New England Journal of Medicine FEBRUARY 04, 2010

A Placebo-Controlled Trial of Oral Cladribine for Relapsing Multiple Sclerosis (CLARITY)

Gavin Giovannoni, Giancarlo Comi, Stuart Cook, Kottil Rammohan, Peter Rieckmann, Per Soelberg Sørensen, Patrick Vermersch, et al.

Bottom Line

In patients with relapsing-remitting multiple sclerosis, oral cladribine at cumulative doses of 3.5 mg/kg or 5.25 mg/kg significantly reduced the annualized relapse rate, risk of disability progression, and MRI measures of disease activity over 96 weeks compared with placebo.

Key Findings

1. The annualized relapse rate (ARR) was 0.14 for the 3.5 mg/kg cladribine group and 0.15 for the 5.25 mg/kg group, compared to 0.33 for placebo, representing a significant relative reduction of 58% and 55% respectively (p<0.001 for both).
2. The risk of 3-month sustained disability progression was significantly lower in both the 3.5 mg/kg group (hazard ratio 0.67; 95% CI 0.48–0.93; p=0.02) and the 5.25 mg/kg group (hazard ratio 0.69; 95% CI 0.49–0.96; p=0.03) compared to placebo.
3. Patients receiving cladribine showed greater freedom from MRI disease activity (no T1 gadolinium-enhancing or active T2 lesions) compared to placebo (p<0.001 for both dosage groups).
4. The most common adverse event associated with cladribine treatment was lymphopenia, which was dose-dependent but generally manageable.

Study Design

Design
RCT
Double-Blind
Sample
1,326
Patients
Duration
96 wk
Median
Setting
Multicenter, International
Population Adult patients with relapsing-remitting multiple sclerosis (RRMS) as defined by the revised McDonald criteria.
Intervention Oral cladribine at a cumulative dose of 3.5 mg/kg or 5.25 mg/kg administered in short courses over 96 weeks.
Comparator Placebo administered according to the same schedule as the intervention.
Outcome Annualized relapse rate (ARR) at 96 weeks.

Study Limitations

The trial was 96 weeks in duration, which may not capture the full long-term safety profile or durability of effect.
The study focused on relapsing-remitting MS, limiting the direct generalizability of these findings to primary progressive or secondary progressive forms of the disease.
Lymphopenia, a known potential side effect of cladribine, necessitates specific patient monitoring protocols which must be considered in clinical practice.

Clinical Significance

The CLARITY trial established oral cladribine as an effective, convenient disease-modifying therapy for relapsing-remitting MS, offering a unique 'short-course' dosing regimen that separates treatment delivery from continuous immunosuppression.

Historical Context

The CLARITY trial was a landmark study in the evolution of MS therapies, moving from daily injectable treatments toward intermittent oral administration, which challenged conventional paradigms of continuous maintenance therapy in neuroimmunology.

Guided Discussion

High-yield insights from every perspective

Med Student
Medical Student

By what biochemical mechanism does oral cladribine achieve its selective effect on lymphocytes, and why is this relevant to the treatment of Relapsing-Remitting Multiple Sclerosis (RRMS)?

Key Response

Cladribine is a deoxyadenosine analogue that acts as a prodrug. It is selectively activated by phosphorylation via deoxycytidine kinase (dCK), which is found in high concentrations in lymphocytes. Because lymphocytes have low levels of the neutralizing enzyme 5'-nucleotidase, the active triphosphate form accumulates, leading to DNA strand breaks and apoptosis. In RRMS, this reduces the pool of circulating T and B cells that drive neuroinflammation and demyelination.

Resident
Resident

In the context of the CLARITY trial, how should a clinician manage the monitoring of absolute lymphocyte counts (ALC) during the two-year treatment course of oral cladribine?

Key Response

The trial demonstrated that cladribine causes significant but transient lymphopenia. Guidelines derived from the study suggest monitoring ALC before starting, and at 2 and 6 months after the first dose in each treatment year. The second year's dose should only be administered if the ALC has recovered to at least 800 cells/mm³ (Grade 0 or 1 lymphopenia) to minimize the risk of opportunistic infections like Herpes Zoster.

Fellow
Fellow

How does the concept of 'Immune Reconstitution Inflammatory Therapy' (IRT) apply to cladribine's dosing schedule in CLARITY, and how does this contrast with 'maintenance' therapies like fingolimod or natalizumab?

Key Response

Cladribine is an IRT, meaning it is administered in brief pulses (short courses in years 1 and 2) to deplete the immune system, which then reconstitutes with a potentially less pathogenic profile. This allows for long periods of drug-free remission. In contrast, maintenance therapies require continuous presence of the drug to sequester or modulate immune cells; cessation of these agents often leads to a rapid return of disease activity or 'rebound' effect.

Attending
Attending

The CLARITY trial showed a numerical imbalance in malignancy cases (10 in the cladribine arms vs. 0 in the placebo arm). How should this influence the 'flipping the pyramid' strategy of using high-efficacy therapies early in the disease course?

Key Response

While the malignancy signal in CLARITY initially caused concern, meta-analyses of multiple trials and long-term extension data suggest the placebo rate in CLARITY was unexpectedly low (0.0), and the cladribine malignancy rate was consistent with other MS populations. Attendings must teach that while early high-efficacy therapy (like cladribine) can significantly delay disability progression, it requires a personalized risk-benefit discussion regarding long-term safety versus the risk of permanent neurological deficit from undertreated disease.

Scholarly Review

Critical appraisal through the lens of expert reviewers and guideline development

PhD
PhD

Critique the use of 'Annualized Relapse Rate' (ARR) as the primary endpoint in the CLARITY trial versus the potential use of 'No Evidence of Disease Activity' (NEDA-3) as a composite metric.

Key Response

ARR is a classic regulatory endpoint but can be sensitive to trial duration and patient recall. NEDA-3 (no relapses, no disability progression, and no new MRI lesions) offers a more holistic view of 'clinical remission.' While CLARITY established efficacy via ARR, the PhD perspective looks for more sensitive measures of neuroaxonal preservation (like brain atrophy or neurofilament light chain) to determine if the immune reset actually halts the underlying degenerative process rather than just the inflammatory spikes.

Journal Editor
Journal Editor

Given the 96-week duration of the CLARITY trial, what are the primary threats to the external validity of the safety data, particularly regarding the risk of opportunistic infections and delayed adverse events?

Key Response

As an editor, the concern is that 96 weeks is insufficient to capture the full safety profile of a drug that permanently alters the immune repertoire. Specifically, the risk of progressive multifocal leukoencephalopathy (PML) or secondary malignancies may not manifest within the trial's window. Rigorous peer review would require an emphasis on the necessity of long-term extension studies (e.g., CLARITY-EXT) and post-marketing surveillance to validate the initial safety findings.

Guideline Committee
Guideline Committee

Based on the CLARITY results, where does oral cladribine fit into current AAN or EAN guidelines regarding 'first-line' versus 'second-line' use for patients with active RRMS?

Key Response

The high efficacy demonstrated in CLARITY (approx. 58% reduction in ARR for the 3.5mg/kg dose) supports its use in patients with highly active disease. While the AAN guidelines generally allow for high-efficacy drugs to be used early, some European guidelines traditionally reserved cladribine for patients who failed other DMTs. However, because of its convenient dosing and lack of 'rebound,' guidelines have increasingly moved toward recommending it as a first-line option for patients with prognostic factors indicating aggressive disease.

Clinical Landscape

Noteworthy Related Trials

2006

AFFIRM Trial

n = 942 · NEJM

Tested

Natalizumab

Population

Patients with relapsing-remitting multiple sclerosis

Comparator

Placebo

Endpoint

Rate of clinical relapse at 1 year

Key result: Natalizumab monotherapy resulted in a significant reduction in the rate of clinical relapse and disability progression compared to placebo.
2007

BENEFIT Trial

n = 468 · Neurology

Tested

Interferon beta-1b

Population

Patients with a first clinical event suggestive of multiple sclerosis

Comparator

Placebo

Endpoint

Time to clinically definite multiple sclerosis

Key result: Early treatment with interferon beta-1b significantly delayed the progression to clinically definite multiple sclerosis compared to placebo.
2010

TRANSFORMS Trial

n = 1,292 · NEJM

Tested

Fingolimod 0.5mg or 1.25mg

Population

Patients with relapsing-remitting multiple sclerosis

Comparator

Interferon beta-1a (intramuscular)

Endpoint

Annualized relapse rate

Key result: Fingolimod was superior to interferon beta-1a in reducing the annualized relapse rate and showing less MRI disease activity.

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