Demyelinating diseases like Multiple Sclerosis and cases of long
segment myelitis, neuromuscular disease like Myasthenia Gravis
and Autoimmune Encephalitis have an autoimmune basis and
occur in a relapsing remitting course. Achieving and maintaining
remission in these patients is quite challenging. Steroids are used
for maintaining remission, but its metabolic side effects ultimately
are deleterious to the patient. Immunomodulators for remission
and Gamma globulin for acute exacerbations are used but are an
expensive mode of treatment. All patients may not be able to afford
them.
Rituximab is a monoclonal antibody which is used as an
immunomodulator in many autoimmune disorders. It selectively
depletes B cells having CD 20 on their cell surface. Antigen specific
helper T(CD4) cells activate B cells which in turn lead to production
of plasma cells generating Ig antibodies found in autoimmune
disorders. Hence destruction of the activated B cells plays a vital
role in effectiveness of Rituximab. It has three specific modes of
action- antibody mediated cytotoxicity, inducing targeted apoptosis
and inhibiting B cell dependant granulocyte macrophage colony
stimulating factor.
In this study we used Rituximab in patients who could not
afford the conventional line of therapy. Two patients of Myasthenia,
one patient each of Long segment myelitis, Multiple sclerosis,
Autoimmune encephalitis were treated with Rituximab infusions
once a week for 4 weeks. The first patient came with unilateral
ptosis and diplopia with diurnal variation. He was diagnosed as
ocular myasthenia and received Rituximab. There was significant
improvement in his symptoms. Second patient had generalized
myasthenia and came with a history having underwent thymectomy
and being positive for AchR antibody. He was on maintenance
therapy with steroids and Azathioprine. But would get symptomatic
frequently and had several exacerbations in the past six months. He
presented with bulbar symptoms. After receiving 4 cycles of 500
mg of Rituximab there was improvement in neck holding, single
breath count, forward arm abduction time and muscle power and
the requirement for his previous maintenance therapy decreased
significantly.
The long segment myelitis patient had presented with onset of
symptoms following an episode of febrile illness. On examination,
power was grade 3/5 in all limb muscles, DTRs were brisk, sensory
examination showed reduced pain and temperature sensations and
loss of joint position sense. After treatment there was improvement
in all the affected modalities and he became independent for
activities of daily living. Multiple sclerosis patient had developed
spasticity in both lower limbs, power of grade 4/5 in upper limbs
and grade 2-3/5 in both lower limbs, DTRs were brisk, right planter
was extensor. He was wheelchair bound. After treatment with
Rituximab there was significant improvement in his disability and
Expanded Disability Severity Score. He also became independent
for activities of daily living.
Autoimmune encephalitis patient presented low grade
intermittent fever for one week during which there was short term
memory loss with several episodes of disorientation to time and
place. He also developed focal onset seizures which occurred once
daily for two days following which it recurred for several times in
a day. Following treatment there was no seizure recurrence and
memory, cognition also gradually improved. All the patients have
received Rituximab and remained in remissions for prolonged
periods because of which the need for maintenance steroids
reduced significantly or became nil. Between the dosing schedules of
Rituximab, the need for maintenance with oral immunosuppressant
was eliminated. This shows that Rituximab can be used as a rescue
medication in newly diagnosed patients and those who have failed
other modalities of therapy and should it also be considered in
those patients who cannot afford the standard medical treatment
or are suffering from the complications of maintenance therapy.