“He had red streaks on his head which were painful and prevented him wearing his hat….The red streaks proved, on examination, to be his temporal arteries, which were on both sides found to be inflamed and swollen. The streaks extended from the temporal region almost to the middle of the scalp and several branches of each artery could be distinctly traced. The conditions were nearly symmetrical. During the first week that he was under my observation, pulsation was freely detected in the affected vessels, but it finally ceased; the redness then subsided, and the vessels were left impervious cords.”
Mr. Rumbold lived several years more, without vision loss or, apparently, other sequelae of GCA.
The contemporary management of GCA involves pharmacologic difficulties beyond the potential toxicities of CS. Should low-dose aspirin be used? Two retrospective studies found a protective effect against so-called cranial ischemic events (vision loss and stroke), two others did not.13-16 Is prophylaxis against pneumocystis pneumonia (PCP) warranted? Is the risk of PCP in fact increased in patients with GCA on monotherapy with high-dose CS? What is the risk–benefit ratio of bisphosphonates for the reduction of fracture risk? It is conceivable that a patient with newly diagnosed GCA could leave the office with prescriptions for high-dose prednisone, low-dose aspirin, a proton pump inhibitor (because of the increased risk of gastrointestinal bleeding in an older adult on concurrent CS and aspirin), trimethoprim-sulfamethoxazole (provided there is no sulfa allergy), and a bisphosphonate. For whatever it’s worth, in such a patient, I currently do not routinely deploy low-dose aspririn or pneumocystis pneumonia prophylaxis, as I am unpersuaded as to their value by either the published studies or clinical experience, but I do aggressively use bisphosphonates, which, despite their recently tarnished reputation, have been of clear value in decreasing fracture risk from CS.
And the management of GCA beyond its initial treatment carries a further set of difficulties. How quickly should CS be tapered, and how long should they be used? As is the situation with the taper of CS in PMR, a biomarker that legitimately gauges disease activity in GCA would be invaluable. For the present, the interpretation of recurring headache or constitutional symptoms, and of the significance of rises in the erythrocyte sedimentation rate (ESR) and the c-reactive protein (CRP), are exercises in clinical judgment. Whether such increases in symptoms or signs are clinically consequential also bedevils formal studies of the treatment of GCA. For example, does a minor headache or a 10-mm bump in the ESR constitute a bona fide “flare” of GCA, thus to be construed as an inadequacy or even failure of a given treatment? In practice, the slavish adjustment of the CS dose against minor rises in the acute phase reactants, without consideration of the patient’s clinical status, is a recipe for CS-related toxicities. Clinical experience suggests that, on the whole, there is a tendency for overtreatment of GCA with CS, which can lead to more morbidity than the disease itself.
