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332 Letters to the editor pressure or hypotensive episodes were technique and rating scale for abnormalities of the thorax, without intravenous contrast seen in and related disorders. recorded during the trial. Among the side Rheum 1981; 24: 1159-65. medium (because of asthma), showed a effects, mild flushing (35%) and 9 Ferri C, La Civita L, Zignego A et al. Plasma normal mediastinum but a small rim of

L, Ann Rheum Dis: first published as 10.1136/ard.55.5.332 on 1 May 1996. Downloaded from (24%) were the most frequent in isradipine levels of endothelin-I in mixed cryoglobulin- pleural fluid on the left. aemia patients. Br J Rheumatol 1994; 33: recipients. 689-90. At follow up, the patient complained of This study has demonstrated the favour- 10 Yanagisawa M, Kurihara H, Kimura S, et al. A right thigh . Radiofemoral delay able effects of isradipine on patients with novel potent vasoconstrictor peptide pro- was noted. CT of the abdomen showed Raynaud's phenomenon; moreover, it is the duced by vascular endothelial cells. Nature internal displacement ofcalcified in 1988; 332: 411-5. first to demonstrate a significant reduction 11 Levin E R. Endothelins [review]. N Engl J Med the , suggesting aortic . Mag- in plasma concentrations of ET-1 during 1995; 333: 356-63. netic resonance imaging (MRI) confirmed calcium antagonist treatment. The most this as distal to the origin of the left sub- usual manifestations of Raynaud's phenom- clavian , extending to the aortic bifur- enon are pain and numbness in the fingers, cation. Eight weeks after discharge, he devel- which in some subjects can be complicated Survival after aortic oped , aching thighs and jaw by skin ulcers5 requiring prompt inter- claudication with temporal artery tenderness. vention.3 4 In the present study, isradipine dissection in Prednisolone 30 mg daily was begun with was able to reduce the frequency, severity, rapid improvement. Clinical improvement and disabling nature of acute attacks of correlated with his ESR decreasing to normal Raynaud's phenomenon; this result agrees Acute in and with gradual resolution of the radio- with previous experience of the use of is rare and usually fatal. It is associated with femoral delay. calcium antagonists in patients with and is commoner in females. Raynaud's phenomenon,' 3 4 and reflects an We describe two patients who were diagnosed PATIENT 2 appropriate selection of patients to receive in life and who survived on medical treatment A 68 year old white women presented with the treatment. In addition, the clinicosero- alone for a prolonged period: one for two five days of worsening anterior pleuritic chest logical manifestations and changes in capil- years; the other still alive at three years. A pain radiating to the left, sweats, nausea, and laroscopic findings in this series of patients, third patient who required aortic grafting has an increased temperature. She had a history including those with type I and type II survived 14 years. A literature review of osteoarthrosis, euthyroid Graves' ophthal- Raynaud's phenomenon, were relatively revealed 43 other cases, with only three mopathy, Raynaud's phenomenon, and long- homogeneous. survivors on medical care alone. standing hypertension. Temporal arteritis The clinical improvement of our patients had been diagnosed on biopsy eight years with Raynaud's phenomenon in response to Case reports previously, following a characteristic illness isradipine was reflected in a reduction in PATIENT 1 accompanied by an ESR of 121 mm/lst h. plasma concentrations ofET-1. Although the A 68 year old white male presented with Prednisolone for three years had achieved pathophysiological ftmction of ET-1 remains acute chest pain on exertion radiating to the remission. unknown,"3 a number of vascular disorders back, accompanied by dyspnoea and On admission, the patient was pyrexial at are characterised by increased concentrations sweating. He had noted weight loss of more 37 70C. The heart rate was 110 beat/min with of this vasoactive peptide." How isradipine than 7 kg over the previous months. A history no radiofemoral delay, and the may influence the titres ofET-1 is difficult to of back and neck ache was ascribed to was 140/90 mm Hg, equal in both arms. There explain but, because ET-l is released in spondylosis, and shoulder pain to a rotator was dullness to percussion at both lung bases response to ischaemic stimuli," we hypothe- cuff lesion. His was 60 beats/min and bronchial breathing at the left base. ESR sise that isradipine is able to induce a change regular, and his blood pressure was 150/70 was 86 mmnlst h, and the leucocyte count I1-4 in ET-l concentrations indirectly, by improv- mm Hg. An ejection murmur was noted at with a neutrophilia. ECG showed left ventri- ing tissue perfusion. The findings of this the left sternal edge. Examination was other- cular hypertrophy. Chest radiograph showed preliminary investigation merit further in- wise unremarkable. bilateral small pleural effusions and a markedly http://ard.bmj.com/ vestigation in larger series of patients with The patient developed intermittent pyrexia widened mediastinum. CT of the thorax and Raynaud's phenomenon. over the next three weeks, and experienced abdomen revealed a dilated thoracic aorta with frequent left sided stabbing chest pain. The a dissection in the descending portion. Trans- LUCA LA CIVITA leucocyte count increased from normal to DIIJA GIUGGIOLI oesophageal echocardiography (TOE) initially MARIA GRAZIA DEL CHICCA 14-5 with a neutrophilia. Erythrocyte sedi- suggested dissection commencing at the aortic GIOVANNI LONGOMBARDO mentation rate (ESR) peaked at 94 mm/lst root; however, repeat CT, arch aortography GIAMPIERO PASERO h and C reactive protein (CRP) at 260 mg/l. and MRI confirmed type B dissection. The

CLODOVEO FERRI on September 25, 2021 by guest. Protected copyright. Istituto Patologia Medica and Blood and urine cultures were negative, and patient was treated with a [3 blocker to main- Fisiologia Clinica (CNR), autoantibody screen was normal. The tain normal blood pressure, and prednisolone University ofPisa, Pisa, Italy electrocardiogram (ECG) showed left ven- at a dose of 20 mg. The pain resolved and she tricular hypertrophy, confirmed by trans- was discharged, remaining well and normo- Correspondence to: Dr Luca La Civita, Istituto di Patologia Medica I, University of Pisa, via Roma thoracic echocardiogram, which showed no tensive for two years. Mediastinal widening 67, 56126 Pisa, Italy. other abnormality. Chest radiograph showed then began to accelerate, MRI imaging con- minor atelectasis at both lung bases. Indium finning a grossly dilated dissecting descending 1 Cleophas T J M, Niemeyer M G. Raynaud's labelled leucocyte scan showed some media- aorta and dilatation of the ascending aorta syndrome, an enigma after 130 years. stinal uptake. Computed tomography (CT) (figure). She died soon afterwards. Angiology 1993; 43: 196-209. 2 Biondi M L, Marasini B, Bassani C, Ago- stoni A. Increased plasma endothelin levels in patients with Raynaud's phenomenon. NEngl JMed 1991; 324: 1139-40. 3 Wollersheim H, van Zwieten P A. Treatment of Raynaud's phenomenon. Eur Heart J 1993; 14:147-9. 4 La Civita L, Pitaro N, Rossi M, et al. Amlodipine in the treatment of Raynaud's phenomenon: a double-blind placebo- controlled crossover study. Clin Drug Invest 1996; 11 (suppl): 126-31. 5 Kallenberg C G M, Pastoor G W, Wouda A, The T. Antinuclear antibodies in patients with Raynaud's phenomenon: clinical signifi- cance of anticentromere antibodies. Ann Rheum Dis 1982; 41: 382-7. 6 Allen E V, Brown G E. Raynaud's disease: a critical review of a minimal requisite for diagnosis. Am J Med Sci 1932; 183: 187-200. 7 Ferri C, Bernini L, Cecchetti R, et al. Cutaneous and serological subset of systemic sclerosis. Their relevance in diagnosis, severity and prognosis of the disease. J Tl weighted axial MR images. Left: Normalflow void in the dilated ascending aorta and a complex Rheumatol 1991; 18: 1826-32. signalpattern in the descending aorta at the origin ofthe dissection. Right: At the level ofthe dilated 8 Maricq H R Widefield microscopy: aortic root, showing the two lumina in the descending thoracic aorta. Letters to the editor 333

Summary ofcases ofaortic dissection associated with giant ceUl arteritis reported in the literature Correspondence to: Dr A M L Lever. Source Number Age Sex Hypertension Comments 1 Klein R G, Hunder G G, Stanson A W, ofcases (yr) Sheps S G. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975; Ann Rheum Dis: first published as 10.1136/ard.55.5.332 on 1 May 1996. Downloaded from Broch 19472 1 68 F + First recorded case (case report) 83: 806-12. Klein 1975' 3 66 F NK Series of 248 cases of GCA. All 3 sudden deaths; 2 Broch A J, Ytrehus 0. Tre nye tilfelle av arteritis 73 F NK diagnosed at postmortem examination temporalis. Nord Med 1947; 34: 1111. 70 F NK 3 Nakashima Y, Kurozumi T, Sueshi K, Larson 19844 2 NK F + Postmortem series of 161 acute dissections; 'healed Tanaka K. Dissecting : a NK F + ' found in both clinicopathological and histopathologic study Leonard 1979' 2 62 F NK Postmortem series of 171 acute dissections of 111 autopsied cases. Hum Pathol 1990; 21: 74 F + 291-6. BMJ 19606 1 70 M + Known GCA. Sudden death (case report) 4 Larson E W, Edwards W D. Risk factors for Save-Soderbergh 2 85 F + Postmortem series of 9 sudden deaths in GCA aortic dissection: a necropsy study of 161 19867 79 F + cases. AmJ Cardiol 1984; 53: 849-55. Nordt 1991'4 1 70 F - Acute myocardial infarction caused by dissection; 5 Leonard J C, Hasleton P S. Dissecting aortic died (case report) : a clinicopathological study. Q Jf N Y State J Med 1 77 F + Known GCA. Sudden death (case report) Med 1979; 48: 55-76. 1985'5 6 Clinicopathological conference. A case of Ainsworth 1961'6 1 83 M - Sudden death (case report) temporal arteritis demonstrated at the post- Harris 1968'7 2 70 F + Sudden death (case report) graduate medical school of London. BMJ 81 F + Previous diagnosis of GCA and sudden death (case 1960; 2: 287-93. report) 7 Save-Soderbergh J, Bo-Eric M, Anderson R, Magarey 1950"8 1 75 F NK Sudden death (case report) Bengtsson B-A. Giant cell arteritis as a cause McMillan 1950'9 1 58 M + Fatal stroke; GCA with dissection at postmortem of death. JAMA 1986; 255: 493-6. examination (case report) 8 Paulley J W, Hughes J P. Giant-cell arteritis or Paulley 19608 1 70 F + GCA and dissection diagnosed; survived months arteritis of the aged. BMJ 1960; 2: 1562-7. (case report) 9 Salisbury R S, Hazleman B L. Successful treat- NEnglJMed 1 72 F + Survival following aortic graft (case report) ment of dissecting due to 199320 giant cell arteritis. Ann Rheum Dis 1981; 40: Salisbury 19819 1 83 F + Survival following aortic graft 14 years+ (case report) 507-8. Ostberg 197210 2 - - NK 998 postmortem examinations; 2 dissections among 10 Ostberg G. Morphological changes in the large 15 GCA cases found in polymyalgia arteritica. Acta Med Murai 1989" 2 - F NK Both died; case reports Scand 1972; 533 (suppl): 135. Evans 1994'" 16 - - From series of 41 patients with GCA and aortic 11 Murai T, Saito K, Nurada N, et al. Acute aortic involvement: 8 died; 6 surgical repairs; 2 long term dissection due to giant cell arteritis. Report of non-surgically treated survivors diagnosed two autopsy cases. Acta PatholJ7pn 1989; 39: retrospectively. Median age ofwhole group 67 yr. 821-6. Male:Female ratio 10:31. 14 of 41 hypertensive 12 Evans J M, Bowles C A, Bjornsson J, This paper 2 68 M LVH Survival on steroids alone for 2 years Mullany C J, Hunder G G. Thoracic aortic 68 F + Survival on steroids alone for 3 years aneurysm and rupture in giant cell arteritis. Total cases 43 Arthritis Rheum 1994; 37: 1539-47. 13 Cigarroa J E, Isselbacher E M, DeSanctis R W, NK = Not known; GCA = giant cell arteritis; LVH = left ventricular hypertrophy. Eagle K A. Diagnostic imaging in the evaluation of suspected aortic dissection. N EnglJ Med 1993; 328: 35-43. 14 Nordt T K, Rauch B, Mattfeld T, et al. Acute myocardial infarction due to proximal aortic Discussion Most of these patients showed evidence of dissection in giant cell aortitis. Am Heart Jf Giant cell arteritis is distinguished from hypertension in life or at postmortem 1991; 122: 1151-3. Takayasu's disease on clinical and patho- examination. Twenty two of 27 reported 15 Clinicopathological conference. A 77-year old woman with giant-cell arteritis, back pain and logical grounds. Medium sized arteries are cases were of women - disproportionate to shock. NY Statef7Med 1985; 85: 502-8. usually affected, although 15% of a large the 1:2 male:female ratio in giant cell 16 Ainsworth R W, Gresham G A. Giant-cell

series were found to have large vessel involve- arteritis. The gender of 16 cases with aortic arteritis with rupture of the aorta. J Path Bact http://ard.bmj.com/ 1961; 82: 203-5. ment. Despite the characteristic histopatho- dissection in the large series reported by 17 Harris M. Dissecting aneurysm of the aorta due logical findings of mononuclear cell infiltrate, Evans et al12 was not specified. to giant cell arteritis. Br Heart ft 1968; 30: disruption of the internal elastic lamina and The diagnosis of acute aortic dissection may 840-4. the presence of giant cells which would be be made by four different imaging techniques. 18 Magarey F R. Dissecting aneurysm due to giant- cell aortitis.J Path Bact 1950; 62: 445-7. expected to reduce the elasticity and tissue Retrograde aortography has a sensitivity of 19 McMillan G C. Diffuse granulomatous aortitis strength of large arteries, aortic dissection is only 81-91%, though specificity may be more with giant cells associated with partial rupture an unusual complication (table). The first than 90%.'3 It can demonstrate involvement and dissection of the aorta. Arch Pathol 1950; case was from in 1947.2 A of branch vessels and coronary arteries. CT 49: 63-9. reported Norway 20 Case records of the Massachusetts General on September 25, 2021 by guest. Protected copyright. postmortem study of 111 cases of dissecting has a sensitivity of 83-100% and a specificity Hospital. NEnglJ Med 1993; 329: 1028-33. aortic aneurysm from western Japan did not of 90-100%. MRI has been shown to be up implicate giant cell arteritis as a cause in any to 100% sensitive and specific in some studies. case,3 and 161 postmortem cases of TOE may be 97-100% sensitive, but dissecting aortic aneurysm from the Mayo specificity as low as 68% has been reported, Clinic also failed to implicate giant cell probably reflecting operator dependence. arteritis with certainty, though two had The cases described had clinical evidence Antibodies to collagens in 'healed aortitis'.4 Klein describes three cases of giant cell arteritis, biopsy proven in two sera from patients of aortic dissection in giant cell arteritis that cases, and aortic dissection. The two presen- were fatal, one in the descending and two in tations with and chest pain did not receiving bovine cartilage the ascending aorta.' Of 125 postmortem immediately suggest the diagnosis. Clinicians graft- cases seen in Manchester, England, two had should be aware of potential life threatening evidence of giant cell arteritis.' The usual large vessel disease in giant cell arteritis, The production of autoantibodies against cause of death is aortic rupture, though old, particularly in female patients with hyper- different collagen types has been described in healed dissection may be an incidental tension. The outcome may be more association with several clinical and exper- finding.6 In a postmortem series of nine favourable than previously believed, if the imental pathological entities. Antibodies to deaths related to giant cell arteritis, Save- arteritis is adequately controlled with collagen type II have been demonstrated in Soderbergh et al mentioned two elderly steroids. The diagnosis of giant cell arteritis ' and in rheumatoid hypertensive females with aortic dissection, should be considered in patients presenting arthritis.2 at least one of whom appears to have had a with acute dissection of the aorta. Cartilage grafts for tissue reconstruction descending aortic lesion.7 have been used in many surgical specialties, M P RICHARDSON The first reported case diagnosed and who A M L LEVER especially in otorhinolaryngology and survived for some months was described in University Department ofMedicine, maxillofacial surgery. Autograft cartilage has Ipswich.8 One previous case from our Addenbrooke's Hospital, Hills Road, the tendency to warp. Furthermore, harvest hospital did well after aortic resection and is Cambndge CB2 2QQ, United Kingdom of the material requires an additional operat- still alive and symptom free 14 years later.9 In A M FINK ive procedure. Recently, we observed two other reports, two of 16 postmortem A K DIXON humoral reactivities against cartilage cases of giant cell arteritis died of aortic University Department ofRadiology components in patients who had undergone dissection,'0 and two further fatal cases in B L HAZLEMAN a nasal contour reconstruction with allo- women have been described from Japan."I Department ofRheumatology geneic or autologous cartilage grafts.3 These