Secondary or severe Raynaud’s, starting Sildenafil

[ShonaGreen]

Hi, I had Rheumatology appointment today originally requested for bad chilblains with brown discolouring on toes which are either freezing & purple or burning & red. 3 months later and still have chilblains (see pic).

Only ANA was positive (1:160 homogenous & cytoplasmic pattern), so didn’t know what to expect today. To be honest I felt a bit intimidated as the consultant was quite stern, didn’t feel like I could ask much and struggling to remember what he said now. But I think it was that although it seemed like primary Raynaud’s, due to late onset (age 40) and recent severity that it’s looking more like secondary Raynaud’s (but he could have said severe rather than secondary?) and though something was going on but not ticking boxes for a diagnosis. I asked about UCTD and they said it didn’t matter whether they said it was UCTD as would be treated in same way. 3 years ago during last visit with Rheumatology I had nailfold capillary test which was negative so I’m thinking it’s unlikely to be secondary Raynaud’s?

They prescribed Sildenafil and can try other medications like Losartan or Fluoxitine if that doesn’t work (already tried Nifedipine but didn’t help). Also had another blood test for more specific Scleroderma antibodies, with next appointment in 3 months.

Does this sound like a similar situation to other folks, any good experiences with Sildenafil? Thanks!

[OldTed60]

Hello. I think your appointment sounds like it went fairly well - although it’s hard to say as only you know how it went and you’re feeling about it.

My Raynaud’s was classed as secondary when it started at age 49 because I was already diagnosed with a couple of autoimmune diseases. My toes and fingers were terribly painful but didn’t look like yours at all - nothing like as bad. I didn’t tolerate Nifedipine and no ulcers, normal capillaries so was told it couldn’t be scleroderma despite a rare, high specific scleroderma antibody. Finally I traveled from Scotland to Royal Free to see the Prof there for diagnostic clarity. I was expecting him to say UCTD with Sjogren’s because of the normal nailfolds. He checked them again two years after last time in my hospital when they were normal - and found changes specific to early scleroderma. So he diagnosed me unequivocally on the spot and my rheumatologist was happy to confirm and treat me further. Things can change quite fast or slowly in rheumatology. Hang in there - 3 months is nothing and you can try Sildenafil while you wait. Good luck 😉

[ShonaGreen in reply to OldTed60]

Thanks so much OldTed60 it’s good to hear other peoples experiences and you replied to my earlier post too mentioning other specific antibody testing which had no idea about so appreciate you taking the time to help!

With the warmer weather coming and the Sildenafil I’m hoping my feet will improve soon 🙏

[OldTed60 in reply to ShonaGreen]

You’re welcome. I wonder if the rheumatologist had also tested you for Lupus and Sjogren’s because you only mention a scleroderma panel being run? I believe there’s something called Lupus chilblains - which are rather different to the usual ones apparently although I’m not sure how as I don’t have Lupus. I would think it’s unlikely to be primary when chilblains are so severe but I could be wrong. It sounds like you also have Erythromelalgia and I have this too. This could mask nailfold changes I would think because it works like an extreme vasodilator in itself.

[ShonaGreen in reply to OldTed60]

Hi OldTed60 think there’s been other tests for lupus and sjogrens and have been negative. I’ve been interested to read posts relating to Erythromelalgia as that could be a possibility too and good to know it can potentially affect nailfold changes, I’ve been learning a lot recently, thank you!

[OldTed60 in reply to ShonaGreen]

You’re welcome. I didn’t mean that scleroderma won’t show in nailfolds because of Erythromelalgia but I think, in my case, it slowed down the abnormal capillaries. They never told me my Raynaud’s was primary though despite entirely normal nailfolds. I was told that a normal capillaroscopy meant it wouldn’t change for about ten years. Then two years later it had changed and I had clear signs of early scleroderma in my nailfolds. Erythromelalgia is thought to be the cutaneous manifestation of small fibre neuropathy and is a neuro vascular disease. If you can get back to the stern rheumatologist then it would definitely be worth asking about it. Mine was finally diagnosed by a Sjogren’s expert rheumatologist. I tend to have this rather than Raynaud’s when I’m attending outpatients appointments so it was easy to confirm!

[tall-tim]

Hello, Shona.

In my experience with Raynaud's and digital ulceration, I can recommend Sildenafil as a good way to get peripheral circulation going. Side-effects can include a bit of heart beat and a feeling of being a bit fat-headed but those are negligible.

I have had Fluoxetine for depression but it ended badly in my opinion - it could even have triggered my Scleroderma! The mind-body system is delicately regulated - and who knows what regulates it! Oh yes - hormones, etc, but what regulates them? Probably epigenenetics, which of course are influenced by circumstances and life experiences as well as underlying genetic predisposition. I wish you all the best on your voyage of discovery, and please let us know how you are getting on.

Tim

[ShonaGreen in reply to tall-tim]

Thank you tall-tim I’m hoping for the best with the Sildenafil, good to know that it’s helped other people so definitely worth a try!

[ShonaGreen]

Hoping Sildenafil will help me, have new chilblain joining the gang with skin hardening like leather just like toe on other foot did recently, hurts like hell, but noticing the chilblains seem to have a red dot like a bullseye in the centre 🥹

[ShonaGreen]

New chilblain today

New chilblain

[Clangerscat]

Hi Shona I’m sorry you are having such a horrible time your poor feet look so painful! All that I have read about Raynauds tells me that primary is usually a more manageable disease and unlikely to produce sores or ulcers which makes me think you probably have secondary Raynauds and it is good that your consultant is checking you out for scleroderma. When I was diagnosed, after about 2 years of illness, I was told that scleroderma has quite specific antibodies and that a GP would be unlikely to test for them so it is good that you have seen a specialist. Can’t help you with the medication as my Raynauds has never got that bad but wish you luck in getting it all sorted.

[ShonaGreen in reply to Clangerscat]

Thanks for taking the time to reply Clangerscat 😊 Am hopeful with the better weather that this is the last of the chilblains for a while at least 🙏