRAVO Trial Comparing 0.6 mg qday with placebo Did not reach primary endpoint of reducing ARR (p=0.075)! However, the laquinimod and placebo groups had dissimilar baseline MRI measures after reanalysis adjusted for baseline MRI there was 21.3% reduction in ARR, 33.5% reduction in risk of progression in EDSS and 27.5% reduction of brain volume loss (all p<0.05)

Safety The most common side effects have been occasional liver enzyme elevations which are reversible

Summary Laquinimod has modest benefit in RRMS Nevertheless, it is well tolerated

Teriflunomide Oral immunomodulator with anti-inflammatory activity Inhibits pyrimidine synthesis in T cells and other rapidly dividing cells Results in reduced T cell proliferation, IFN-gamma, IL- 2, IgG1, and cytostatic action on B cells Ongoing trials TOPIC (CIS trial) and TERACLES (teriflunomide added to interferon or placebo)

Teriflunomide O Connor et al. NEUROLOGY 2006;66:894-900 Phase II, randomized, double blind, placebo controlled trial in RR (157) and SP with relapses (22) Primary end point # combined unique active MRI lesions - Treatment with either terflunomide 7 or 14 mg/day resulted in the significant suppression of 61.1% or 61.3%, respectively (p < 0.03 or p < 0.01) Also fewer enhancing and new T2 lesions, trend in fewer relapses and less disability Efficacy of teriflunomide on the primary outcome measure in the phase II study.

TEMSO Trial involving 1088 patients with multiple sclerosis, 18 to 55 years of age, with a score of 0 to 5.5 on the Expanded Disability Status Scale Randomly assigned (in a 1:1:1 ratio) to placebo, 7 mg of teriflunomide, or 14 mg of teriflunomide once daily for 108 weeks The primary end point was the annualized relapse rate, and the key secondary end point was confirmed progression of disability for at least 12 weeks N Engl J Med. 2011 Oct 6;365(14):1293-303. Randomized trial of oral teriflunomide for relapsing multiple sclerosis. O'Connor P, Wolinsky JS, Confavreux C, Comi G, Kappos L, Olsson TP, Benzerdjeb H, Truffinet P, Wang L, Miller A, Freedman MS; TEMSO Trial Group.

TEMSO Teriflunomide reduced the annualized relapse rate (0.54 for placebo vs. 0.37 for teriflunomide at either 7 or 14 mg), with relative risk reductions of 31.2% and 31.5%, respectively (P<0.001 for both comparisons with placebo) The proportion of patients with confirmed disability progression was 27.3% with placebo, 21.7% with teriflunomide at 7 mg (P=0.08), and 20.2% with teriflunomide at 14 mg (P=0.03)

TENERE Phase II trial comparison of teriflunomide with Rebif Two-year, randomized, rater-blinded study that included 324 people with RR MS No statistical superiority was observed between the Rebif and teriflunomide arms (7mg and 14mg) on risk of treatment failure (confirmed relapse), the primary composite endpoint of the study Just in Secondary endpoints (MRI activity) were statistically significantly different (85% reduction in Gad + lesions at 7 mg dose; P=0.0005) Freedman et al. 2012. Neurology 78: 1877-1885

TOWER Just Out Phase III randomized study of 1169 RR MS patients assigned to either 7 mg or 14 mg teriflunomide or placebo Teriflunomide 14 mg reduced relapses by 36.3% versus placebo and 7 mg by 22.3%. Disability reduced by 31.5% in the 14 mg group.

Don t you get tired of DUMB acronyms? D Duke U University M Marching B - Band

Teriflunomide Adverse Events Elevated liver enzymes (rarely serious -> withdrawal) Low WBC s Alopecia Paresthesias Possibly UTI s, nasopharyngitis and diarrhea Other rare complications include neutropenia, rhabdomyalysis, and possibly trigeminal neuralgia Possible teratogenic effect Pregnancy should be avoided as with other similar agents

Summary of Teriflunomide Reasonable efficacy in RR MS Reasonable side effect profile Pending FDA I don t think they have officially filed yet

BG-12 (Dimethyl Fumarate) BG-12 is a fumaric acid ester with immunomodulatory properties Demonstrated benefits in animal models of EAE Fumaric acid esters may decrease leukocyte passage through the blood brain barrier and exert neuroprotective properties by the activation of antioxidative pathways (Nrf-2 cellular pathway)

DEFINE Phase III, randomized, double blind, placebo controlled, dose comparison in 1234 patients with RR MS 240 BID or TID vs placebo Both BG-12 doses were associated with a significant decrease in the proportion of patients who relapsed at 2 years compared with placebo (P<0.0001)

DEFINE Both BG-12 doses were significantly superior to placebo in reducing ARR, the number of new or newly enlarging T2 hyperintense lesions, and the number of new gadolinium-enhancing lesions. The reduction in 12-week disability progression was 38 and 34% for the twice and three-times daily doses, respectively (P<0.05 for both). Gold R, Kappos L, Bar-Or D, et al. Clinical efficacy of BG-12, an oral therapy, in relapsing-remitting multiple sclerosis: data from the phase 3 DEFINE trial. Program and abstracts of the 5th Joint Triennial Congress of the European and Americas Committees for Treatment and Research in Multiple Sclerosis (ECTRIMS/ACTRIMS); 19 22 October 2011; Amsterdam, The Netherlands.

CONFIRM Preliminary Results Phase III trial in RR MS BG-12 met the study s primary endpoint, significantly reducing the annualized relapse rate by 44% for the twice-daily (BID) dose and by 51% for the thrice-daily (TID) dose vs placebo at 2 years Treatment also reduced T1 lesions, T2 lesions, and the risk for relapse, as well as the 12-week confirmed disability progression, although this latter finding was not statistically significant

Safety of BG-12 DEFINE results indicated that BG-12 had a safety profile comparable to that for placebo! Results from a phase 2b study of BG-12 (120 or 240mg three times per day) in 257 patients with RRMS indicated that adverse events occurring more often with BG-12 vs. placebo were abdominal pain, flushing, and hot flush

Summary for BG-12 Good efficacy Good adverse effects profile Not yet filed with FDA

Overall Summary of Oral Agents for MS Fingolimod is FDA approved for relapsing MS and is probably a first line Rx There are no plans to resubmit cladribine for FDA approval in MS Teriflunomide, laquinimod and BG 12 are oral agents that likely will be submitted to the FDA soon for consideration in relapsing MS Rx

Summary A number of treatments are on the horizon for MS Where they stand in terms of preference with existing treatments is unclear at this time Only with time, further study and experience will we be able to determine a rational approach to the use of current and newer treatments The complexity of MS treatment may necessitate referral to specialists

Emory MS Center