Trying to establish what happened to my Mum but don't know who to ask.

[DoreenM]

Could I ask please whether anyone here has any knowledge of, or had experience of, bilateral occlusions of the femoral arteries resulting in severe ulcers, necrotic toes etc as a complication of PMR/GCA – possibly a rare manifestation of GCA affecting the lower limbs? Thank you.

[PMRpro Ambassador]

Not quite sure what it is you want to know.

Yes, peripheral vascular disease, in particular peripheral arterial occlusive disease, can be a late consequence of vasculitis because of the damage done by the inflammation to the lining of the arteries. This then tends to "catch" any passing clots or plaques and other things which causes a build up on the inside of the artery and eventually to occlusion - like a traffic jam on a motorway as traffic gets heavier and lanes are closed for some reason.

If the blockage occurs over a long period the body builds an alternative blood supply, called collateral circulation, like the back roads around the motorway hold-up, and the deliveries of oxygen and nutrients continues, albeit reduced. If the final blockage occurs suddenly the body doesn't have time to do that - the blood supply to the other side of the blockage is reduced too far and eventually the oxygen and nutrient supply fails. The result is the cells die off. First you get ulcers and black toes, eventually you get gangrene. Sometimes it is possible to remove the blockage and blood flow gets back to being enough but not always. It is the difference between the hold up being due to an accident - which you can remove - or due to too much traffic heading for 3 lanes turning into only 1, which you may not b able to remove.

PVD and PAOD can both happen anyway but GCA probably does increase the risk.

[DoreenM in reply to PMRpro]

Thank you for your reply which has explained things very clearly and what I feel sure is what happened to my mother. She actually passed away over two years ago but to this day I have been unable to make sense of how a previously healthy, fit and active 86 year old with no history of vascular disease could deteriorate so rapidly that in a few short months she was no longer with us.

She was diagnosed with PMR in May 2014 (straight after a bout of shingles) and commenced with 20mg prednisolone. After improved blood tests in July her dose was reduced to 10mg on which she continued. However, by then her legs were becoming painful and rapidly discolouring. She could not be on them for any length of time due to the pain. Her GP referred her to the Vascular Consultant and a CT angiogram in September reported bilateral severe occlusive arterial disease from the common femorals all the way to the feet with no continuous run off vessels. A lack of significant atheroma within the arteries led to the conclusion that the blockages were a result of vasculitis/PMR rather than atherosclerotic disease. She apparently had excellent collateral circulation. Even so, her legs were breaking down rapidly with deep ulcers on the calves and black patches on the toes etc. A biopsy of the ulcer showed it to be a typical ulcer with no evidence of vasculitis. Thereafter the ulcers were cared for at home by the district nurses, they became infected and it was impossible to see how they could heal. We had been told that surgical intervention was not an option due to issues of risk v. outcome. Sadly all this took a terrible toll on her both physically and mentally. She considered her life was effectively over as she could do little except sit in the chair and was dependant on others for everything. In early November she went downhill, gradually stopped eating and drinking and, we believe, allowed herself to slip away.

Having mulled all this over during the last two years, the question I keep coming back to is whether Mum would have stood a better chance if her steroids had been increased again earlier on when it was clear something major was going on with her legs? She had been on 10mg prednisolone since July when her blood markers had gone down to ESR 5, CRP 28, but these increased again as her condition worsened and in September were ESR 30, CRP 82. There was never any mention of increasing the steroids or introducing other similar medication, so do I take it this was because it wouldn't have made any difference?

Thanks again for your kind reply. It was hard watching my mother suffer and I want to understand her illness as best I can.

[PMRpro Ambassador in reply to DoreenM]

While you say "with no history of vascular disease" since they won't ever have looked they wouldn't have known. There was good collateral circulation - so there would probably have been no claudication which is the usual sign there is something wrong. I doubt that giving higher dose pred at the outset when there was no evidence of GCA would have made a difference either to the occlusion - it wasn't just inflamed tissue, occlusion that is as extensive as that doesn't develop in just a short while, and most doctors feel that 20mg will deal with most GCA, the high doses are used to reduce the risk of visual loss when it is affecting the cranial arteries. The high doses have significant side effects, especially in the older patient.

But you have also to consider - at 86 she was already a venerable age and we do not and can not live for ever. We have to die of something and I think you are probably correct in thinking she allowed herself to slip away - all that made her life worth living was gone and her quality of life was poor. My 79-year-old mother knew she was soon likely to lose her independence due to advancing cardiac insufficiency and quite deliberately stopped taking the medication that was supporting her cardiac function. My brother was beside himself, blaming the doctors, but the evidence was there in the form of unopened packs of medication that it was her choice.

The increasing ESR and CRP were likely to have have been the result of the advancing tissue damage rather than inflammation. They are very non-specific markers and also rise in tissue necrosis, you mentioned the toes were necrotic. As the necrosis advances it releases toxins into the body - which themselves progress the deterioration. Where a patient is fit enough to survive surgery, amputation of small amounts of necrotic tissue may result in recovery - but it all depends how far the damage goes. If the occlusion was all down the legs - it could also have been into the abdomen. There is only so much that could have been done. And life as a bilateral amputee would have been no better than she had anyway. Once a certain amount of necrosis is present it progresses and there no turning the tide back I'm afraid.

I think you can rest assured that had there been something to be done it would have been - but there are not medical answers to all such problems. Hold on to the good memories you have of your mother. And remember - she lives on in you and your children if you have any.

[Judyliz in reply to PMRpro]

Having read the letter from DoreenM, I am sure there will be many of us who can empathise and remember being full of questions once our initial grief following the death of a loved one had subsided.

The response from PMR Pro is, in my opinion, one of the most helpful, fact filled and readable without being too scientific replies I have read on this forum. It covered much that DoreenM needed to help her accept the circumstances of her mother's death. Death can be a messy business and not straight forward, so, having an explanation at a time we are able to hear and understand will allow a measure of acceptance and calm. This might be straight away but also it might be 2 years later. Well done to you both.

[PMRpro Ambassador in reply to Judyliz]

Thank you.

[DoreenM]

Thank you PMRpro. I totally agree from a philosophical point of view that at 86, death from one cause or other is likely. And yes, there was no knowing all the underlying things which may have been present in a person of that age. It was not her death per se that upset me, more the bewilderment of it all and then the lingering doubts since about whether I did enough or, worse, whether the medical professionals did enough. This came about because I had not had a clear explanation, such as you have now given, about how the PMR is implicated in the arterial disease. Of course I have known many people who have experienced PMR but I have never heard of anyone dying from this kind of complication. I would be interested to know how common this is statistically? I actually admire my Mum's decision, if that's what it was, to bale out rather than wait for death in worsening pain with no quality of life - especially if gangrene and amputation may have been likely - and I have always been comforted by the fact that she died peacefully in her own bed.

I feel like something of a weight has been lifted now and I can 'let this go' as they say. Thank again so much for taking the time and trouble to reply, I really appreciate it.

Judyliz: Thank you - I agree completely with what you say. PMRpro's reply has indeed brought acceptance and calm for me and hopefully these posts may also be able to help others in a similar position.

[PMRpro Ambassador in reply to DoreenM]

ncbi.nlm.nih.gov/pubmed/262...

suggests that the relative risk of DEVELOPING peripheral arterial disease is 1.88 - almost twice as likely as someone with no history of GCA. That isn't the same as dying of it - patients can have PAD that can be managed/treated, depending on where it manifests and how severe it is.

Thank you both for your kind words. If the time I spend online has helped someone it has made what I do worth it. PMR (together with age) has stopped me doing many things I enjoyed - if my voluntary work to fill my time achieves something then it is very satisfying.

[piglette in reply to DoreenM]

I think guilt and doubt is very usual for many of us when a loved one dies, did we do enough? Did we do the right thing? I know I still worry about my actions when my mother died and that was twenty years ago. I really sympathise with your worries.

[DoreenM]

Thank you. x