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Vasculitis in the Very Old: The Eyes Have It

— Vision loss a major complication; mortality also high in the over-85 group

Last Updated March 8, 2019
MedpageToday

Among patients diagnosed with giant cell arteritis (GCA) -- a potentially severe vasculitis afflicting the elderly -- those 85 and older at the time of diagnosis typically had more severe disease manifestations and greater mortality, French researchers reported.

Compared with those diagnosed before age 85 on a multivariate analysis, the over-85 group were twice as likely to have permanent visual loss (OR 2.24, 95% CI 1.29-3.81, P=0.003), according to Eric Liozon, MD, of University Hospital of Limoges, and colleagues.

The older group was also more likely to die during treatment (OR 2.61, 95% CI 1.45-4.65, P=0.001), the researchers reported online in .

Giant cell arteritis is characterized by inflammation of the large and medium-sized arteries and symptoms such as headache, scalp tenderness, and jaw claudication. Some patients also report symptoms of polymyalgia rheumatica. The most worrisome manifestation is irreversible vision loss.

found the highest incidence of GCA in women ages 70 to 79, and with the aging of the population, the rates of disease are expected to rise among the oldest. The mainstay of treatment has long been high doses of glucocorticoids, and the burden of adverse effects with these drugs is high in the elderly. However, little is known about the pattern of disease, morbidity, and mortality associated with GCA among the oldest patients.

Therefore, to examine these concerns, Liozon and colleagues reviewed the records of 865 patients seen at two French centers who had been diagnosed from 1976 to 2017. A total of 87 were 85 or older at the time of diagnosis.

Most of the patients were being treated with standardized protocols of prednisone. Those without ischemic disease manifestations initiated treatment with 0.6-0.8 mg/kg/day followed by tapering, while those with ischemic involvement began treatment with dosages of 0.9-1.1 mg/kg/day, often preceded by pulses of methylprednisolone, and subsequently tapered.

Among the over-85 subgroup, the mean age was 87.6, and most were women. Compared with the younger patients, the oldest group had more comorbidities and cardiovascular risk factors but less often reported having polymyalgia rheumatica and arthritis.

In the overall cohort, risk factors for developing permanent visual loss included age 85 or older, transient ischemic visual disturbances, and positive temporal artery biopsy. Diagnostic delay of more than 35 days and the presence of fever were associated with a lower risk of vision loss.

The finding of a negative association with diagnostic delay was surprising, the researchers said: "The different clinical patterns of older patients and younger patients and more expeditious diagnostic workups in complicated cases may explain this puzzling finding, at least in part," the team observed.

On univariate analyses comparing the older and younger subgroups of the cohort, the older group had shorter follow-up and fewer relapses, and were less dependent on prednisone but more often had infections.

On the multivariate analysis, being 85 or older was associated with increased risks of permanent vision loss and death but lower rates of steroid dependence (OR 0.48, 95% CI 0.23-0.94, P=0.038) and relapse (OR 0.52, 95% CI 0.31-0.87, P=0.015).

In a further regression analysis looking at factors associated with overall mortality, risk of death in the over-85 subgroup was associated with the presence of an abnormal temporal artery (HR 4.51, 95% CI 1.92-10.6, P<0.001) and a higher mean prednisone starting dose (HR 8.46, 95% CI 2.11-33.92, P<0.01), but mortality risk declined with stopping prednisone (HR 0.23, 95% CI 0.12-0.44, P<0.0001).

Causes of death occurring within 2 years of GCA diagnosis more often in the over-85 subgroup compared with the younger group included the following:

  • Infection, 11.5% vs 1.4% (P<0.001)
  • Cardiovascular disease, 8.1% vs 2.2% (P=0.001)
  • Malignancy, 5.8% vs 1% (P<0.001)
  • Severely impaired health/bedridden, 9.2% vs 0.1% (P<0.001)

However, only one patient in the oldest group died of GCA complications compared with 10 in the younger group.

Among the entire cohort of 865 patients, overall mortality was similar to what was seen in the surrounding regions of France. In the oldest group, survival rates at years 1, 2, and 3 were 76%, 66%, and 63%, respectively. Previous studies have demonstrated increased mortality in the first year or two after GCA diagnosis, which is the time when most patients are taking glucocorticoids.

"Very elderly patients who overcome the first year following GCA diagnosis might have above average health state and higher longevity than the age-matched population," Liozon and colleagues wrote. "Our patients might also have received closer monitoring and better overall care than the background population."

A limitation of the study, the team said, was the retrospective design.

Disclosures

The authors reported no financial conflicts.

Primary Source

Seminars in Arthritis & Rheumatism

Liozon E, et al "Features and prognosis of giant cell arteritis in patients over 85 years of age: a case-control study" Semin Arth Rheum 2019;doi:10.1016/semarthrit.2019.02.011.