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GCA Can Lead to Blindness, Legal Liability: The risks of giant cell arteritis

By Gene Moen

What do a tender area of the temple and jaw pain have in common? In combination, they can be key symptoms of a condition called giant cell arteritis (GCA), which could lead to permanent vision impairment if not promptly treated with high-dose steroids. GCA can also result in medical negligence claims because the outcome – sometimes total bilateral blindness – is so devastating. Blindness caused by GCA is almost always non-reversible.

GCA, which used to be called temporal arteritis or cranial arteritis, is inflammation of arteries of the head and neck. First described pathologically in 1932, GCA is most often confined to the cranial arteries, but can occasionally affect arteries in other parts of the body. In this article, we will focus only on GCA that impacts the temporal artery.

Although the incidence of GCA across the population is relatively small (fewer than 50 per 100,000), its rate is much higher in those over the age of 70. With an aging population, it is likely that more cases of GCA and GCA-related blindness will occur. Already, GCA is among the most common conditions leading to prolonged corticosteroid therapy in the elderly.

There is a relationship between GCA and a clinical condition called polymyalgia rheumatica (PMR), an inflammatory rheumatoid process that can cause joint stiffness throughout the body. The causes of GCA and PMR are not known at this time, but they usually affect those over 60, and, more frequently, those over 70. How and why there is a relationship between these two conditions is not known with certainty, but their co-occurrence is 38 times more likely than they would be if they were independent factors.

From a legal perspective, the importance of this relationship is that a patient who has a history of PMR may present with non-specific symptoms that would not lead a doctor, who is unaware of the relationship, to consider GCA as the cause of those symptoms.

The standard of care requires family practice physicians and “front-line” physicians to know about this relationship. This emphasizes the importance of good history-taking. A patient with a remote history of PMR may not mention this to a physician if not asked.

Let’s look at a typical case involving GCA. A 72-year-old patient sees his primary care physician with symptoms of a sudden headache and pain in his temple. If the patient had a history of PMR, then those non-specific symptoms should lead to GCA being on the differential diagnosis, and further testing should be done. A blood test to measure the erythrocyte sedimentation rate (sed rate) can be done on an urgent basis. If it is elevated (above 50), then a presumptive diagnosis of GCA should be made. The reason is that the sed rate is usually elevated when there is an inflammatory process or infection in the body. It would be unusual for a patient to have GCA without an elevated sed rate.

If the patient has other potential GCA symptoms, such as jaw pain when chewing (jaw claudication), then GCA would be higher on the differentia, and many clinicians believe that if GCA is high on the differential, prompt administration of high-dose steroids is required without waiting for lab results to come in. The majority of GCA liability cases arise from delays, sometimes as short as several hours or a few days, in administering steroids.

But even with typical signs and symptoms of GCA, as well as a high sed rate, the final diagnosis of GCA is not made until there is a temporal artery biopsy. Lawsuits involving a delay in treating GCA often occur because a physician suspects GCA and schedules a biopsy that will not occur for several days. Waiting for a definitive diagnosis, and not treating GCA in the meantime with high-dose steroids, can lead to blindness in one or both eyes before the biopsy results are available.

The exact cause of blindness from GCA is very complicated and somewhat theoretical. However, the idea is that inflammation leads to changes in the walls of the arteries, which, in turn, can result in blockage or ischemia. The temporal artery is the source of the blood supply to the optic nerves, and when that blood supply is disrupted it leads to arteritic anterior ischemic optic neuropathy (AAION). The vision loss caused by AAION can range from mild (and sometimes temporary) visual disturbances to blindness in one or both eyes. Approximately 15% of patients with diagnosed GCA end up with permanent vision loss.

Claims involving a delay in treating GCA are not confined to primary care physicians. If a patient has symptoms of GCA and sees his or her ophthalmologist because of development of visual problems, then the GCA diagnosis must lead to prompt administration of steroids. GCA is considered by ophthalmologists to be a true emergency. Since most ophthalmologists do not have steroid medications in their office, that often leads to a referral to the patient’s primary care physician.

Ophthalmic Mutual Insurance Company (OMIC), the largest malpractice carrier for ophthalmologists, puts out risk management bulletins to its insureds. A 2015 bulletin was headed “Giant cell arteritis claims are costly and difficult to defend.” It discussed a number of closed claims involving ophthalmologists who (1) failed to take an adequate history, (2) failed to appreciate the symptoms that could be suggestive of GCA, or (3) did not communicate their concerns about GCA to the patient’s primary care physician or to the patient. These all led to a delay in administration of high-dose corticosteroids and resultant permanent vision loss.

Lack of communications then often becomes the basis for a claim. An ophthalmologist simply telling a patient to make an appointment with their primary care doctor can lead to a several-day delay, during which vision loss can suddenly occur. Sending a fax to the doctor may not result in prompt action; a telephone call and immediate referral is needed to ensure prompt steroid administration.

Many ophthalmologists believe that the standard of care requires that a patient with a high suspicion of GCA be immediately sent to the nearest emergency department for IV steroid administration. Options also include sending the patient directly to a nearby pharmacy where the doctor calls in a prescription for oral steroids.

Two of the cases we have handled involved lack of knowledge by ophthalmology front-desk personnel in getting the patient in as quickly as possible. In one of them, the patient told the receptionist on a Friday afternoon that she had a sudden loss of vision in one eye that day. The patient was told that, since she already had an appointment on Monday, she could see the ophthalmologist then. On Sunday, she lost vision in her other eye. A basis for liability was lack of staff training to ensure emergency appointments.

In summary, for the plaintiff attorney reviewing a case involving GCA-related blindness, the key questions to consider are:

Common defenses in GCA cases include failure of the patient to adequately describe his or her symptoms, or failure to mention a prior history of PMR. Another defense is that the patient failed to act promptly when referred to someone who can administer high-dose steroids.

An ophthalmologist may also be reluctant to directly order steroid medications if the patient has other medical issues, such as diabetes. If referred by an ophthalmologist to their primary care doctor to order the steroids, the patient may wait for a call from their doctor or the doctor’s staff to schedule an appointment. This emphasizes the need for a referring physician to clearly explain to the patient the importance of administration of steroids to avoid vision loss. The referring physician should also promptly call the primary care physician to discuss the patient’s diagnosis and required treatment.

There are several options available to an ophthalmologist when GCA is diagnosed or is high on the differential diagnosis, but they all come down to one simple rule: GCA is an ophthalmology emergency that requires very specific and prompt treatment to avoid blindness.

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