Relapse Risk After Stopping Immunosuppressive Therapy in Patients With NMOSD

 

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  • The authors of this study examined the risk of relapse after discontinuing disease-modifying therapy (DMT) for patients with neuromyelitis optica spectrum disorder (NMOSD). They examined 17 patients with a median relapse-free interval of 62 months. There were 14 patients (82%) who had a relapse at a median interval of 6 months. Of these patients, 3 had a severe relapse, all of whom had a history of severe relapse; 2 of the 3 patients with severe relapse had poor recovery.

  • These data suggest that relapses are common in patients with NMOSD who stop DMT. Patients should continue DMT until additional studies determine which patients, if any, can safely discontinue it.
–  Kyle Binder, MD

 Neurology

The research regarding the diagnosis and treatment of NMOSD has seen a huge leap during last 2 decades. However, many unsolved mysteries about this disease continue to exist, one of them being the consensus on the duration of immunosuppressive therapy (IST). Although many experts believe that all patients with NMOSD should be treated with immunosuppressive therapy for 5 years after the last attack, there are some who favor lifelong treatment considering the risk of recurrence after discontinuation of therapy.1,2 This study by Kim et al has further reinforced this concept. In this retrospective study, the author has shown that 81% of cases of NMOSD, in whom IST was discontinued after a median relapse-free period of 62 months, relapsed at a median interval of 6 months. It was also observed that 3/14 cases who had severe relapse had severe attacks in the past as well.

Certain prognostic factors have been utilized in the past to help decide about discontinuation of IST, including the number of relapses, severity of relapse at onset, AQP4 antibody seropositivity, and older age of disease onset.3 From the current study, however, no factors that could predict the long-term remission after stopping IST could be determined. Even the patients who had experienced a single attack before IST relapsed when IST was stopped. This again highlights the unpredictable course of the disease. Rituximab, among the immunosuppressive agents, has been reported to result in prolonged periods of remission in NMOSD even after discontinuation.4 Similar results were demonstrated in this study as well.

Larger studies on long-term follow-up of NMOSD patients after discontinuation of IST are lacking. This study emphasizes the fact that any duration of IST may never be sufficient enough to allow discontinuation of the therapy in NMOSD. Decisions will always have to be individualized, considering the adverse effects of the drug or the patient’s choice to discontinue after having been educated regarding the disease course and the chances of disease recurrence after discontinuation of therapy.

References

  1. Montcuquet A, Collongues N, Papeix C, et al. Effectiveness of mycophenolate mofetil as first-line therapy in AQP4-IgG, MOG-IgG, and seronegative neuromyelitis optica spectrum disorders. Mult Scler J. 2017;23(10):1377-1384. https://journals.sagepub.com/doi/10.1177/1352458516678474
  2. Carroll WM, Fujihara K. Neuromyelitis optica. Curr Treat Options Neurol. 2010;12(3):244-255. https://link.springer.com/article/10.1007%2Fs11940-010-0071-z
  3. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53(5):1107-1114. https://n.neurology.org/content/53/5/1107.long
  4. Weinfurtner K, Graves J, Ness J, et al. Prolonged remission in neuromyelitis optica following cessation of rituximab treatment. J Child Neurol. 2015;30(10):1366-1370. https://journals.sagepub.com/doi/10.1177/0883073814553974

OBJECTIVE

To evaluate the outcomes of immunosupressive therapy (IST) discontinuation in patients with neuromyelitis optica spectrum disorder (NMOSD) after a sustained remission period.

METHODS

We retrospectively reviewed the medical records of 17 patients with antiaquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period of ≥3 years.

RESULTS

IST was discontinued at a median age of 40 years (interquartile range [IQR], 32-51) after a median relapse-free period of 62 months (IQR, 52-73). Among the 17 enrolled patients, 14 (82%) relapsed at a median interval of 6 months (IQR, 4-34) after IST discontinuation, 3 (18%) of whom experienced severe attacks; notably, all 3 of these patients had a history of severe attack before IST. These 3 patients received steroids, followed by plasma exchange for acute treatment, but 2 exhibited poor recovery and significant disability worsening at 6 months after relapse.

CONCLUSIONS

IST discontinuation may increase the risk of relapse in seropositive patients with NMOSD even after 5 years of remission. Given the potentially devastating consequence of a single attack of NMOSD, caution is advised with IST discontinuation, particularly in patients with severe attack before IST.

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