NANOSNET

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Lulu,

Was there leakage on the fluorescein angiogram to indicate active disease?  If so, she would need to be on more immunotherapy.  What dose of IVIG is she on?  You could potentially increase the dose or frequency and that may be the easiest/safest way to control the disease.  One of the most effective treatments is cyclophosphamide, but we usually reserve that for severe Susac syndrome, often at time of diagnosis.

Best,

John

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

The FA does look like active disease rather than nonspecific leakage from prior damage based on how focal it looks. I agree with Robert that I would increase the IVIG to 2g/kg every month or increase frequency to 1g/kg every 2 weeks.  Increasing the prednisone to 10mg would also be an option in addition to the IVIG increase.  I agree with avoiding cyclophosphamide if that is the only disease activity.

Best,

John

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

The FA does look like active disease rather than nonspecific leakage from prior damage based on how focal it looks. I agree with Robert that I would increase the IVIG to 2g/kg every month or increase frequency to 1g/kg every 2 weeks.  Increasing the prednisone to 10mg would also be an option in addition to the IVIG increase.  I agree with avoiding cyclophosphamide if that is the only disease activity.

Best,

John

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

The FA does look like active disease rather than nonspecific leakage from prior damage based on how focal it looks. I agree with Robert that I would increase the IVIG to 2g/kg every month or increase frequency to 1g/kg every 2 weeks.  Increasing the prednisone to 10mg would also be an option in addition to the IVIG increase.  I agree with avoiding cyclophosphamide if that is the only disease activity.

Best,

John

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

I would consider this uncontrolled Susac's. Susac's is not easy to control, and I would not consider your regimen "maximal medical therapy". Several of my patients have required cyclophosphamide to come under control.

Best,

Bart

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214

I would consider this uncontrolled Susac's. Susac's is not easy to control, and I would not consider your regimen "maximal medical therapy". Several of my patients have required cyclophosphamide to come under control.

Best,

Bart

Dear all,

I have a 34 year old woman with Susac syndrome, who has had multiple BRAOs with significant field loss. She is currently on prednisone 8mg, mycophenolate, rituximab and monthly IVIG. All was quiet in December, and her most recent MRI in May showed no new lesions. She remains asymptomatic, but at her most recent visit with me last week, there was yet another new BRAO.

Does this mean that the disease is still active despite being on maximal medical therapy?

Do I ask her to go back up on the prednisone?

Or do I just accept that she will continue to have recurrent BRAOs every year, which will slowly chip away at her remaining field?

I have not had many Susac patients, and certainly none as treatment resistant as this patient. Appreciate any advice!

Thanks,

Lulu

Lulu Bursztyn

Comprehensive and neuro-ophthalmology

Western University, London, Ontario

lulu.bursztyn@sjhc.london.on.ca

519-646-6214