"Temporal Arteritis:
an Exciting Journey"

Case history: One Man's Giant-Cell Arteritis

Giant Cell Arteritis (GCA)
also known as "Temporal Arteritis"

"Giant cell arteritis (GCA) is an inflammation of the lining of your arteries -- the blood vessels that carry oxygen-rich blood from your heart to the rest of your body. Most often, it affects the arteries in your head, especially those in your temples. For this reason, giant cell arteritis is sometimes called temporal arteritis or cranial arteritis.

"Giant cell arteritis frequently causes headaches, jaw pain, and blurred or double vision. Blindness and, less often, stroke are the most serious complications of giant cell arteritis.

"Prompt treatment with corticosteroid medications usually relieves symptoms of giant cell arteritis and may prevent loss of vision. You should start feeling better within days of starting your treatment."

Summary of My Experience

1. October 5, 2009: I had a "Red-Eye" -- the only one in my life. A Red-Eye is a broken capillary in the white of the eye. It passed in a few days.
2. October to February: my resting heart rose steadily from 72 to 92. My weight fell to 10 pounds below my normal 180.
3. December 22: I saw a pulmonary doctor about spasms of dry cough and generally not feeling well. I have a history of chronic bronchitis.
4. December 22: chest X-RAY
5. February 6: CT-scan only showed aging lungs. The diagnosis was post-nasal drip but I was given an anti-biotic in case there was an infection.
6. February 9, 2010: I began a severe night headache on the right side and persistent constipation; both new experiences for me.
7. March 4, 2010: A pulmonary function test showed a normal decline with age.
8. March 9, 2010: I saw my primary-care doctor regarding the headache and constipation.
9. March 11: An MRI brain scan for age 87 -- no brain tumor.
10. March 11: the doctor phoned to send me to the pharmacy for prednisone. The lab tests of ESR "sed rate" and CRP had led him immediately to the diagnosis of giant-cell arteritis. This doctor is a geriatrician and GCA is a disease of age over 55. The prednisone level started at 60 mg each morning. I did not take supportive drugs other than calcium and vitamin D.
11. March 12: the doctor finished my colonoscopy and announced "you don't have cancer" but I had already begun writing my Memoirs.
12. March 17: the biopsy of a right-temple artery confirmed giant-cell arteritis. My weight had fallen another 8-10 pounds.
13. April 19: I was feeling well and began the exploration of local hiking trails. I rapidly regained my resting heart rate of 72 and my weight of 180.
14. June 14: at 12 mg, Addisonian prednisone withdrawal symptoms started
15. June 22 and 23: a fast trip to the emergency ward. In the hospital the prednisone was restored and countless tests revealed nothing apart from age 87.
16. October 11: At 10 mg in mid Ocober I had a flare-up of GCA with fleeting head pains and two inflamed arteries above my right ear, one with a painful spot.
17. June 1:I am taking 5 or 6 mg and just got back from a long hike in the hills. Who ever heard of GCA? What a strange affliction.
18. August 13, 2010: my 89th birthday. I am taking four mg and testing for going down to three.
19. September 1: My doctor declined to order my ESR & CRP lab tests. I requested them and the doctor then ordered them to "make you feel better." The tests were high. The doctor's message was don't worry, it's just your age. On my own, I will go back to four mg for another four weeks. See the discussion of "Prednisone Withdrawal" below.

My GCA diagnosis

American College of Rheumatology recommends that GCA be diagnosed if three of the following are true:

1. Age is 50 or older
2. New, localized headache
3. Temporal artery tenderness or decreased pulse
4. ESR greater than 50 mm/hr
5. Temporal artery biopsy positive for GCA

Out of curiosity I was occasionally recording my blood pressure and resting heart rate and noticed later that my heart rate had been rising steadily for three months.

I was not aware of a health problem during this rise until other symptoms sent me to a pulmonary doctor at the end of the three months.

GCA Symptoms as reported in the literature -- (I experienced those marked **red.)

1. ** Weight loss -- (10 pounds during months before GCA diagnosis; another 10 before it began to rise)
2. ** Fatigue
3. ** Malaise: The feeling of overall ill health and weakness.
4. ** Constitutional symptoms: In this subset of GCA, the disease process. is dominated by manifestations of system-wide inflammation, including rise in ESR and/or CRP
5. ** Dry cough
   a cough is not often listed as a symptom of GCA
   The Mayo Clinic Proc. 1997 Nov;72(11):1048-50 reported such a case.
6. ** Headaches: throbbing, sharp or dull headaches may bring you to your doctor.
   February 9, 2009: headache started and severe constipation (unusual for me).
   March 11, 2009: the awful headaches did get me to my first-care physician
   The headache stopped within two days on prednisone.
7. Tenderness near the temples: Noticed most when wearing glasses, combing hair or lying on a pillow.
   
   ** experienced only at a flare-up of GCA months later
8. Fever: Spiking temperature and chills will bring you to the doctor. Or low-grade fever
9. Loss of appetite --
   ** (not noticed -- but I had lost 10 pounds of my 180)
10. Jaw claudication: pain, tension and weakness of the muscles that allow you to chew and talk.
11. Less common: Tongue, throat and neck pain
12. Amaurosis fugax: A fleeting visual blurring with heat or exercise, or posture-related visual blurring; it may precede partial or complete blindness and requires an emergency visit to the doctor.
13. Double vision (diplopia) --
    ** I had one brief experience of this, date not recorded.
14. Blindness: Loss of vision is sudden, painless and usually permanent.

Prednisone has serious side effects

1. ** Insomnia (only at high prednisone levels)
2. Elevated pressure in the eyes (glaucoma)
3. ** Fluid retention, causing swelling in your lower legs
4. Increased blood pressure
5. Mood swings
6. Increased appetite,
   ** weight gain (I recovered what I had lost)
7. High blood sugar, which can trigger or worsen diabetes
8. Increased risk of infections
9. Thin skin, easy bruising and slower wound healing
10. Blurred vision. Perhaps -- like "watering" of eyes.
11. Acne
    **( No acne but I observe scaling spots.)
12. Stomach irritation
13. Nervousness, restlessness
    -----------------------

    Longer term effects:

14. Loss of calcium from bones which can lead to osteoporosis and fractures
15. Increased growth of body hair
16. Muscle weakness
17. Swollen, "puffy" face
18. Weight gain; fat deposits abdomen, face, back of neck
19. Cataracts

Insomnia and Sleeping Pills

Insomnia is a common, perhaps universal, effect of high prednisone doses. I had great fun with this, but one bad experience with a pill.

I was given a prescription for Ambien, a widely promoted sleeping pill -- apparently the best available by some accounts. It gave me a good night's sleep -- or was it a coma? The following night -- without it -- was a night-long nightmare -- no sleep whatever. The rebound, the backlash, from its use was total. One could get trapped into lifelong use. In a second experiment I awoke at night and nearly fell into the furniture. I could have been injured. No more Ambien for me.

Some years ago I had occasion to study sleep and sleeping pills in detail -- just curiosity.

I concluded then and believe now that all such drugs should be outlawed.
They either do not work or are addictive and may be downright dangerous.
(This is not the place to publish my research on the subject.)

My insomnia became a game. I would sleep two hours, be awake three, then sleep three hours. I could choose to get up and work during the three awake hours or just meditate in bed. The five hours of sleep were supplemented by napping during TV watching.

The insomnia stopped when the prednisone level got down to about 20 mg or 15 mg from the initial 60 mg.

Prednisone Withdrawal

There are three objectives:

1. prevent damage to the arteritis by the GCA inflammation
2. minimize prednisone side effects
3. avoid an Addison's crisis of too little cortisol

GCA may be be self-curing but may last from six months to six years or longer.
Prednisone wards off the damaging inflamation but does not cure GCA.

The words of William Blake are the guide to prednisone withdrawal:
    "The road of excess leads to the palace of wisdom."
    "We never know what is enough until we know what is more than enough."

GCA may be thought of as hiding under the carpet of prednisone.
Only by lifting the carpet -- withdrawing the prednisone -- can we learn it is there and then increase the prednisone to suppress it.

We learn it is there when it causes trouble. GCA symptoms at this stage may be very subtle.
The first sign of its presence may by some degree of vision loss.

In addition to the clue given by symptoms, blood tests are used.
The "sedimentation rate" (ESR) or the C-reactive protein (CRP) may reveal the GCA but these are affected by other events, such as a common cold.

Prednisone withdrawal is an exciting and dangerous game.

The graph below shows my prednisone withdrawal, the rise and fall of ESR, and the occurrence of a relapse -- a flair up of GCA.

1. an Addison crisis that put me in the hospital for two nights, getting a strong dose of prednisone intravenously.
2. a relapse signaled by headaches and two swollen arteries above my right ear, one with a spot painful to touch. (It could have been an artery affecting my vision.) The giant cell arteritis smolders under the prednisone carpet, waiting its chance to flair up.

"My study has shown that determining the maintenance dose of steroid therapy is a slow, laborious, painstaking job, taking months or even years. The guiding principle, obviously, is to maintain the lowest level of ESR and CRP with the lowest dose of Prednisone. As mentioned above, my study showed marked inter-individual variation among GCA patients in: (a) the amount and duration of steroid required to control the active disease, (b) the time needed to reach a maintenance dose, (c) the maintenance dose required to keep the disease under control to prevent blindness, and (d) the total length of treatment. Therefore, steroid therapy for GCA has to be individualized. I have found that most GCA patients require a virtually life-long, very small maintenance dose, which has little or no systemic side-effects. A common mistake made by physicians in these cases is to taper the steroids down rapidly to a very low dosage and then discontinue it. There is a common belief among rheumatologists that GCA burns itself out in a year or two, and steroids can then be tapered off unless the patient develops systemic symptoms. As discussed above, I have found this notion to be completely wrong, and can prove disastrous, because repeat temporal artery biopsy has shown evidence of active disease even after 9 years of steroid therapy. The advice to base treatment on the clinical picture, rather than laboratory tests (i.e. ESR and CRP) may be true for polymyalgia rheumatica patients. But it can be dangerous for patients with GCA, who may lose vision irrevocably without developing any warning systemic symptoms at all; moreover, 21% of patients with visual loss have occult GCA. I have found that the only trustworthy and safe parameters to regulate the steroid therapy and to prevent visual loss are the levels of ESR and CRP, and nothing else."

Withdrawal Symptoms (Addisonian)

A deficiency of the adrenal cortex hormone, cortisol, is an Addison's-disease crisis.
After weeks of prednisone the adrenal cortex "goes into hibernation."
Then withdrawal of prednisone too quickly may induce an Addison's crisis until the adrenal's get back into their business. In my case this happened after going from 15 mg to 12.5 mg.
Neither I nor my doctor saw this crisis coming although there were warning symptoms for a week.

I had leg pain on Sunday, lethargy all week, no appetite after Thursday. Upon awakening in a sweat the following Monday morning, my sitting or standing blood pressure was 70/50 and I could barely walk.
My doctor ordered me to the emergency ward where I received intra-venous cortisone and was hospitalized for two nights. Countless tests confirmed my age of 88 but nothing else to worry about. These tests were necessary for the hospital to rule out other causes of my collapse.

I experienced the warnings marked **red.

1. ** Sudden penetrating pain in the legs,
   lower back, or abdomen
2. ** Severe lethargy
3. ** A general ill feeling
4. ** Fever
5. ** Loss of appetite
6. ** Lightheadedness standing (my blood pressure: 70/50)
7. ** Profound weakness
8. Muscle weakness
9. Loss of consciousness
10. Body aches
11. Abdominal pain
12. Rapid heart rate
13. Skin rash or lesions
14. "vibration" (soles of feet?)
    I have experienced this from time to time.
15. Headaches
16. Nausea or vomiting; dehydration
17. Low blood sugar (hypoglycemia)
18. Flank pain, joint pain
19. Confusion or coma
20. Rapid respiratory rate (see tachypnea)
21. Shaking chills
22. Slow, sluggish movement
23. Unusual and excessive sweating on face or palms
24. Difficulty breathing
25. Mental changes
26. Salt craving
27. Longer term effects: unintentional weight loss; darkening of the skin

Withdrawal Symptoms -- guides: relapse and blood tests

Warnings of high values for ESR and CRP at my medical laboratory are 20 mm/hr and 9 mg/L.
These are arbitrary limits somehow established as convenient warning levels.
Mine ESR levels have been: 49, 4, 11, 7, 15, 7, 17, 9, 15, 10, 6, 16, 10 .
The 15, 16, and 17 are warning peaks for me (see the graph).
My CRP has shown little variation since its initial high (a slight rise when I had a common cold).
The individual's record, not arbitrary limits, are the correct guide.

That is my understanding of the urgent recommendation from the Opthalmology Department of the University of Iowa:

Hayreh and Zimmernam; Management of Giant Cell Arteritis; 2003 (See the bibliography.)

"In our study when a rise in ESR and CRP occurred soon after the steroid dosage was lowered, there was never a simultaneous corresponding change in systemic symptoms of GCA. So any reliance on systemic symptoms of GCA as a guide to flare-ups exposes the patient to the risk of blindness. Therefore, the frequent advice in the rheumatologic literature that 'alterations in treatment should be based on clinical picture, rather than on laboratory tests' can be dangerous – patients may lose vision irrevocably without any clinical warning systemic symptoms of GCA..."

"Our study has revealed that ESR and CRP are the two most reliable and sensitive parameters to monitor GCA activity and to regulate steroid therapy, and not the clinical picture or systemic symptoms of GCA."

and:

"To attain an endpoint of reaching the lowest dose, the patient must have stayed at the lowest dose level for at least 3 months with no further change in dose beyond that period. This is because it was not uncommon to find that on lowering the dose of prednisone, the ESR and CRP started to go up within few days or weeks, indicating that that dose was not adequate to control the GCA."

At this time I have the difficult task of detecting subtle hints of relapse and am watching the ESR and CRP with great interest. See the graph above.

With my doctor's permission, I am "micro managing" my prednisone.

Bibliography

There are many reliable sources,who provide the accepted medical knowledge and practice, for example:

Mayo Clinic's internet article on GCA

Cleveland Clinic article on GCA

The internet is easily searched for "gca" or "temporal arteritis".

The Johns Hopkins hospital has a web page for GCA: Johns Hopkins GCA

Their more general page on vasculitis also has useful GCA information: Vasculitis FAQ's

The Opthalmalogy Department of the University of Iowa hospital has been referenced in the text above. Their articles are valuable reading for anyone with GCA.

This "Exciting Journey" you are looking at is a counter-point to James W. Rupp's sad story of his wife's long and difficult medical history:
"Giant Cell Arteritis - An Elusive Odyssey"

Single cases do not make medical science, but Rupp's story illustrates the complexity of giant cell arteritis.

Rupp gives a 134 page collection of abstracts giving the essence of many articles.
"Giant Cell Arteritis -- A collection of Medical Journal Abstracts"