GCA at 46...my story

My name is Jonathan and this is my story; I am a 46 year old male who contracted temporal arteritis (also known as giant cell arteritis or GCA) with amaurosis fujax and nearly went permanently blind.

By setting out the sequence of events it is my intention to assist others to diagnose this rare (2.2/10 000 persons) auto-immune disorder when it arises in younger sufferers and thereby prevent blindness and other serious complications which can occur when the condition is not identified sufficiently early.

My GCA was not diagnosed early as a result of a combination of circumstances, most notably my age and gender which both contradicted an obvious diagnoses, and the onset of the swine flu epidemic which at a key time diverted resources at my GP’s surgery from blood tests to inoculations.

I have tried to be succinct and objective (this account runs to four pages), to include sufficient detail to inform, but not enough to bore; I hope I have achieved my objective, if not then I apologise.

I have maintained a good level of CV fitness all my adult life, and although I am slightly overweight (6’0 tall, 95kg) and drink above the recommended level, I don’t smoke and am by and large in very good physical and mental health. My research indicates that it may be relevant that from 13 to 16 years old I suffered from juvenile rheumatoid arthritis.

That said, by the summer of 2009 I had been suffering for some months from a painful right shoulder that ached particularly at night. On occasions the pain was severe and only reduced by the application of heat and the taking pain-killers and anti-inflammatory ‘over the counter’ drugs.

Eventually I consulted my G.P. and, following an x-ray which showed there was no injury to the joint, I was diagnosed with ’frozen shoulder’ (with hindsight, this was most certainly the first symptom of my GCA and when I began taking prednisolone it was the first to disappear).

Then on 10th September 2009 I banged my left knee against the sharp corner of a heavy chair whilst at work. Although I took very little notice of it at the time, this event would coincide to the day with a rapid decline in my health.

What the injury to the knee most certainly did (I had in fact I had broken my cartilage) was to mask other symptoms of GCA that were beginning to emerge; most notably of which was an intense aching in both my lower legs; I erroneously attributed this pain however to the knee injury. No more than a week after my legs started to ache I experienced severe fatigue, but I put the fatigue down to the aching legs and the aching legs down to the knee; it all seemed to make sense, for a short while at least.

Over the next week the aching got worse, especially in the morning, the fatigue became more debilitating and the shoulder breathtakingly painful. In addition, I began to suffer from night sweats but with no accompanying fever. It began to dawn on me that these symptoms could not all emanate from a relatively unexciting knock to my knee.

So on 18th September I went to my GP presenting fatigue; he ordered a blood test, but the swine flu epidemic meant that I had to wait until 5th October for the test, then over another two weeks to get the results and see my GP again. When I did, on 23rd October, I was informed my C-Reactive Protein (CRP) was 137. My GP referred me to a consultant rheumatologist and suggested that I might be suffering from systemic lupus.

At this time (5th to 22nd October) I could manage no more than a few hours work before having to return home early to collapse exhausted on the settee by mid-afternoon; pain-killers and anti-inflammatory tablets became part of my daily diet. I was losing weight, about a stone, or to be more precise I was losing all muscle tone. I was feeling very unwell; so unwell in fact that I gave up drinking for a week!

I continued to work, proud of my impressive sickness record and contemptuous of those lesser mortals who succumbed to illness; whatever else was happening, the air was thick with hubris.

Whilst awaiting to see the consultant (23rd October to 2nd November) I started to improve. The fatigue reduced significantly and the pain in my shoulder became sporadic; the aching legs continued but lessened.

During this period however a completely new and quite disconcerting symptom emerged, an extremely tender scalp. At first this was at the back of the head, just above the neck; then it moved over both temples and latterly to behind both ears. It was as if my head was bruised and my skull was sensitive to the touch; it was not a head-ache but something distinctly different.

On 3rd November I had my first appointment with my consultant, a very experienced Senior Rheumatologist at the Royal Berkshire Hospital, who after careful and protracted examination assessed that I was unlikely to have systemic lupus but was more than likely suffering from either vasculitis, temporal/GCA or possibly lymphoma; incidentally a more recent blood test showed an CRP of 87 and an E Sedimentary Rate (ESR) of 88.

One significant matter seemed to be troubling my consultant, as I was too young to have vasculities or temporal arteritis; he expressed concern that I may in fact be suffering from endocarditis, which he explained was a medical condition emanating from an infection of the heart/aorta disorder; to assist his diagnosis he arranged a CT scan of my heart and abdomen.

On 13th November he started me on a low dose of steroids (15mg of prednisolone); he was proceeding cautiously, an approach with which I was concurred.

I researched GCA and learnt of the threat it presented to my sight; I learnt that men were more likely to go blind than women and that blindness in either eye could be followed by blindness in the other within a week. Once lost, the sight could not be returned.

Within days, the shoulder improved, the legs ached less but were still uncomfortable, and the scalp tenderness decreased but still troubled me.

Then less than a week later on 19th November, I lost my sight almost completely four times in my left eye within a two hour period. Each time an area of deepest black appeared across my vision and increased until I was nearly totally blind, and then, five to ten seconds later it cleared.

I went to A&E immediately and, some hours later having argued hard with the staff to convince them I wasn’t imagining it, I was eventually prescribed 30mg of soluble aspirin and 60mg of prednisolone. A blood test taken at this time showed a CRP of 67. I was sent home and erroneously and repeatedly reassured the loss of vision was nothing to do with my GCA.

The following day (20th November) upon learning of my loss of vision my consultant immediately admitted me to hospital to facilitate the rapid administration of a serious of drugs, 1000mg of methylprednisolone, tinzaparin, simvastatins and dispersible aspirin to combat the threat to my sight. On this day I reported sick from work.

The CT scan on my heart and abdomen showed nothing untoward, importantly I did not have lymphoma, and several detailed eye tests revealed no permanent damage.

Both eye consultants agreed with my consultant, I had experienced several episodes of amaurosis and been extremely fortunate not to go blind; truly terrifying though the episode was, it strongly supported the GCA diagnoses which my consultant had made less than a week previously.

On 23rd November my CRP was 15 and ESR 17 and with it the threat to my sight was slightly diminished. I wasn’t sure whether I have been extremely unlucky or extremely unlucky. At this time, I was on 60mg prednisolone a day.

Over the coming week (24th November to 1st December), and whilst on the very high dose of prednisolone, several things happened:

The sensitivity in my head reduced significantly.

My left knee, which the attentive reader may recall I injured months previously, actually started to hurt with the onset of an intermittent sharp pain and was uncomfortable to walk on.

The pain in my shoulder ceased.

My legs no longer ached.

The night sweats stopped, although they had already significantly reduced.

The prominent veins in the back of my hands re-appeared.

Having extensively researched the symptoms and reading the accounts of others with this condition, I have absolutely no doubt that the diagnoses is correct; one symptom I have never experienced though is the jaw stiffness about which some 50% of sufferers complain, research suggests the ‘jaw’ symptom is more common in females and ‘blindness’ in males.

Interestingly, the onset of GCA is more prevalent in summer and in the south rather than the north of the UK, both factors are in common with my own story (for these and other findings see Dr Liam Smeeth’s 2001 study entitled “Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990–2001”: Department of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine).

On 27th November, a further blood test, whilst still on 60mg of prednisolone a day, returned a CRP of 3.2 and a ESR of 5.

On 30th November I had an MRI scans on my orbits and brain and optical nerves; both were undamaged with venus flow in the cavernous sinus normal.

On 3rd December my consultant instigated a plan to reduce my prednisolone from 60mg per day whilst monitoring my CRP and ESR readings.

On 8th December my CRP was 5.4 and my ESR 5; it appeared the GCA was coming under control; at this time my prednisolone dosage had reduced to 25mg.

On 17th December an ECG, an echocardiogram and an examination by a cardiologist eliminated endocarditis or any other heart related cause.

On 18th December my CRP was 7.2 and ESR 5; at the time of this blood test my prednisolone dosage had been at 18mg for a week.

And on 21st December the results of a temporal artery biopsy of 15th inst. showed no evidence of inflammation to suggest the presence of a temporal arteritis. However, temporal arteritis is a segmental process which is not excluded by a negative arterial biopsy.

By now I was feeling significantly better and only troubled by, pain is the wrong word, sporadic feelings of pressure in my head; sometimes its over the temples, others at the back of the head - it definitely moves and some days are worse than others. The sensation not painful, just disconcerting.

So by late December 2009 the GCA appeared to be in remission, and the process of gradually reducing the prednisolone whilst carefully monitoring the blood for inflammation had began.

Throughout these events I have come across medical professionals who because of my age either did not consider or, once I had been diagnosed were sceptical about my very experienced consultant’s diagnoses of GCA; but my research clearly indicates that whilst it’s occurrence in persons under 50 is rare, the medical world is in fact littered with cases of GCA involving people under 50, and interestingly a higher proportion of ‘younger’ sufferers appear to be male.

Indeed, in 2008 a 46 year old male was diagnosed with GCA in Derbyshire.

To quote Anthony Allen MD Chief, Interventional Radiology, Brooke Army Centre US, an Associate Professor of Radiology as recently as 2008; “Although temporal arteritis (giant cell arteritis) occurs almost exclusively in patients older than 50 years, well-documented cases have been reported in patients as young as 40 years“.

So guys, don’t dismiss GCA…our sight lies literally in your quick diagnosis and swift treatment.

Kind regards

A 46 YEAR OLD GIANT CELL ARTERITIS SUFFERER.

(Jonathan Cox, ***** ******)

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