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. 2013 Sep 20;27(12):1418–1419. doi: 10.1038/eye.2013.208

Giant cell arteritis with normal ESR and/or CRP is rare, but not unique!

A Grzybowski 1,2,*, A Justynska 3
PMCID: PMC3869507  PMID: 24051406

Sir,

We read the paper by Levy et al1 with interest, however, we believe that it contains several weaknesses which should be discussed.

As it is well known the diagnosis of GCA is based on the combination of clinical symptoms, laboratory test (CRP and ESR), and histopathology.2 The treatment must be started immediately, thus the temporal artery biopsy result usually confirms presumed diagnosis made on the basis of clinical picture and laboratory tests.

It is well known that laboratory tests in GCA have some limited sensitivity: CRP 86.9% and ESR 84.1%.2 Both combined gave specificity 97%, but it was shown and discussed in several studies that they might be normal in GCA:2, 3, 4, 5 ESR was normal in 5–30% of patients with GCA.3 The authors1 inappropriately stated that ‘including their case there are only three published cases of isolated CRP-negative GCA and only two cases of simultaneous ESR and CRP negativity' as they did not notice nor analyze many similar cases (Table 1). Noteworthy, it was postulated that elevated ESR with normal CRP might occur even in 1.7% of GCA patients,3 and that these markers can be normal at the early stage of the disease.4

Table 1. Some publications reporting normal ESR and/or CRP.

References Normal ESR (number of cases/percentage of cases) Normal CRP
Raja et al6 1 1
Rahman and Rahman7 5–30% —
Ellis and Ralston8 22.5% —
Kermani et al9 18 (4%) 18 (4%)
Man and Dayan10 1 1
Weintraub11 1 —
Wong and Korn12 36 —
Parikh et al13 1 (0.8%) 2 (1.7%)
Laria et al14 4–15% 0.8%
Yoeruek et al15 1 1
Myklebust and Gran16 1.2% 1.2%
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Having in mind problems with ESR and CRP in GCA, the role of clinical picture of the disease seems to be very important. They include usually headache, tenderness of the scalp, jaw claudication and some systemic symptoms, including malaise, fever, anorexia, and weight loss.5 The symptoms are based on ischemia and/or inflammation. Thus, it is hard to understand why in the discussed case none of the clinicial symptomes was presented.

Concluding, we agree that diagnosis of GCA might be problematic, but it must be based on clinical picture and laboratory tests, including ESR and CRP, and confirmed by temporal artery biopsy. GCA with normal ESR and/or normal CRP level is rare, but many cases of this form of disease were already described.

The authors declare no conflict of interest.

References

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