Giant cell arteritis causing aortic dissection and acute hypertensionBMJ 2008; 337 doi: https://doi.org/10.1136/bmj.39503.769225.BE (Published 04 July 2008) Cite this as: BMJ 2008;337:a426
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the next challenge is to establish the prevalence of extracranial vasculitis in polymyalgia rheumatica
Anecdotal reports(1)(2)(3) may underestimate the prevalence of
extracranial vasculitis in patients with polymyalgia rheumatica(PMR) who
do not have clinical or histological evidence of concurrent or remote
temporal arteritis. In one study, involving 74 patients with
subclavian/axillary giant cell arteritis(GCA)diagnosed by angiography, PMR
was documented in 28, and headache and/or jaw claudication in only eleven.
There were positive findings on temporal artery biopsy(TAB) in 33 of the
57 patients in whom this procedure was undertaken, but it is not clear how
many of the 24 with negative histology belonged to the PMR subgroup. What
the study did show, however, was that, even if the eleven subjects with
headache and/or jaw claudication were included in the subgroup of 24 with
negative TAB rather than in the subgroup of 33 with positive TAB, there
would remain at least thirteen TAB-negative subjects with large-vessel
GCA(4). The resulting prevalence of TAB-negative cases would be of the
order of 17.55 in GCA patients with large-vessel vasculitis. The true
prevalence of extracranial GCA in patients with the exclusively PMR
subtype of GCA may yet emerge from prospective studies of the type
exemplified by anecdotal reports or pilot studies where temporal arteritis
is ruled out by TAB and extracranial vasculitis is validated either by
magnetic resonance imaging(5) or by positron emission tomography(6). In
the latter study evidence of extracranial vasculitis was found in 31% of
35 patients with isolated PMR
(1) Smulders YM., Verhagen DWM
Giant cell arteritis causing aortic dissection and acute hypertension
British Medical Journal 2008:337:1052-4
(2) Honing ML., Bredie SJ., Smulders YM
Aortic involvement in patients with temporal arteritis and polymyalgia
Ned. Tijdschr Geneeskd 2005:149:1947-53
(3) Ninet JP., Bachet P., Dumontet CM et al
Subclavian and axillary involvement in temporal arteritis and polymyalgia
American Journal of Medicine 1990:88:13-20
(4) Brack A., Martinez-Tabaoda V., Stanson A., Goronzy JJ., Weyand CM
Disease pattern in cranial and large-vessel giant cell arteritis
Arthritis and Rheumatism 1999:42:311-7
(5) Marzo-Ortega H., McGonagle D., O'Connor P., Pease C., Emery P
Subclinical vasculitis in polymyalgia rheumatica
Annals of the Rheumatic Diseases 2001:60:1058-9
(6) Blockmans D., De Ceuninck L., Vanderrschueren S et al
Repetitive 18-fluorodeoxyglucose positron emission tomography in isolated
polymyalgia rheumatica: a prospective study in 35 patients
Competing interests: No competing interests
Dr Smulders and Dr Verhagen report the case of a patient with aortic
dissection complicated by renal ischemia with accelerated hypertension,
hypokalaemia and hyponatremia . These findings initially evoked primary
hyperaldosteronism to the authors, until the plasma renin-aldosterone
profile proved them wrong.
However, hyponatremia in association with hypertension and
hypokalaemia is highly suggestive of secondary hyperaldosteronism due to
renal ischemia . By contrast, primary hyperaldosteronism typically
induces mild hypernatremia, because of the lowered antidiuretic hormone
release related to hypervolemia .
Moreover, acute blood pressure rise over a stable baseline was not
suggestive of primary hyperaldosteronism, which is a progressive disease.
On the contrary, accelerated hypertension is a consensual indication to
screen for renovascular hypertension .
Faced with the revelatory pattern of accelerated hypertension with
hyponatremia and hypokalaemia, the authors might have suspected acute
renal ischemia due to critical renal artery stenosis, renal artery
embolism, renal artery dissection, or aortic dissection. Primary
hyperaldosteronism was an unlikely hypothesis in this setting. Therefore,
urgent renal artery imaging might have been performed right away, rather
than plasma renin and aldosterone assays.
1. Smulders YM, Verhagen DWM . Giant cell arteritis causing aortic
dissection and acute hypertension. BMJ 2008;337:a426.
2. Agarwal M, Lynn KL, Richards AM, Nicholls MG. Hyponatremic-
hypertensive syndrome with renal ischemia: an underrecognized disorder.
3. Gregoire JR. Adjustment of the osmostat in primary aldosteronism.
Mayo Clin Proc 1994;69:1108-10.
4. Hirsch AT, Haskal ZJ, Hertzer NR, Bakal CW, Creager MA, Halperin
JL, et al. ACC/AHA 2005 Practice Guidelines for the management of patients
with peripheral arterial disease (lower extremity, renal, mesenteric, and
abdominal aortic). Circulation 2006;113:e463-654.
Competing interests: No competing interests