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Catheter angiography and angioplasty in patients with scleroderma

Departments of ¹Radiology, ²Vascular Surgery and ³Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK

Correspondence: Dr A F Watkinson

	Abstract

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The objectives of this study were (i) to identify patterns of angiographic disease in scleroderma patients with and without other vascular risk factors and (ii) to define patients with scleroderma in whom angiography and angioplasty is useful. The records of 26 patients with scleroderma who underwent angiography and angioplasty over an 8-year period were reviewed. Angiographic disease patterns were assessed using a modified Brewster classification. Angiography of the upper limb demonstrated distal disease alone in 86% of patients, both with and without other vascular risk factors such as smoking. In the lower limb there was a highly significant association between the presence of other vascular risk factors and macrovascular disease potentially amenable to angioplasty, and conversely between the absence of other vascular risk factors and distal disease in the lower limb. Good early but poor late clinical results were achieved in three of five patients who underwent angioplasty. Angiography of the upper limb is likely to demonstrate distal disease alone, and angiography and angioplasty of the lower limb may be useful only if other vascular risk factors are present.

	Introduction

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Scleroderma is a generalized connective tissue disorder [1]. It affects women three times more commonly than men and its incidence is 10 per million of the population per year [2]. The mechanisms of the disease are incompletely understood but include vasospasm and endothelial damage. The main target organs are the skin, lungs, gut, heart and kidneys. Raynaud's phenomenon is an early manifestation, followed by severe digital ulceration and pain [3–5]. The indication for angiography in scleroderma has not been clearly defined.

	Materials and methods

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Study group
There are 1400 patients with scleroderma at our institution, which is a national referral centre. 26 of these patients have had catheter angiography or angioplasty performed between 1992 and 2000. 7 patients had upper limb angiography and 21 had lower limb angiography, and 2 patients had both. All patients were jointly under the care of rheumatologists and vascular surgeons. Data collected on each patient who underwent angiography included age, sex and presence of other vascular risk factors (including smoking, hypertension, diabetes and hypercholesterolaemia). Patients were classified into those with one or more vascular risk factor or those with no vascular risk factors. The number of years with Raynaud's phenomenon and scleroderma was noted. Using the Fontaine classification for lower limb ischaemia, the presence of claudication (II), rest pain (III) and ulceration/gangrene (IV) was noted as well as the indication for angiography [6].

Indication for angiography
The indication for upper or lower limb angiography in 24 patients was critical limb ischaemia, i.e. ulceration, gangrene and/or rest pain (Fontaine III/IV). In the remaining two patients the indications were intermittent claudication (Fontaine II, n=1) and the investigation of renal artery stenosis (n=1).

Angiographic assessment
The angiograms were reviewed by an experienced vascular radiologist who was aware of the diagnosis of scleroderma but was blinded to the patient's identity and vascular risk factors.

The original Brewster classification [7,8] was modified to include disease in both the upper and lower limb and also to include disease that only involved vessels below the knee and elbow. The modified Brewster classification is as follows (see Figure 1). In type I disease of the lower limb there is localized aortoiliac disease with occlusive lesions involving the distal aorta as far as the common iliac arteries. In the upper limb, type I disease involves the arch of the aorta and great vessels (brachiocephalic, left common carotid and left subclavian arteries).

View larger version (30K): [in this window] [in a new window]   Figure 1. Modified Brewster classification of disease of the upper and lower limb. (a) Type 1 disease: involving the aortoiliac vessels in the lower limb, and the aortic arch and great vessels in the upper limb. (b) Type II disease: aorta to external iliac or femoral arteries (lower limb), or aorta to axillary arteries (upper limb). (c) Type III disease: multilevel disease in the upper and lower limbs. (d) Type IV disease: vessels above the knee or elbow are normal but vessels below the knee or elbow are diseased.

In type III disease, the most common pattern observed by Brewster, there is multilevel disease, often with occlusive infra-inguinal lesions. Patients with both proximal and distal disease are included in this group. In the upper limb this corresponds to multilevel disease from the thoracic aorta to below the elbow.

In type IV disease, vessels above the knee or elbow are normal but those below the knee or elbow are diseased.

Types I–III are termed macrovascular disease, whereas type IV disease is termed distal disease.

Angioplasty
Angioplasty was performed in a small group (n=5) of patients and the radiological and clinical outcome was assessed.

Statistical analysis
The association between the pattern of disease and the presence or absence of other vascular risk factors was assessed with the Fisher Exact Test.

	Results

Top Abstract Introduction Materials and methods Results Discussion References

 
Of the 26 patients with scleroderma who underwent catheter angiography between 1992 and 2000, 22 were female and 4 were male. The mean age at diagnosis of scleroderma was 52 years (range 22–79 years). Angiography was performed on average 10 years later (mean age 62 years, range 38–84 years).

Vascular risk factors
With regard to vascular risk factors, 17 patients were current or ex smokers (a higher proportion than the general population). One patient had a serum cholesterol level above 7.0 g dl^-1. All patients with hypertension (n=4) were also smokers. One patient had non-insulin dependant diabetes, treated with oral hypoglycaemics, but was also a smoker. 18 patients in total had vascular risk factors; 8 out of the group of 26 patients had no vascular risk factors.

Upper limb angiography (Table 1a)
Seven patients underwent angiography of the upper limb, of whom six had type IV (distal) disease; one patient had type III disease. Four patients had vascular risk factors (three smokers and one patient with hypercholesterolaemia). Three patients had no vascular risk factors.

View larger version (79K): [in this window] [in a new window]   Figure 2. Digital subtraction angiography of the wrist and hand in a 51-year-old female with limited scleroderma. Type IV (distal) disease in the upper limb. Normal vessels above the elbow (not seen), with tapering and obliteration of the ulnar, radial and severaldigital arteries and incomplete filling of the palmar arches.

Lower limb angiography (Table 1b)

Patients with type I–III disease had angiographic appearances similar to those described by Brewster [8] and others [9]. Two patients with type III disease (defined as multilevel disease) had typical macrovascular disease but with severe distal disease as well, suggesting treatment of proximal lesions would not improve symptoms. Patients with type IV disease had normal proximal vessels but tapering or obliteration of calf vessels with poor collateral vessel formation and minimal digital filling. These appearances were similar to that of the upper limb.

Angioplasty
Five patients, all with macrovascular disease, were suitable for angioplasty. Four patients had other vascular risk factors, one did not. One patient had a poor radiological and clinical result. A second patient had a good radiological result but a poor clinical result (illustrated in Figure 3). Three patients had good radiological results and initially good clinical results, but in two patients there was a return of critical limb ischaemia within 1 day (the single patient without other vascular risk factors) and 2 months, respectively. Thus, angioplasty was technically successful in 4 of 5 cases, with only short-term clinical success in 2 of 5 and long-term success in 1 of 5.

View larger version (66K): [in this window] [in a new window]   Figure 3. 55-year-old female with limited scleroderma who smokes. Pre- and post-angioplasty subtraction images of the left thigh and calf. (a) Pre-angioplasty angiography shows occlusion of the left superficial femoral artery as far as the adductor canal. This was dilated, with a very satisfactory post-angioplasty appearance (b). However, images over the calf (c) show occlusion of two of three calf vessels, with poor collateralformation, the likely explanation for the poor clinical result.

	Discussion

Top Abstract Introduction Materials and methods Results Discussion References

To our knowledge, this is the first study to investigate the association between lower limb angiographic appearances and the presence of other vascular risk factors in patients with scleroderma. There is a strong association in the lower limb between the presence of other vascular risk factors and macrovascular disease, and the absence of other vascular risk factors and distal disease. Patients with other vascular risk factors appeared to have macrovascular disease of the type described by DeBakey and Brewster [7–9]. Thus, angiography is indicated because a lesion potentially amenable to radiological or surgical intervention may be identified in patients with other vascular risk factors. Our results suggest that patients with scleroderma but without other vascular risk factors were highly likely to have distal (below knee) disease not amenable to traditional radiological and surgical intervention. However, the development of new radiological (angioplasty of plantar/palmar arch vessels) and surgical (distal bypass techniques onto foot vessels) techniques may offer benefit for the treatment of distal (below knee) lesions (see below) [15, 16].

Critical limb ischaemia is a difficult diagnosis to make in scleroderma, as many patients have lower limb rest pain and digital ulceration. There have therefore been several suggested modifications to the indications for angiography in scleroderma.

Edwards and Porter [2] suggested that angiography be performed in patients with unexplained digital occlusions in an asymmetrical distribution, for example several digits in one hand only, to rule out a treatable proximal lesion causing distal embolisation (blue digit syndrome). Mills et al [11] concur, suggesting that arteriography is unnecessary if finger gangrene occurs in a patient in whom a firm diagnosis of associated disease has been established and digital plethysmography is diffusely abnormal bilaterally. However, angiography should be performed if symptoms and signs are unilateral. We suggest that in patients with scleroderma the following criteria for angiography be adopted.

Upper limb catheter angiography should not be employed routinely in symmetrical disease, as there is likely to be distal disease not amenable to current radiological or surgical intervention. However, emerging techniques, including distal digital bypass and distal angioplasty using microballoons, may prove beneficial in the future.

In the lower limb, patients with other risk factors may benefit from angiography, as a lesion potentially amenable to radiological or surgical intervention may be identified. Patients with scleroderma but without other vascular risk factors are highly likely to have distal (below knee) disease that may not be amenable to angioplasty or vascular surgery. Catheter angiography and angioplasty may therefore not be helpful in these patients, although newer surgical and radiological techniques outlined above may also be useful in lower limb distal disease.

Our study has several weaknesses. It is retrospective and some patients with critical limb ischaemia may not have been included owing to concurrent severe medical problems. As our institution is a national referral centre, some patients may have had angiography performed at their local hospital, which will not have been included in this study. None of this cohort of patients underwent magnetic resonance angiography (MRA). Contrast enhanced three-dimensional turbo MRA has been shown to have a sensitivity of 91% and a specificity of 89% compared with conventional catheter angiography in detecting stenoses above the knee and would therefore be suitable for patients with scleroderma [17].

In summary, 86% of this cohort of patients with scleroderma who underwent angiography had distal vascular disease in the upper limb, whether or not other vascular risk factors were present. In the lower limb, patients with other vascular risk factors were highly likely to have proximal vascular disease, potentially amenable to intervention, while patients without any other vascular risk factors were highly likely to have distal (below knee) disease alone.

	Acknowledgments

 
We wish to thank Mr Knight of the Scleroderma Unit for his help in tracing patient's records.

Received for publication May 2, 2001. Revision received July 23, 2001. Accepted for publication July 27, 2001.

	References

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