High-resolution MRI in giant cell arteritis with multiple inflammatory stenoses in both calves

T. A. Bley, K. Warnatz1, O. Wieben, M. Uhl, C. Scholz1, P. Vaith1, H. H. Peter1 and M. Langer

Department of Diagnostic Radiology and 1 Department of Clinical Immunology and Rheumatology, University of Freiburg, Freiburg, Germany

Correspondence to: T. A. Bley, Department of Diagnostic Radiology, University Hospital Freiburg, Hugstetter Strasse 55, 79106 Freiburg, Germany. E-mail: bley{at}mrs1.ukl.uni-freiburg.de

SIR, giant cell arteritis (GCA) commonly involves the cranial arteries in a segmental pattern [1–3]. In the majority of cases, the affection of the temporal arteries can be demonstrated by duplex ultrasonography [4] and is confirmed by biopsy. In a study of 248 patients with proven GCA, however, 23 had a widespread arteritis, and three of these 23 patients presented solely with symptoms due to vasculitis of the lower extremities [5]. Here we present the capacity of high-resolution MRI to depict the unusual vascular involvement in a 65-yr-old man with proven GCA.

He presented with increasing claudication of both legs for the previous 3 months. The medical history was inconspicuous, except for a well-controlled allergic bronchial asthma. No cardiovascular risk factors, such as hypertension, hyperlipidaemia, diabetes and smoking, were present. Just before admission, new onset of a bilateral headache had been interpreted as sinusitis. Additionally, he reported increasing night sweat during the last 3 months. The initial laboratory findings revealed a severe inflammatory response [ESR 85 mm/1st h, CRP 131 mg/l (normal value <5)] but otherwise normal results for the differential blood count and renal and liver function. ANA, ANCA and rheumatoid factor were undetectable. In the physical examination, both dorsalis pedis arteries were impalpable, while the superficial temporal arteries were hard and tender to palpation. Chest X-ray and ultrasound of the abdomen showed no signs of underlying malignant disease. A digital substraction angiography (DSA) was performed, and showed multisegmental stenoses and occlusions of the lower limb arteries and the formation of multiple collaterals (Fig. 1A). High-resolution MRI of the superficial temporal artery (Fig. 1B) and right leg (Fig. 1D) revealed inflammatory changes of the vessel walls, such as thickening and contrast enhancement, underlying the stenoses and obstructions. Suspecting GCA, a biopsy of the right temporal artery was performed. The histology demonstrated a severe mononuclear infiltration of the arterial wall compatible with GCA. The patient was immediately started on a high dose of steroids (1 mg/kg body weight) and aspirin 100 mg once daily. Due to the systemic involvement, empirical treatment with five pulses of monthly intravenous cyclophosphamide was added to the therapy and switched thereafter to oral methotrexate (15 mg/wk). The steroids were gradually tapered to 5 mg/day according to clinical and serological assessment. The initial headache improved within 24 h and the pain-free walking distance increased within 9 months from 25 to 400 m. Angiological re-evaluation after 6 months showed a still decreased but improved perfusion of both dorsalis pedis arteries. After 15 months of therapy with MTX and low-dose steroids, the patient reported no further improvement of the claudication and CRP values were slightly elevated [8 mg/l (normal value <5)]. Re-evaluation by MRI revealed the absence of inflammation in the temporal artery (Fig. 1C) and significantly decreased inflammatory signs in the lower calf arteries (Fig. 1E).



View larger version (106K):
[in this window]
[in a new window]
 
FIG. 1. (A) DSA shows multisegmented stenoses and occlusions of the lower limb arteries with the formation of collaterals. High-resolution MRI of (B and C) the right superficial temporal artery and (D and the E) the right calf. Contrast-enhanced, fat-saturated spin echo sequence clearly depicts mural thickening and contrast enhancement of the inflamed superficial temporal artery (arrow in B) and the anterior tibial artery (arrow in D). The accompanying veins (arrowheads in D and E) show a homogeneously increased signal intensity due to intravenous contrast agent within their comparatively larger lumen. Biopsy of the temporal artery confirmed the diagnosis of GCA. A follow-up examination after 15 months of corticosteroid treatment revealed the absence of mural inflammatory signs in the temporal artery (arrow in C) and significantly decreased mural inflammatory changes in the anterior tibial artery with less restricted lumen of the artery (arrow in E).

 
We present an unusual case of GCA with stenoses and occlusions of the lower limb arteries leading to claudication as pertinent clinical findings. In such a case, reaching the proper diagnosis is difficult unless the superficial temporal arteries are involved. The diagnosis of extracranial involvement is often only indirect and is made on the basis of clinical findings. Ultrasound is the primary imaging procedure of choice in GCA. However, it is observer-dependent, restricted to superficial arteries and not suitable to the examination of widespread arterial involvement. PET scanning has been suggested as an alternative method to depict vascular involvement [6], but radioactivity, lower resolution and high cost are disadvantageous. Recently, the first results of high-resolution MRI vessel wall imaging of the temporal artery in GCA [7] demonstrated mural thickening and contrast enhancement in medium-sized arteries defining the extent and intensity of vascular involvement and thus active disease [8].

In our case, the involvement of the superficial temporal arteries and the lower leg arteries was correctly depicted by high-resolution MRI. A control MRI under treatment showed an improvement in vessel wall inflammation. Interestingly, at that time the patient still presented with low systemic inflammation and no further improvement of claudication. Accordingly, the MRI of the lower limb detected residual contrast enhancement. Based on the clinical and laboratory parameters and supported by the MRI finding, immunosuppressive therapy was increased.

This case demonstrates that GCA can be more widespread than commonly expected, including arteries of the lower extremity. Especially for patients without involvement of the temporal arteries or in case of negative biopsy, high-resolution MRI provides excellent additional diagnostic support and will enhance our knowledge of the extent of the arterial involvement in GCA.

The study group was supported by BMBF grant: BMBF/KNR 01 91/9949.

The authors have declared no conflicts of interest.

References

  1. Seo P, Stone JH. Large vessel vasculitis. Arthritis Rheum 2004; 51:128–39.[CrossRef][Medline]
  2. Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc 1976;51:504–10.[ISI][Medline]
  3. Hall S, Persellin S, Lie JT, O'Brien PC, Kurland LT, Hunder GG. The therapeutic impact of temporal artery biopsy. Lancet 1983;2:1217–20.[ISI][Medline]
  4. Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle EJ. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med 1997;337:1336–42.[Abstract/Free Full Text]
  5. Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975;83:806–12.[ISI][Medline]
  6. de Leeuw K, Bijl M, Jager PL. Additional value of positron emission tomography in diagnosis and follow-up of patients with large vessel vasculitides. Clin Exp Rheumatol 2004;22(6 Suppl. 36):S21–6.
  7. Bley TA, Wieben O, Uhl M, Schmidt D, Thiel J, Langer M. High resolution MRI in giant cell arteritis: vessel wall imaging of the superficial temporal artery. Am J Roentgenol 2005;184:283–7.[Abstract/Free Full Text]
  8. Stanson AW. Imaging findings in extracranial (giant cell) temporal arteritis. Clin Exp Rheumatol 2001;18(Suppl. 20):S43–8.
Accepted 10 March 2005





This Article
Full Text (PDF)
All Versions of this Article:
44/7/954    most recent
keh646v1
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Search for citing articles in:
ISI Web of Science (2)
Disclaimer
Request Permissions
Google Scholar
Articles by Bley, T. A.
Articles by Langer, M.
PubMed
PubMed Citation
Articles by Bley, T. A.
Articles by Langer, M.
Related Collections
Other Rheumatology