CAPS or Primary APS with Alveolar Hemorrhage

[kiminabmw]

Anyone have CAPS or Primary APS with Alveolar Hemorrhage? Looking for treatment options .... we have done Plasmapheresis, Rituxan, Steroids, Plaquenil, Azathioprine and the Rheumy is now talking of doing IVIG if tomorrows blood work doesn't show an improvement in platelets. Anyone have any experience with any of this??

[HollyHeski Administrator]

Sorry no, sounds like you are trying everything and have a good Rheumy that knows APS?

Wishing you a speedy recovery.

[kiminabmw]

Hello, I am a relative of the patient. My nephew (44 years old) was diagnosed with APS in 2015 after having a MRI show he had 2 small strokes. Early May 2019, he had extreme shortness of breath and presented to the Emergency Room (In the US, state of Illinois) with 72 oxygen level. They diagnosed CAPS and also Diffuse Alveolar Hemorrhage and Respiratory Failure. He spent 9 days in Intensive Care, 6 of those days in a medically induced coma and on life support with a Ventilator breathing for him. Very high doses of steroids, antibiotics and Plaquenil, Plasmapheresis, 5 days in a row and then Rituxan infusions once a week for 4 weeks saved his life. Unfortunately, after a month in the hospital and also a Rehab hospital his lungs hemorrhaged again. After the second hemorrhage his Rheumy added in another immunosuppressant, Azathioprine. His platelets have dropped again. I am taking him for his weekly blood work this morning, so we will see if his Plasmapheresis from a week ago has helped his platelets, hemoglobin and hematocrit recover.

[MaryF Administrator]

Hi, Sorry your nephew is having such a difficult time, thank goodness you have a good consultant in the picture helping him. We do have a few people on here who have had CAPS, I am sure they will reply at some stage. In the meantime sending you best wishes for his speedy recovery, I enclose our charity website for you, this is expanding all the time, there is a brief bit on CAPS. Do check in with us for support when you need it, and your nephew is welcome to join us also when he is up to it: ghic.world/

MaryF

[Jmiller623]

He needs IVIG. Do it. I’ve only ever seen one case of CAPS as an internist. It is very rare. They should plasmapherese daily if doable. Sometimes they run out of plasma to replace. It can make bleeding worse. I am so sorry he is going through this. Any mention of using anakinra?

Has DIC, TTP and all the platelet eating problems been ruled out? Again I am so sorry he is going through this. They should also make sure all infections are ruled out. I mean all infections including viral. Is he on antibiotics?

[kiminabmw in reply to Jmiller623]

Hi and thank you!! Plasmapheresis when he was in a medically induced coma with a Vent breathing for him for 6 days. It was done once a day, for 5 days in a row. Then he did weekly Rituxan infusions, once per week for 4 weeks. All his blood work looked great, except he remained Anemic. He's been on 80mgs of Prednisone for 6 weeks, and they are just starting to taper him down, slowly. He is also on Plaquenil, and they added in Azathioprine 2 weeks ago. Yes, he is on Bactrim daily and has been for almost 2 months. He was on 2 other antibiotics the first 2 weeks after he presented in the ER with Respiratory failure from his lungs hemorrhaging. I will look up Anakinra, as well as DIC, TTP and any other platelet eating problems I can find, print them out and take them to his Doctor next week. They have not ruled any eating issues out. Thank you so much.

[Jmiller623 in reply to kiminabmw]

If he’s off the vent, they are doing the right thing. Trust in your team. If he’s getting better then pray it continues. That’s tough to come back from. I’m sure they’ve ruled out all the other things listed above. Hang in there!

One last thing, check the Bactrim. I’ve seen that destroy counts before and cause fevers as a drug reaction in lupus.

[KellyInTexas Administrator]

Hello, kiminabmw.

What a wonderful aunt you are!

This must be devastating for your family.

The only thing I will add about IVIG and aps comes from Dr Jill Schofield, who is an APS Specialist Rheumatoligist in Denver Colorado. ( Center for Multi system disease on East Lowery Street.) she is my APS specialty Rheumatoligist.

IVIG can cause micro clots- this does not mean it should not be used. She uses it also, but she has a few tricks to help minimize adverse effects. APS patients can be a little more prone to react.

Extra fluids starting day before ( I realize he’s on IV fluids now) and benedryl - to help stabilize mast cells. Also- warm all IV solutions to close to body temperature in warm water before administering. Also- she administers a bit more slowly than the average session to avoid reactions. There may be an additional mast cell stabilizer she would suggest.

She might take a call from your nephews lead physician. Just say that it’s your understanding from a patient of hers that she has a specialized APS protocol. ( non bacterial meningitis is a bit of a risk so she likes to minimize this.)

He is already weak.

I’m on Rituximab infusions, and my Rheumatoligist in Texas uses this protocol for me. He did not slow it down, I had a minor- very minor itchy reaction- and he has learned I must have it very slow. 8 hours instead of 4.

I also add zofran to it. It’s standard with the Rituximab to have solumedrol and Tylenol and benedryl given about 30 min before the IV infusion.

[kiminabmw in reply to KellyInTexas]

Thank you so much!! How many Rituxan infusions have you had? How far apart are they spaced? My nephew had 4 infusions, spaced 1 week apart.

[KellyInTexas Administrator in reply to kiminabmw]

I am not being administered Rituximab for full on CAPS- I’m being administers Rituximab for progressive, Refractory APS.

I’ve only just started my regiment, and if successful it will be life long, or until a better solution is discovered by scientists,

May 6 th was treatment # 1

May 22nd was treatment # 2

( both high dose- 1000 ml)

Then the plan was to wait 6 months- but I think that’s been changed now to maybe 3-4 months. I did clot in mid June, but my Rheumatoligist said it may have been a chemo like response to cell death. He gave me a solumedrol 120 IM injection. I’ve done very well.

If I continue to clot he will change treatment to IVIG instead.

[kiminabmw in reply to KellyInTexas]

Thank you, the more I know the better is the way I figure it! Be well.

[InSpain]

Hello.

I am a CAPS survivor. I had multi organ failure. Unfortunately CAPS is rare and I was told what may work for another patient may not work for me.Therefore, I can only share the treatment I had with you and hope that it may be of some help to your nephew.

I was treated firstly with high dose antibiotics, prednisolone which was given intravenously and double-filtration plasmapheresis for five days, whilst being in an induced coma too. I then continued with high dose Prednisolone for six months which was then tapered down over the following six months. When I came out of intensive care I was then given cyclophosphamide infusions for six months. I won't lie to you it is a tough treatment but I benefitted sufficiently to help my recovery.

I now continue with immunosuppressive treatment and, of course, the Warfarin alongside all of the other medications that I have to take for my heart, lungs, kidneys, liver etc.

I sincerely hope that a effective treatment plan may be found and implemented to help in your nephew's recovery.

Best wishes from here InSpain.

[kiminabmw in reply to InSpain]

Thank you so much InSpain. I am so sorry for all you have been through, but so happy you are doing well and grateful for your willingness to share your experience. I honestly wonder if they are trying to taper the steroid too quickly. I do understand that 80mgs daily is a lot, and it has caused my nephew to have high blood sugar and is now on insulin, but I believe that might just be temporary until they can get him off the steroid, or lower it significantly. They are in the process of lowering it 10mgs weekly to get him to 40mgs daily until we see his Rheumotologist on July 31st. His Rheumy has suggested cyclophosphamide as an option, but she said she does not want to use it if it can be avoided, like you said, it is a very tough treatment. Thank you again so much!!

[Lure2 in reply to InSpain]

We must be grateful you have survived here!

Best wishes to you from Stockholm

[InSpain]

10mg weekly wow! That is really really fast. I was taking 100mg orally for 6 months. I then tapered it down by 10 mg per month until I reached 40mg. I now continue to take the 40mg dose daily and have done so for quite a few years now. I too had steroid induced diabetes. I used insulin for about a year. I now am able to control it with diet and two tablets in 24 hours - metaformina (sorry not too sure what it's called in English).

Incidentally, I have hemarthrosis as a consequence of bleeding into the joints for quite a few years before the CAPS. Nothing cured it until I had the infusions. Might be worth weighing up the pros and cons of going down that road?

Message me if you want any help or information. 😊

[kiminabmw in reply to InSpain]

Thank you again, and I will message you as we navigate ahead.