Vasculitis and Limb Threatening Ischaemia

Dr RL Cornell                             Foundation Doctor                                 

Mr SCA Fraser                           Consultant Vascular Surgeon, Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh, EH16 4SA

Dr VB Dhillon                              Consultant Rheumatologist, Western General Hospital, Crewe Road South, Edinburgh, EH4 2XU

CORRESPONDENCE TO:

Dr Rachel Cornell

Simpson Centre for Reproductive Health

Royal Infirmary of Edinburgh

Little France Crescent

Edinburgh

EH16 4SA

Email:  Rachel.Cornell@luht.scot.nhs.uk

SMJ 2009 54(1): 58

ABSTRACT

Giant cell arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Classically patients present with temporal pain and visual disturbance, with less than 9% presenting with extracranial involvement.  We present three cases GCA presenting with limb threatening ischaemia. Each diagnosis was made after clinical suspicion prompted measurement of inflammatory markers. Following treatment with high dose steroid each patient made a rapid and dramatic recovery and avoided unnecessary major surgical revascularisation or lower limb amputation.

 

INTRODUCTION

In Western countries, giant cell arteritis (GCA) is the most common systemic vasculitis in patients over the age of 50,1 with a 4:1 female to male ratio.2

 

Classically, patients present with temporal pain, often with tenderness and jaw claudication.2 Visual manifestations occur in 30% of patients, with irreversible loss of vision in 15%.3 Symptoms result from granulomatous inflammation of vessel walls, predominantly the branches of the temporal and ophthalmic arteries. Proximal muscle pain and stiffness is often present because of the frequent association with polymyalgia rheumatica (PMR).1 Large artery involvement is uncommon and only 9% of patients have extracranial involvement.4 We describe three cases of giant cell arteritis presenting with lower limb ischaemia.

 

CASE ONE

A 60 year old female, presented with a three-week history of right calf claudication at 200 metres and right foot pain at rest. She described vague muscle pains and numbness in her right foot. Her only cardiovascular risk factors were a body mass index of 28 and mild hypertension.

 

On examination, there was a blood pressure discrepancy, with the right arm measuring 140/85 and the left measuring 162/79 mmHg. Femoral pulses were palpable bilaterally but popliteal and pedal pulses were absent. The right foot felt cool and turned pale on minimal elevation. Ankle brachial pressure index (ABPI) was 0.45 on the right and 0.9 on the left.

 

Blood tests revealed a microcytic anaemia, with normal white cell count and mild thrombocytosis. Cholesterol, renal function and coagulation were normal. An arterial duplex scan demonstrated concentric thickening of the walls of both superficial femoral arteries (SFA) throughout their length, with narrowing of the lumen. Magnetic resonance angiography (MRA) demonstrated extensive, diffuse linear stenoses of the SFAs to the level of the adductor segments, more marked on the right side. There were multiple stenoses and occlusions of the popliteal and calf vessels bilaterally. MRA of the aortic arch demonstrated a diffusely narrowed left subclavian artery, consistent with the blood pressure discrepancies.

 

 

 

The history, examination and investigations were consistent with critical limb ischaemia. However, the acute onset of severe disease, without significant risk factors, was not typical of an atherosclerotic process so a vasculitis was suspected. Further blood tests demonstrated elevated inflammatory markers with a C-reactive protein (CRP) of 150mg/L and erythrocyte sedimentation rate (ESR) of 90 mm/hr. Autoantibody and thrombophilia screens were negative. A subsequent temporal artery biopsy demonstrated intimal oedema, causing significant stenosis and low grade inflammation involving the media and adventitia. This confirmed the diagnosis of GCA and the patient was commenced on high dose corticosteroids.

 

Ten weeks later, all symptoms had improved significantly and the ESR had fallen to 7. The patient’s claudication distance improved considerably and the steroid dose was reduced.


CASE TWO

A 55-year-old male, presented with a two-month history of bilateral leg claudication, at 250 metres, principally affecting his left side. He denied rest pain. He had never smoked and had no medical history other than an episode of polyarteritis affecting his hands, nine years previously.

 

On examination, he was normotensive and femoral pulses were palpable bilaterally. Popliteal and pedal pulses were however markedly reduced. ABPI was 0.6 on the left and 0.9 on the right. He had soft bruits over both SFAs.  Investigation demonstrated normocytic anaemia, reactive thrombocytosis and a marked inflammatory response with ESR of 94 and CRP of 152. Other blood tests were normal.  An arterial duplex scan revealed diffuse stenosis within both common femoral arteries, with bilateral focal stenosis at the level of the adductor canal. Findings were worse on the left side.

In view of his young age and speed of symptom progression, it was assumed a vasculitis was responsible and an urgent temporal artery biopsy demonstrated the classical appearance of GCA. Corticosteroid treatment was started immediately. 

 

The patient subsequently complained of intermittent chest pain. MRI of the thorax and abdomen demonstrated no great vessel involvement however echocardiogram demonstrated mild left ventricular dilatation and the possibility of coronary artery involvement by GCA was raised. Aspirin and ramipril were started.

 

After two months of steroid therapy, improvement was seen in the claudication distance and the dose reduced. The ESR and CRP fell to 23 and 10 respectively and there were no further symptoms of myocardial ischaemia. Repeat duplex scanning demonstrated a 30% improvement in the stenosis.

 

CASE THREE

A 60-year-old male, presented with a sudden onset of bilateral leg pain after three minutes of walking.  The pain was relieved by rest and was worse on the left. He complained of numb toes on this side. Mr GD had no other symptoms other than occasional headache.  His symptoms appeared following a prolonged undiagnosed illness of severe malaise, nausea and headache. His only cardiovascular risk factor was mild hypercholesterolaemia.

 

On examination, he was normotensive with a regular pulse. Femoral pulses were palpable but all distal pulses were absent. A bruit was heard over the left SFA and there was dusky discolouration to the left foot. A positive Buergers test confirmed critical ischaemia. ABPI was 0.5 on the right and 0.4 on the left. In keeping with an inflammatory process, the CRP was 235 and ESR was 88. Full blood count demonstrated a normocytic anaemia with thrombocytosis. Duplex scanning demonstrated diffuse disease throughout the left SFA and run-off vessels. There was no focal stenotic lesion but the walls appeared thickened. MRA confirmed these findings. Suspicion of GCA prompted a temporal artery biopsy, which demonstrated age-related changes only. There was no evidence of inflammation.

 

This patient had only a single vascular risk factor. Skip lesions are common in GCA and clinical suspicion remained high, so despite the negative biopsy, steroids were started. The patient enjoyed a rapid improvement in his symptoms. The rapid response to treatment and elevated inflammatory markers supported the diagnosis of vasculitis.

 

After 6 months of a reducing steroid regime, inflammatory markers were normal and walking distance had improved considerably. Further assessment by angiography confirmed a major vasculitic component.

 

DISCUSSION

All three patients presented with severe, potentially limb threatening ischaemia. There were no classical symptoms or signs of giant cell arteritis.

 

GCA is characterised by granulomatous involvement of large and medium-sized blood vessels. Although it may be widespread, symptomatic vessel inflammation usually involves the cranial branches of the arteries originating from the aortic arch5. GCA rarely causes limb ischaemia but early recognition is the key to achieving a good outcome. Gonzalez-Gay et al demonstrated that only 1.9% of patients with GCA had lower limb claudication due to ischaemia8. Le Hello et al reported on eight patients with lower limb ischaemia, secondary to vasculitis, and documented one death and one amputation6. There are reports of limb amputation for vasculitic ischaemia6, but none of successful surgical or endovascular revascularisation. In reporting a study of GCA induced ischaemia, Evans et al recommended that bypass grafts should not be considered in patients with active GCA because of poor patency9. Technical results of bypass surgery to inflamed vessels are unpredictable.

 

The treatment of GCA is glucocorticoid therapy. Prednisolone, typically 40-60 mg daily, is prescribed until symptoms fully resolve and the ESR and CRP return to normal7. Maintenance therapy is required for at least one year and it is not unusual for patients to require life-long maintenance low dose steroid2. Relapse occurs in approximately 30% of patients and is an indication to increase the steroid dose, with additional immunosuppressive therapies2.

 

Early measurement of ESR, temporal artery biopsy and treatment with prednisolone allowed each of these patients with GCA to avoid major revascularisation surgery and limb amputation surgery. In each case, simple medical treatment was sufficient to halt disease progression and improve symptoms considerably. Potential side-effects of medical treatment were anticipated and prevented.

 

REFERENCES 

  1. Gonzalez-Gay MA, Garcia-Porrura C. Epidemiology of the vasculitides. Rheum Dis Clin North Am. 2001; 27: 729-749.

  2. Churchill Livingston. Haslett C, Chilvers ER, Boon N, Colledge NR. Davidson’s Principles and Practice of Medicine. 19th Edition, 2002.

  3. Aillo PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder GG. Visual prognosis in giant cell arteritis. Ophthalmology. 1993; 100: 550- 555.

  4. lein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell arteritis. Ann Intern Med 1975; 83: 806-12.

  5. Salvarini C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant cell arteritis. N Engl J Med. 2002; 347: 261-271.

  6. Le Hello C, Levesque H, Cailleux N, Galateau F, Peillon C, Veyssier P, Watelet J, Letellier P, Courtois H, Maixa D. Lower limb giant cell arteritis and temporal arteritis: follow up of eight cases. Journal of Rheumatology. 28(6): 1407-12, 2001.

  7. Hunder GG. Treatment of giant cell arteritis. In: Rose BD, Wellesley MA, 2005. 

  8. Gonzalez-Gay MA, Pineiro A, Gomez-Gigirey A, Garcia-Porrua C, Pego-Reigosa R, Dierssen-Sotas T, Llorca J. Influence of traditional risk factors of atherosclerosis in the development of severe ischaemia complications in giant cell arteritis. Medicine. 83: 343-347. 2004.

  9. Evans DC, Murphy MP, Lawson JH. Giant cell arteritis manifesting as mesenteric ischaemia. Journal of Vascular Surgery. 142(5): 1019-1022. 2005.

Back to February Contents