to HOME PAGE

Giant Cell Arteritis; One Man's Exciting Experience
GCA - also known as Temporal Arteritis

A detailed explanation of the treatment of GCA
Mason A. Clark see last update: August 2016


Important -- Read this first !

GCA is an emergency, can cause some loss of vision. If you have or may have GCA you must consult a physician immediately and you must participate intelligently in the treatment.
This post describes my exciting GCA experience with a favorable result.
I am not a medical doctor. Some of my understandings of my own experience may differ from that of my doctor and your doctor.
This report is not medical advise.   It is simply one person's experience with GCA.

NOTE: Before using these links scan or read the article below the Table of Contents.

Here is a forum where you may share experiences with GCA, PMR, and prednisone:
NOTE: Those links will take you away. Come back or first scan or read the article below.
Table of Contents
  1. Definition of GCA
  2. Diagnosis of GCA
  3. My Warning Signs
  4. Diagnosis of My GCA
  5. Treatment of GCA -- Prednisone
  6. Cortisol Insuficiency -- the Addison's-Disease Effect
  7. Prednisone Side Effects
  8. Prednisone Withdrawal
  9. The Management of GCA with Prednisone
  10. The Paradox: Flare First, Treatment After
  11. Appendices
  12. Understanding Blood Tests
  13. GCA Symptoms
  14. Symptoms of Cortisol Insufficiency (Addisonian)
  15. Bibliography
  16. Support Groups


Definition of GCA by Mayo Clinic staff

"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."

Diagnosis of GCA

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. Blood test: erythrocyte sedimentation rate, ESR, greater than 50 mm/hr
  5. Temporal-artery biopsy positive for GCA
An additional, strong clue is the very quick relief of symptoms by taking prednisone, usually in one or two days.

It is universally agreed that if GCA is suspect, prednisone should be given immediately to prevent loss of vision.

(A lengthy list of GCA symptoms is given in an Appendix)

My Warning Signs


I had several unusual health experiences in the six months before my GCA crisis:

    Warnings?
  1. An ear infection, only time in my life. Antibiotics cured it.
  2. A red eye, only time in my life. It subsided in a week or two.
  3. A loss of weight from about 183 to about 173. Unusual for me
  4. A rising resting heart rate from 72 to 90 over a three-month period.

I was not otherwise aware of a health problem during this time until a dry cough sent me to a pulmonary doctor. The x-ray and CT scan of my lungs did not explain the cough and the diagnosis was "post-nasal drip" but an antibiotic was prescribed "just in case."' The CT scan was done on February 5.

Then, on February 9, 2009 there began a severe headache which on the next day was entirely on the right side. Simultaneously -- on the same day -- there began a severe, persistent constipation -- unusual for me.

On February 27 I visited my primary-care physician and had a blood test including ESR (erythmocyte sedimenation rate).

Diagnosis of My GCA

The diagnosis of GCA was made on March 11, 2009 based on severe headaches on the right side and blood tests showing a high ESR of 50.
Prednisone was begun at 60 mg. each day.
A biopsy of a temporal artery was done on March 17, 2009 and confirmed GCA.

A brain MRI and a colonoscopy had been ordered. Neither showed anything important (but the constipation was brought to an abrupt end).

Treatment of GCA -- Prednisone

Table of Contents

Note: prednisone is commonly prescribed in the U.S., prednisolone in the U.K. Other variations of prednisone may be prescribed, such as methyl prednisone and hydrocortisone. Coated tablets are sometimes used to minimize stomache irritation. The differences are not important, although the quantities prescribed and schedule of use may differ. For this article, only the word "prednisone" will be used,

Prednisone or other corticosteriods are the only treatment of GCA. Prednisone does not cure the arteritis. It only provides relief from the symptoms and reduces the inflammation. The inflammation is harmful to large, flexible arteries including the large aorta leading from the heart and large leg arteries. It is important that the inflammation be reduced.

The arteritis subsides on its own over a period of time of its own choosing. That time may range from less than a year to two years, five years, or the remainder of lifetime. As time goes on, less prednisone is needed culminating in as little as zero or one milligram per day to as much as 16 mg. per day.

The treatment task is to reduce the prednisone as quickly as possible without permitting a dangerous flare-up of the arteritis. Dangerous because some degree of loss of vision may occur. Too-rapid a withdrawal may also cause a cortisol insufficiency.

Cortisol Insufficiency -- the Addison's-Disease Effect

The endocrine system controls the amount of cortisol -- the adrenal cortex hormone -- that is fed to the blood stream. The prednisone treatement causes the system to reduce the generation of cortisol. The adrenal cortex goes into hybernation and in a few weeks time loses its ability to produce cortisol. Addison's disease is a failure of the glands to produce cortisol. Withdrawing prednisone leaves the body short of cortisol. The symptoms of Addison's disease appear, possibly including a life-threatening crisis.

(A list of cortisol-insufficiency symptoms is given as an appendix: Appendix

Prednisone must be withdrawn slowly enough to allow the adrenal glands to recover and to supply the natural amount of cortisol. But there is an urgency in this withdrawal because prednisone has serious side effects.

Prednisone Side Effects

Table of Contents
Your doctor will discuss these with you. Some side effects demand regular attention.
I experienced those marked **
  1. ** Insomnia (only temporarily at highest prednisone levels)
  2. ** Fluid retention, causing swelling in lower legs (only temporarily at highest prednisone levels)
  3. ** Increased appetite, weight gain -- I recovered only what I had lost
  4. ** Blurred vision Perhaps, like "watering" of eyes, not significant
  5. ** Cataracts -- barely perceptible August 2015 after 17 grams of prednisone over six years.
  6. Loss of calcium from bones which can lead to osteoporosis and fractures
         protective drugs are often prescribed (these have side effects)
  7. Elevated pressure in the eyes (glaucoma) (tests are imperative)
  8. Stomach irritation
         protective drugs are often prescribed (these have side effects)
  9. High blood sugar, which can trigger or worsen diabetes (glucose tests are imperative)
  10. Increased blood pressure (should be monitored)

Muscle weakness; Mood swings; Increased risk of infections
Thin skin, easy bruising and slower wound healing
Acne; Nervousness, restlessness; Increased growth of body hair
Swollen, "puffy" face; Weight gain; fat deposits abdomen, face, back of neck
and others

Prednisone Withdrawal -- the Story of GCA Treatment

Table of Contents

There are three objectives:

  1. prevent damage to arteries by the GCA inflammation, especially the artery supplying the optic nerve behind the eye. Damage to this nerve causes loss of vision, perhaps complete and irreversible. Leg arteries and the large aorta arteries may also be damaged.
  2. minimize prednisone side effects
  3. avoid a crisis of too little cortisol, simulating Addison's disease and dangerous.

GCA may be be self-curing but may last from six months to two years or longer. During its life it may do great damage. Treatment is essential. Prednisone wards off the damaging inflamation, giving GCA time to subside, but does not cure GCA.

Withdrawal of prednisone is experimental. Paraphrasing the words of William Blake:

The road of excess leads to the palace of wisdom.
We never know how much is enough until we know how much is not enough.

GCA is hiding under the carpet of prednisone.
Lifting the carpet -- withdrawing the prednisone too aggressively -- can let the GCA tiger out.
The experience of GCA patients is expressed in the words of Blake:

Tiger, tiger, burning bright
In the forests of the night,
What immortal hand or eye
Could frame thy fearful symmetry?"

GCA symptoms at this stage may be very subtle or the first warning symptom may by some degree of vision loss. For that reason, caution is required.

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

Prednisone withdrawal is an exciting and dangerous, but necessary game.

The following description of my experience is easy to analyze long after the fact.
Hindsight is a wonderful tool. Making decisions in real time is far more difficult.
Nothing here should be read as a criticism of the decisions of my doctor or myself.

A detailed chronology of my experience is given in the book.

The graphs below shows my prednisone withdrawal, the rise and fall of ESR and CRP, and important events.

  1. At 12.5 mg, an Addison crisis that put me in the hospital for two nights, getting a strong dose of a kind of prednisone intravenously.
  2. At 10 mg, a relapse signaled by headaches and two swollen arteries above my right ear, one with a spot painful to touch. (This flare-up could have struck an artery affecting my vision.)
  3. at 5 to 3 mg, uncomfortable, stiff legs discouraging long walks

Giant cell arteritis smolders under the prednisone carpet, waiting its chance to flare

flames3.gif
The blood tests, ESR and CRP, along with symptoms give us instruction for the management of the prednisone.

The new patient and doctor will not know the patient's normal ESR and CRP. The normals for the population do not apply. They are well-known but useless in managing the prednisone for a particular patient. The individual's normal can only be learned by observation as treatment proceeds.

Dr. Hunder, Professor Emeritus at the Mayo Clinic College of Medicine recommends:

"Results of ESR and CRP testing should be interpreted in the context of the patient's typical values prior to the onset of illness, when available, and the patient's age."

The patient's healthy ESR and CRP are not usually known and must be learned from the lowest values seen during treatment. It is essential to understand that the population normal so ofter mentioned may be dangerously misleading in the on-going treatment of an individual.

The chart below shows that more than 84% of men and 66% of women have a normal ESR less than eight (8).
It is important to recognize that a person with a normal ESR of four (4) who is found to have an ESR of -- say -- 12 may have inflammation that needs more prednisone. Prolonged, unsupressed inflammation is damaging to the arteries.

esr-dispersion

CRP also has a distribution among the healthy population:
The values less than two or three are only measured by a high-sensitivity test.
These hsCRP values *may* be an indicator for heart condition.
With GCA, the values are likely to be greater than two or three.
See my chart below for an example of the relation between ESR and CRP.
gca/crpdistribution.gif

Both ESR and CRP increase somewhat with age and both are slightly higher for women.

esr-age

Looking at the lows on the graph below, my normal ESR appears to be between four and ten, perhaps six.
My normal CRP must be less than 2.9. (The lab usually reported "less than 2.9" -- its test limit.)
Laboratories tag test numbers to call the doctor's attention to abnormal values. For ESR, 20 is a common level to be tagged. It is not the number to use in treatment of an individual, whose normal may be significantly different.




to TOP OF THIS PAGE My history from March 2009 to April 2017 is summarized in the graph below:
My esr-crp-graph annotated-3


April 2016 update. On January 27-28, 2016 I had a 2-day emergency-ward hospitalization for profound weakness. I have no other word to describe my problem. On Thursday I had difficulty dressing in the morning and dressing for bed. I made it through the weekend but on Tuesday I put on my robe and my son took me to the emergency ward.

Many tests were made -- including blood, x-ray, CT-scan, echocardiograph, ECG -- but the cause of my being there was not determined. I did have atrial fibrillation, presumably started with this incident. I had no fever. Suggested diagnoses included: bronchiectasis and congestive heart failure. Xray showed the bronchiectasis, congestive heart failure seemed not the case. The hospital doctor apologized for giving me an oral antibiotic as a precaution "in case".

Later discussion with the cardiologist agreed that the cause of my emergency hospitalization was not determined.

However, the treatment included immediate "stress corticosteroid dosing with hydrocortisone." This seems to be a common emergency procedure. On release, I resumed my 6 mg prednisone schedule.

On February 8, twelve day from the hospital, my ESR was 40 and my CRP was 20 -- very high for me. Both have returned to my normal range after a few days of increased prednisone (10 mg quickly tapered to 6 mg). Perhaps the inflammation had nothing to do with GCA.

Isolated AFib incidents and temple inflammation coincided on April 22 and May 23. This raises the question: can GCA inflammation trigger atrial fibrillation?

(June 17, 2016 -- apart from three years of increasing leg weakness and uncertain sense of balance, I'm doing fine.)

End of August 2016 Uupdateto TOP OF THIS PAGE


The graph below shows the experience at the University of Iowa Hospital.
My prednisone schedule is superimposed.
Iowa experience

Each GCA / PMR patient differs, because of differences in them or differences in their treatment. Here are six examples of actual prednisone/esr/crp graphs:

sixgraphs-.gif

The Management of GCA with Prednisone

Table of Contents There is a controversy about the guides for management of prednisone.
Rheumatologists are rightly anxious to reduce the amount of prednisone.
Prednisone has serious side effects.
Opthalmologists are rightly anxious to prevent loss of vision, a too-common effect of GCA.
Dr. Hayreh summarizes this controversy in his articles and in this letter to me:

The cause of confusion about the role of ESR and CRP in the management of GCA is due to the following reasons:

Rheumatologists and ophthalmologists have a totally different perspective on management of GCA. For rheumatologists GCA is a disease with aches and pains and other systemic symptoms. For ophthalmologists GCA is a blinding disease. That determines marked difference in the management of GCA. Rheumatologists tend to mix PMR and GCA when managing them. However, PMR is a mild disease and there is no risk of blindness. In sharp contrast to that, the most important complications of GCA is blindness - thus it is a serious disease with devastating complication. That determines the difference in basic management of GCA. In my studies of about 300 patients with GCA, I found that the only way to manage GCA to prevent blindness is to regulate the therapy and management is by doing ESR and GCA regularly. Rheumatologists advocate that the therapy should be regulated by symptoms only, which I have found totally wrong and dangerous strategy. Symptoms are no guide to the activity of GCA. I have a large number of patients in whom ESR and CRP went sky high without recurrence of systemic symptoms. I have seen patients who were managed by rheumatologists with that outlook coming to me with loss of vision.

Thus, the only way to prevent blindness due to GCA is by ESR and CRP estimations.

S.S. Hayreh

Sohan Singh Hayreh, MD, MS, PhD, DSc, FRCS, FRCOphth (Hon)
Professor Emeritus of Ophthalmology & Director Ocular Vascular Clinic
Department of Ophthalmology & Visual Sciences
College of Medicine, University of Iowa

Avoiding flares by the slow reduction of prednisone may result in the use of less prednisone.
The slow reduction minimizes symptoms and leaves the blood tests as the best guide.

The Paradox: Flare First, Treatment After

Many articles on PMR and GCA make a statement such as this:

"A rise in ESR or CRP without clinical evidence of a flare does not justify a change of corticosteroid therapy."

"An isolated rise in ESR or CRP does not justify an increment in the prednisone dose."

But, in the same paragraph one may find, "a high relapse rate that may be caused by rapid reduction of prednisone leads to an increased cumulative dose and subsequent adverse events."

It is not a paradox that slow reduction uses less prednisone than a too-fast reduction. The flare let loose by the fast reduction forces the use of more prednisone.

Appendices

Table of Contents

Understanding Blood Tests - ESR and CRP

ESR and CRP are measures of inflammation. They support one another.
"ESR" Erythrocyte Sedimentation Rate. "CRP" C-Reactive Protein

These blood tests, along with symptoms, guide treatment.

For ESR the population healthy values, increasing with age from 55 to 90
average 6 to 11 for men and 9 to 11 for women
95% of healthy men are below 14 to 18. (ages 55 to 90)
95% of healthy women are below 21 to 23. (ages 55 to 90)

This is data from 3910 healthy Norwegian adults.

To determine YOUR healthy value look for low numbers as you go along in your treatment.
For ESR it will be somewhere between zero and 40, probably beteween zero and ten.

A graph of ESR and CRP against time is helpful -- just pencil and paper

My fancy graph was shown above.

See on the graph that my normal ESR seems to be about six -- something among the lowest values. The values above ten were certainly not normal for me. It is apparent that we (my doctor and I) were withdrawing the prednisone too fast.

At the early ESR of 15 I had a flare with two temporal arteries swollen that could have cost me some loss of vision if they had been optic-nerve arteries.

During the times of ESR numbers above ten, there were weak, fleeting head-pain symptoms -- symptoms that could be denied, but the numbers revealed the inflammation.

Inflammation of my leg arteries brought the day when the ESR and CRP both screamed "STOP" and I skidded to a stop on prednisone withdrawal, perhaps barely in time to save my legs from more serious artery inflammation. Such inflammation does the arteries no good.

Notice that the ESR and CRP may be raised by other inflammations, such as the 17 and 3.6 at a common cold.

It may also be worth noting that keeping a careful record may reveal a laboratory error. Errors do happen and could cause an error in treatment.


GCA Symptoms reported in the literature

Table of Contents
  1. Weight loss -- (I went from 180 to 172 pounds during months before GCA diagnosis; lost another 8 or 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 but has been reported)
  6. Headaches: throbbing, sharp or dull headaches may bring you to your doctor.
  7. Tenderness near the temples: Noticed most when wearing glasses, combing hair or lying on a pillow.
  8. Fever: Spiking temperature and chills will bring you to the doctor. Or low-grade fever
  9. Loss of appetite
  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)
  14. Blindness: Loss of vision is sudden, painless and usually permanent.

Symptoms of Cortisol Insufficiency (Addisonian)

Table of Contents

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 apparently 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 fluid and was hospitalized for two nights. Countless tests confirmed my age of 88 but nothing else to worry about. These tests were thought necessary to rule out other causes of my collapse (but Medicare refused to pay for them).

I experienced the warnings marked **.

  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


Bibliography

Table of Contents

There are many reliable sources of information. GCA is complex and there some disagreements. The internet is easily searched for "gca temporal arteritis".

I have an 18 minute YouTube video here: Giant-Cell Arteritis: One Man's Experience

I publish a 45-page book on Amazon: Giant-Cell Arteritis: One Man's Experience.

Major clinics offer articles on GCA

Mayo Clinic       Cleveland Clinic       Johns Hopkins

There are two chapter in a free book that provide much detailed information about GCA, intended for reading by specialists:   These may be downloaded in .pdf Acrobat format and printed:

Ch. 14  "Diagnosis and Treatment of Giant Cell Arteritis (Temporal Arteritis)"

Regarding attacks of the arteritis on other arteries than the head:  

Ch. 15  "Extra-Cranial Manifestations of Giant Cell Arteritis"

Giant Cell Arteritis by: Sohan Singh Hayreh, MD, MS, PhD, DSc, FRCS, FRCOphth; Professor Emeritus of Ophthalmology
Opthalmology Department of the University of Iowa hospital
and: Ischemic optic neuropathies -- where are we now? have been referenced in the text above. These articles by Dr. Hayreh are must reading for anyone with GCA. They give a perspective on treatment that differs from the standard practice of rheumatologists, who are rightly worried about the side effects of prednisone.

Support Groups

There are several support groups on the internet for GCA and PMR.
These groups as well as many articles on GCA and PMR may by found by searching the internet.
Participation in such a group may prove valuable, both for medical information and for friendly support.
This is a friendly and helpful group in the United Kingdom: http://pmrandgca.myfunforum.org
with more than 50 very smart and PMR-experienced members:
The United Kingdom has many local support groups this forum can help you contact.. This is a valuable US support group: https://groups.yahoo.com/neo/groups/pmrusa/info
A member of that group, JamesWRupp has collected countless references and much information about GCA. Join one or more of these groups and exchange experiences.
to TOP OF THIS PAGE       to HOME PAGE
sc