British Journal of Rheumatology 1996;35:800-802

CASE REPORT THREE YEAR FOLLOW-UP OF A CASE OF GIANT CELL PRESENTING WITH A CHRONIC COUGH AND UPPER LIMB ISCHAEMIC SYMPTOMS M. RISCHMUELLER, R. P. DA VIES* and M. D. SMITH Departments of Rheumatology and * Radiology, Flinders Medical Centre, South Road, Bedford Park, South Australia 5042, Australia

SUMMARY Downloaded from https://academic.oup.com/rheumatology/article/35/8/800/1783114 by guest on 27 September 2021 A case of presenting in an atypical fashion with respiratory and upper limb ischaemic symptoms is described. The subclavian and axillary arterial involvement is demonstrated in this case, and followed up with repeated angiographic examinations over the next 3 yr. Despite resolution of all symptoms and return of pulses and a recordable in the ischaemic upper limb, there was no angiographic evidence of resolution of the axillary stenoses. KEY WORDS: Limb , Respiratory tract involvement, Giant cell arteritis.

GIANT cell arteritis (GCA) is an inflammatory right renal calculus, cholecystectomy for gallstones and a condition of the arterial tree, which can present in a right calf deep 30 yr previously, whilst on number of different ways [1-3]. When the condition oral contraceptives. There was no significant family history. presents with the more widely recognized symptoms of She was a non-smoker and denied any alcohol intake. On headache, amaurosis fugax and polymyalgia further questioning, she admitted to weight loss, occipital and rheumatica, the diagnosis is usually suspected and right frontal headaches, and night sweats over the preceding 3 months which had become more persistent immediately confirmed rapidly, with institution of appropriate prior to presentation. She denied any tongue or jaw treatment. In some patients, however, these presenting claudication and had not experienced any visual disturbance. features are overshadowed by less typical symptoms Examination revealed the absence of any palpable pulses [4-6, 9-11], leading to a failure to suspect this diagnosis in the right arm with no recordable blood pressure and her and enquire about the presence of more typical right hand was cooler than the left. Loud bruits were audible symptoms. This may result in a potentially serious (on auscultation) over both subclavian . Neither delay in diagnosis and the initiation of appropriate temporal artery tenderness nor nodularity was present on therapy. palpation, but there was tenderness of the left occipital artery We describe one such patient who presented with on palpation. unusual symptoms causing delayed recognition of The diagnosis of temporal arteritis was strongly suspected GCA, despite the presence of typical symptoms on and was supported by a normochromic, normocytic anaemia direct questioning. We also report the long-term of 109 g/1, an erythrocyte sedimentation rate (ESR; follow-up of this case in relation to the effect of steroid Westergren method) of 77 mm/h and a C-reactive protein of 105 mg/1 (normal range <6). Immunological screens, treatment on the resolution of her upper limb arterial including a cANCA, were negative. A Doppler study involvement. demonstrated a 40 mmHg pressure difference between the right and left brachial arteries, with a 20 mmHg difference CASE REPORT between the right and left radial and ulnar arteries. A A 64-yr-old woman presented in 1991 with a history of a pulmonary function study revealed mild small airways cold right arm and hand with associated post-exertional pain. obstruction with a significant improvement after bron- She had presented to her local physician some 4 months chodilator therapy, normal lung volumes and gas transfer. earlier with a persistent, non-productive cough and lethargy. Digital subtraction angiography of the aorta, great vessels Investigations at that time, including a chest X-ray, sputum and the arteries of the upper limbs was performed. The aorta culture for bacteria (including acid-fast bacilli) and and great vessels in the mediastinum were normal, as were the spirometry, had only revealed a small reduction in measured subclavian arteries. The right axillary artery showed an FEV,. Therapeutic trials of diuretics and bronchodilators irregular, severe producing a luminal reduction failed to relieve her symptoms, following which a course of varying from 50 to 90% and extending to involve the prednisolone resulted in the elimination of her symptoms and proximal brachial artery over ~8cm (Fig. 1). The left a substantial improvement in her FEV,. Subsequent cessation axillary and proximal brachial arteries showed a more of the prednisolone resulted in the return of her cough. uniform concentric stenosis of up to 80% over a 7 cm length. Previous medical history included for 3 yr, a A segmental biopsy of the left temporal artery was performed as this was considered to be less invasive than a Submitted 1 November 1995; revised version accepted 28 February biopsy of the symptomatic left occipital artery. Histopatho- 1996. logical examination of the temporal artery biopsy revealed Correspondence to: M. D. Smith, Department of Medicine, low-grade chronic , with multinucleated giant Flinders Medical Centre, South Road, Bedford Park, South cells and focal disruption of the internal elastic lamina, Australia 5042, Australia. consistent with the diagnosis of GCA. The patient was

© 1996 British Society for Rheumatology 800 RISCHMUELLER ET AL.: LARGE VESSEL INVOLVEMENT BY GIANT CELL ARTERITIS 801

with a chronic non-productive cough unresponsive to bronchodilators, but responding to steroid therapy. Respiratory symptoms are not widely recognized as potential manifestations of GCA and clearly were not recognized as such in this case. The literature contains sporadic reports of respiratory symptoms associated with GCA in addition to a review of 16 GCA patients with respiratory symptoms [5]. The authors of this review estimated that

in 4% of patients with GCA, respiratory symptoms are Downloaded from https://academic.oup.com/rheumatology/article/35/8/800/1783114 by guest on 27 September 2021 the initial manifestation, with up to 9% experiencing respiratory symptoms as part of GCA at some time during their illness. The association of such symptoms with the onset of the illness, their rapid resolution with steroid therapy and recurrence with relapse of the condition, or reduction or cessation of steroid therapy, provide strong evidence that these symptoms were related to the arteritic process, as was seen in our case. Large artery is common in GCA, but is more frequently identified at autopsy or on investigation than is apparent at initial presentation [7-11]. It has been estimated that large artery involvement is a presenting feature in 5% of cases with Fio. 1.—Digital subtraction arteriogram of the right axillary and GCA. The more typical large artery involvement leads brachial arteries showing irregular stenoses involving the proximal to readily recognized ischaemic symptoms such as 8 cm of the artery. The ribs and lungs can be seen to the right of the tongue necrosis and jaw claudication. One large study image. reported 10 patients presenting with GCA with upper limb ischaemia and noted that recanalization of commenced on 60 mg a day prednisolone with rapid occluded vessels could not be demonstrated in the three resolution of her headache, night sweats and lethargy. She was discharged back to her home in outback Australia, on prednisolone in addition to cyclical etidronate and calcium therapy, given as prophylaxis against steroid-induced osteoporosis. The prednisolone dose was gradually reduced over the next 3 yr whilst she was under regular out-patient review, with no recurrence of any symptoms related to her GCA. Her right arm pulses returned during the initial 6 months of prednisolone therapy, with the subsequent return of a recordable blood pressure. When reviewed 3 yr after the initial presentation, her prednisolone dose had been reduced to 5 mg daily, with a right brachial systolic blood pressure of 140 mmHg, compared to 160 mmHg in the left brachial artery. Diastolic blood pressure was recorded at 90 mmHg in both arms. Several adverse effects of prednisolone therapy had occurred, including weight gain, easy bruising and the recent detection of bilateral early cataracts. A decision was made to gradually taper off the prednisolone, which was successfully achieved over the next 5 months. Follow-up digital subtraction arteriograms were per- formed 4 months, and 1 and 3 yr after commencement of prednisolone (Fig. 2). The extent and severity of bilateral axillary stenosis were not altered angiographically, but collateral circulation was more prominent. Follow-up pulmonary function tests revealed a resolution of the obstructive pattern with near normalization of the FEV|. FIG. 2.—Digital subtraction arteriogram performed after 4 months DISCUSSION of prednisolone therapy. A magnified view of a normal calibre right subclavian artery and persisting irregular stenosis of the proximal Patients with GCA usually present with symptoms right axillary and brachial arteries is shown. Several prominent which can be broadly classified into the areas of collateral branches are seen extending into the muscles of the deltoid systemic, myalgic and arteritic features [10]. The and upper brachial region. A similar appearance was seen on all subject of this case report presented to her family subsequent arteriographic examinations in this patient up to 3 yr physician 4 months prior to referral to this hospital, after treatment. 802 BRITISH JOURNAL OF RHEUMATOLOGY VOL. 35 NO. 8 patients having follow-up angiographic studies, despite delays in diagnosis and the commencement of clinical improvement in all cases [7]. The sites of appropriate treatment. Despite resolution of ischaemic arterial occlusion, the angiographic appearance of the symptoms and return of pulses and a recordable blood stenosed arteries and the presence of bilateral pressure, the stenotic lesions in the arterial tree may not occlusions, despite the presence of symptoms in only recover despite long-term corticosteroid treatment. one arm, were features of these cases, similar to that seen in this case. ACKNOWLEDGEMENTS Our patient recovered clinically with resolution of The authors would like to express their thanks to Drs the typical GCA symptoms of headache, weight loss P. Shah and R. Hynes of Broken Hill, New South and night sweats, as well as the ischaemic symptoms Wales, for referring this patient for our initial with the return of all peripheral pulses. Although the assessment and management. Downloaded from https://academic.oup.com/rheumatology/article/35/8/800/1783114 by guest on 27 September 2021 recordable blood pressure in her right arm remains lower, it is approaching that of the left arm. However, REFERENCES the arterial stenoses remain unchanged, without 1. Strachan RW, How KJ, Brcwsher PD. Masked giant-cell angiographic improvement of the axillary arterial arteritis. Lancet 1980;l:194—6. stenoses following 3 yr of corticosteroid treatment. 2. Malmvall BE, Bengtsson BA, Alestig K, Bois G. The clinical pictures of giant cell arteritis: temporal arteritis, Clinical resolution of symptoms is likely to correspond polymyalgia rheumatica and fever of unknown origin. to the progressive development of collateral circulation Postgrad Med 1980;67:141-50. around the stenotic segments, which was already 3. Calamia KT, Hunder GG. Clinical manifestations of evident in this case on the follow-up angiogram, 4 giant cell (temporal) arteritis. Clin Rheum Dis 1980;6: months after presentation to this hospital. It is 389-403. suggested that the modulation of corticosteroid 4. Klein RG, Hunder GG, Swanson AW, Sheps SG. Large therapy for cases of GCA with involvement of limb artery involvement in giant cell (temporal) arteritis. Ann arteries should be based on the usual parameters of Intern Med 1975;83:806-12. clinical response (improvement in symptoms, fall in 5. Larson TS, Hall S, Hepper NGG, Hunder GG. ESR) rather than any consideration of the Respiratory tract symptoms as a clue to giant cell arteriographic appearance of affected arteries, arteritis. Ann Intern Med 1984;101:594-7. especially if adequate collateral circulation is clearly 6. Karam GH, Fulmer JD. Giant cell arteritis presenting as interstitial lung disease. Chest 1982;82:781-4. demonstrated. The persistence of bilateral subclavian 7. Ninet JP, Bachat P, Dumontet CM et al. Subclavian and and axillary arterial stenoses, however, raises a axillary involvement in temporal arteritis and polymyal- potential problem in the ongoing management of her gia rheumatica. Am J Med 1990;88:13-20. hypertension, since upper limb blood pressure 8. Walz-Leblanc BAE, Ameli F, Keystone EC. Giant cell measurements may not reflect the systemic blood arteritis presenting as limb claudication. Report and pressure. review of the literature. / Rheumatol 1991; 18:470-2. 9. Hunder GG. Giant cell (temporal) arteritis. Rheum Dis Two unusual presenting features of GCA occurred in Clin North Am 1990; 16:399-409. this case which went unrecognized as manifestations of 10. Jones JG. Clinical features of giant cell arteritis. arteritis and resulted in delayed diagnosis, despite the Bailliere's Clin Rheumatol 1991;5:413-30. fact that direct questioning of the patient revealed 11. Tsapatsaris NP. Temporal arteritis. General review with typical features of GCA. Greater awareness of the emphasis on atypical manifestations and cardiovascular varied presentations of this condition would avoid such complications. Cardiol Clin 1991;9:547-53.