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Several diagnostic tests were performed during Pearl’s illness – those initially were done to help diagnose GCA. Some were done during the course of her illness to evaluate conditions that were suspected or had occurred. Several were repeated on a periodic basis to monitor status.
Diagnosing GCA
X-ray - An X-ray was taken of her sinuses a month before diagnosis. She had sinus involvement for a month that hadn’t responded to three courses of antibiotics treatment. It confirmed on-going sinus inflammation. The sinuses eventually cleared after prednisone was started for treatment of GCA a month later.
Doppler scan – A scan of her carotid arteries was done in the hospital ER three days after suspicion of GCA. Blockage, if present, could be a possible explanation of loss of vision in her eye. The carotids were clear, supporting the suspicion of GCA.
Biopsy - Two days after diagnosis of GCA a temporal artery biopsy was performed on her right side. A short segment (about an inch) of artery is removed and examined for presence of giant cells in the wall. This artery is visible near the surface – often when GCA is present it will appear to be bunched up under the skin because the walls are thickened and require more space than normal. The biopsy was positive indicating the presence of giant cells. This is the typical method used to confirm GCA. If giant cells are not found the patient is in a dilemma. Doctors often will continue to treat for GCA if other symptoms are present but some doctors will cease treatment because GCA hasn’t been proven by the biopsy. Pearl’s temperature was 95 degrees at the time of the biopsy. This was found to be due to underactive thyroid metabolism which corrected itself a few weeks after prednisone was started.
CT scan - A liver biopsy and CT scan of the abdomen were done at the insistence of the FPP because alkaline phosphatase had been found elevated in a blood test one month before diagnosis of GCA. This was unnecessary because once GCA was identified and alk phos returned to normal after start of treatment it was known to other doctors that alk phos was elevated earlier because of the GCA. The tracer used in CT scans is iodine based, which is an allergen for Pearl. This required administration of a high dose of prednisone prior to the scan. Another undesirable aspect of an unnecessary CT scan is the high amount of radiation given off in the process – about 200 times that of a typical chest xray. Pearl had several more CT scans for various reasons during her illness.
Diagnosing unusual conditions
Thyroid - Because of weakness, soreness in knees and legs and legs buckling, the Rheumatologist checked Pearl’s blood for hypothyroidism – underactive thyroid. It indicated low in a test done two weeks after diagnosis but was found normal in a repeat test two weeks later. Another symptom of low thyroid metabolism is low body temperature as Pearl experienced during the temporal artery biopsy a few weeks earlier.
Doppler scan - Pulse in foot arteries was suspiciously low six months after diagnosis. Pearl was referred to a Vascular Surgeon who performed a doppler scan of both legs. The doppler scan measures pressure at various parts of the leg and ankle and compares that to the brachial pressure – that of the upper arm, typical of usual blood pressure readings. An ankle/ brachial index is derived for each section of leg or ankle by dividing the leg reading by the brachial reading. Before results were obtained by Pearl we rushed her to the hospital ER on an emergency basis to check out major circulation problems in both legs.
Arteriogram - The hospital staff repeated a doppler scan, kept her in the hospital and followed up two days later with an arteriogram of the legs. The tests showed major blockages (ischemias) and narrowed areas (stenoses) in major leg arteries of both legs. Fortunately they used a high dose of corticosteroids to prepare her for the arteriogram since iodine dye is used and Pearl is allergic to iodine. The corticosteroids in retrospect opened up the arteries sufficient for the crisis to pass.
The diagnosis at the time was atherosclerosis – this turned out to be erroneous. The cause later was found to be GCA inflammation caused by insufficient prednisone to manage it. Prednisone dosage was 8 mg/day at the time of hospitalization; it was reduced to 7 mg/day weeks later, then 6, causing more damage to the legs and feet. Sed rate became significantly elevated finally – about three months after leg symptoms started. Prednisone was restored at that time to a proper managing dose of 15 mg/day.
Doppler scans were repeated by the Vascular Surgeon about every two years to monitor status of the legs.
An angiogram of the heart and upper abdomen was done a few years later by the Cardiologist in February, 2003. All heart and abdomen arteries were found to be completely clear of atherosclerotic plaque.
HBO - An xray was taken of Pearl’s right foot and ankle to evaluate a bruise that had formed. The bruise progressed to an ulcer which required hyperbaric oxygen (HBO) treatments to heal. These were done daily for ten sessions. A group of patients and observers entered a large tank and an environment of pure oxygen was applied at elevated pressure for about two hours. The same process is used to cure deep sea divers of the "bends" which can occur if they rise to the surface too fast. This was the only time she needed HBO to assist in healing wounds. She visited the Wound Care Clinic many times later for special care of wounds that developed over the years of illness. With the later wounds her skin had sufficient oxygenation to heal on their own.
EKG, Echocardiogram, Stress Tests - Before Pearl could qualify for the HBO treatment a cardiologist had to evaluate her condition to see if heart was capable of withstanding the treatment. An EKG, Echocardiogram, and Dual Isotope Stress Test were conducted by the Cardiologist. Pearl performed well in these tests and was approved for the HBO treatment. The Cardiologist would repeat these tests on Pearl annually, always with satisfactory results. EKGs were done often during her illness by others also and always indicated good function.
Berkeley Heart Panel Test – This test was done by the Cardiologist because of suspicion the leg arteries were not atherosclerotic. It consisted of sending a blood specimen to the University of California at Berkeley where several parameters affecting the heart are evaluated. The result was favorable for Pearl, indicating she had no evidence of heart disease – her arteries were clear, so leg artery blockages were assessed by the Cardiologist to be due to GCA type arteritic inflammation, not atherosclerosis.
MRI – Pearl’s right eye socket was swollen so the eye was protruding about 2 mm beyond the other. An Open MRI was done of the orbits and the brain. Findings were normal for the socket. Gadopentatate was used as the tracer. Several MRIs were done during the course of her illness both at local hospitals and at the remote Clinic – some of these used gadolinium as the tracer. Pearl seemed to tolerate the tracers well.
Lipids – A lipid test was prescribed by the Rheumatologist. Results showed Pearl continued to be in the lowest risk group for atherosclerosis and heart disease. Lipid tests were done every year during her illness with the same findings – high HDL, low total and LDL and low ratio.
Interluken (IL-6) Test – This test is intended to check for GCA inflammation though it isn’t commonly used to manage GCA. Results showed inflammation was under control at the time with a reading of < 3.1; < 6.1 is normal. C-rP is reported to closely resemble IL-6 in reflecting changes in GCA inflammation within 24 hours. Sed rate lags changes in inflammation by about four or five days.
Chromosome analysis and FISH Test – These were done looking for a specific gene by the Hematologist at the recommendation of the remote Clinic Hematologist. The Chromosome analysis had been done in the bone marrow test at the time of GCA diagnosis so this was a repeat. Results were normal. Bone iron deficiency was demonstrated in the Chromosome test; serum levels were normal.
Adrenal, Pituitary Glands Function – It appeared that Pearl could taper off of prednisone in mid-2002 so she engaged an Endocrinologist to test her cortisol producing capabilities, namely the functioning of the hypothalamic-pituitary-adrenocortical axis. The Pituitary gland was found to be working properly. ACTH reading was 15 (over 9 is normal) but the Adrenal gland output was low. Dr expected the Adrenal gland output would increase as prednisone was tapered. Dr said Pearl should be careful in tapering because inadequate cortisol can cause coma and death. He prescribed a tapering plan which would have her off of corticosteroids in two months. She had a flare the following week and was never able to get that low on prednisone again.
Allergen Test – The Allergist performed a scratch test for allergens – findings were pine trees and Johnson grass, both of little consequence for Pearl. Results likely were affected by high prednisone she was taking at the time.
MRA – The Clinic did an MRA evaluation of her heart arteries in November, 2001, and found all were normal. Subsequently MRAs were done in July, 2005, to evaluate her thoracic aorta which was found negative for aneurysms, and her lower extremities in May of 2006. In that evaluation mild narrowing of the celiac artery, stenosis and ischemia were found in some leg arteries and two renal arteries. Other renal arteries were wide open as was at least one leg artery.
PET Scan – This was done by the Clinic in October, 2002, viewing the chest, abdomen and pelvis. No active vasculitis was found. It was unfortunate that the legs weren’t included in this evaluation since that was where all the inflammation historically had been focussed and suspected of still being active.
Upper Endoscopy and biopsy – The Gastroenterologist performed this evaluation in December, 2002. Barrett’s Metaplasia and a sliding hiatal hernia were noted as findings. The procedure was repeated in 2004 and 2006 and the Barrett’s Metaplasia had resolved and wasn’t present in both evaluations. The hernia required no follow-up.
Bone Density Scan – This was done by the Rheumatologist about every two years. The initial scan showed significant bone loss in spine and hips causing a diagnosis of osteoporosis but the loss reduced in subsequent scans to a point where the diagnosis was osteopenia – borderline between normal and osteoporosis. She was taking a bisphosphonate plus calcium supplements throughout her illness. It is l likely the high doses of prednisone initially after diagnosis caused the initial deteriorated condition. As prednisone was decreased the condition of her bones improved.
Conductivity Test – The Neurologist ordered this to evaluate cause of pain pulses that ran through Pearl’s right arm. Results were inconclusive.
Homocysteines – The Neurologist measured these in January, 2004. They were slightly elevated at 12.3 – the desired value is < 10. No follow-up was needed.
FA - Fluorescein angiography (fluorescein - the type of dye that is used; angiogram - a study of the blood vessels) is an extremely valuable test that provides information about the circulatory system and the condition of the back of the eye. The Ophthalmologist viewed Pearl’s retinas using this technique to monitor eye status at every visit starting in 2004. In most instances it showed good blood flow to the retina so we grew concerned about overtesting and stretched time between app’ts to more than the three months he wanted. Besides unnecessary expense it involved intravenous dye and skin bruising.
Ultrasound of aorta – This was prescribed by the Rheumatologist in 2004 to observe for aortic aneurysms. None was found. Unfortunately it was intended that both the thoracic (upper) aorta and abdominal aorta be checked but only the latter was viewed. The Rheumatologist used MRA in the future to check the aorta.
IgG – An Anticardiolipin Antibody (IgG) blood test was specified by the Rheumatologist to check for GCA/PMR activity. Results were negative indicating GCA/PMR were being managed at that time – January, 2006.
Chromosome, JAK 2, Bone Marrow – These tests were prescribed by the Clinic Rheumatologist and Clinic Hematologist in May, 2006. Results were normal except for low iron stores. Elevated platelets were confirmed to be reactive (reactive thrombocytosis) - caused by GCA. Before this test the Clinic Rheumatologist was suspicious that Pearl’s elevated platelets were due to primary thrombocytosis.
B12, folate, cryoglobulins, cryofibrinogen, monoclonal protein – These were measured by the Clinic Hematologist in June, 2006 and found to be negative or normal. There were no hematological concerns.
64 slice heart and aorta CT Scan – This was done by the Cardiologist in May, 2007. No evidence was found of aneurysms, plaque or vascular disease in the heart arteries and aorta.
CT Scan and X-rays of chest and abdomen – These were the final tests done on Pearl on the morning of admittance to the hospital ER the day before death on 10/19/07. They showed no signs of perforation or infection in her chest and abdomen vessels. Everything appeared to be normal.
See Appendix 8 for a chronology of tests and surgical procedures that were performed since the start of GCA/PMR in 1998.