726 Matters arising, Letters associated diseases in southern Chinese among whom anti-MPO predominate.

S S LEE JWMLAWTON KHKO Department of , The University of Hong Kong, Queen Mary Hospital Compound, Pokfulam Road, Hong Kong Special Administrative Region, China Correspondence to: Dr S S Lee, 5/F Yaumatei Jockey Club Clinic, 145 Battery Street, Yaumatei, Kowloon, Hong Kong SAR [email protected]

1 Esnault VL, Testa A, Audrain M, Roge C, Hamidou M, Barrier JH, et al. Alpha 1-antitrypsin genetic polymorphism in ANCA- positive systemic . Int 1993;43:1329–32. 2GriYth ME, Lovegrove JU, Gaskin G, White- house DB, Pusey CD. C-antineutrophil cyto- plasmic antibody positivity in vasculitis pa- tients is associated with the Z allele of alpha-1- antitrypsin, and P-antineutrophil cytoplasmic antibody positivity with the S allele. Nephrol Dial Transplant 1996;11:438–43. Figure 1 Skin specimen showing in within subcutaneous 3 Savige JA, Chang L, Cook L, Burdon J, Daska- tissues (haematoxylin and eosin, × 400). lakis M, Doery J. Alpha 1-antitrypsin defi- ciency and anti-proteinase 3 antibodies in anti- neutrophil cytoplasmic antibody (ANCA)- h. Anaemia was noted with a acute deterioration of renal function.5 Cyclo- associated systemic vasculitis. Clin Exp 9 Immunol 1995;100:194–7. count of 2500×10 /l, while the patient’s WBC phosphamide and PSL improved the symp- 4 Elsouki AN, Eriksson S, Lofberg , Nasberger L, count was high at 12×109/l. His platelet count toms, but cyclophosphamide was discontin- Wieslander J, Lindgren S. The prevalent clini- (304×109/l) was within the normal range. ued and the PSL dose was reduced because cal significance of á1-antitrypsin deficiency (PiZ) and ANCA specificities (proteinase 3, Biochemistry showed high levels of blood renal and skin showed cholesterol BPI) in patients with ulcerative colitis. Inflamm nitrogen (10.0 mmol/l of urea), creati- . Subsequently, the patient died of Bowel Dis 1999;5:246–52. nine (710 µmol/l), and CRP (11.3 mg/l). intractable cardiac failure. 5 Ying QL, Liang ZQ. Allelic frequencies of plasma á1-antitrypsin in Chinese. Sci Sin Complements components were within nor- Kaplan-Pavlovcic et al reported two cases 1984;27:161–8. mal ranges. PR3-ANCA and MPO-ANCA of renal failure with positive MPO-ANCA.6 6 World Health Organization. á1-Antitrypsin were high at 82E and 29E, respectively. The details are unknown for one patient. The deficiency: memorandum from a WHO meet- ing. Bulletin World Health Organ 1997;75: After admission to hospital, circulatory other patient was treated with corticosteroid 397–415. disturbance in his toes worsened. A diagnosis alone. This patient required haemodialysis 7 Lee SS, Lawton JWM. Heterogeneity of anti- of ANCA associated vasculitis was made and amputation of the toes. Although their PR3 associated disease in Hong Kong. Post- grad Med J 2000;76:287–8. based on systemic inflammatory findings and treatment did not result in the improvement high levels of WBC, CRP, PR3-ANCA, and of vasculitis, the combination of PSL and MPO-ANCA. High dose steroid treatment cyclophosphamide was eVective in our pa- A case of cholesterol was started. Biopsies of the right heel skin tient with ANCA. and thigh quadriceps showed cholesterol This result suggests that active treatment embolism with ANCA embolism (fig 1). However, PSL treatment with corticosteroid and cyclophosphamide treated with corticosteroid was continued together with three courses of should be considered in ANCA positive cases cyclophosphamide pulse treatment because of cholesterol embolism. and cyclophosphamide of persistent and high ANCA values. The treatment reduced the fever and toe E MAESHIMA We report a case of a patient with cholesterol Y YAMADA , and the ulcers improved. ANCA M MUNE embolism who showed positive for both gradually decreased to normal. The PSL myeloperoxidase antineutrophil cytoplasmic S YUKAWA dosage was reduced to 15 mg/day and the antibody (MPO-ANCA) and proteinase 3 Third Department of Internal Medicine, patient was discharged. antineutrophil cytoplasmic antibody (PR3- Wakayama Medical College, Cholesterol embolism predominantly af- ANCA) and who was treated with pred- 811-1, Kimiidera, Wakayama City, fects elderly men with a history of hyperten- nisolone (PSL) and cyclophosphamide. Wakayama 641-0012, Japan sion, atherosclerotic vascular diseases, and A 50 year old man underwent cardiac Correspondence to: Dr Maeshima catheterisation for back pain. The examina- renal insuYciency at the time of diagnosis. At tion disclosed 90% of the right coro- least 31% of patients had a preceding history nary and a saccular in the of use or the antecedent performance of a vascular procedure aVect- 1 Fine MJ, Kapoor W, Falanga V. Cholesterol thoracic . The patient underwent percu- 1 crystal embolization: a review of 221 cases in taneous transluminal coronary angioplasty ing the arterial circulation. The presence of the English literature. Angiology 1987;38:768– and the aneurysm was wrapped with an arti- these cholesterol embolisms within the vascu- 84. lar lumen triggers a characteristic localised 2 Lie JT. Vasculitis look-alikes and pseudovasculi- ficial . Postoperatively, the patient tis syndromes. Curr Diagnost Pathol 1995;2: had a fever, pleural eVusion, , inflammatory and endothelial vascular reac- 78–5. and increased (WBC) count, tion. The inflammatory changes resulting 3 Cappiello RA, Espinoza LR, Adelman H, Agui- C reactive protein (CRP), and serum creati- from cholesterol embolism may be responsi- lar J, Vasey FB, Germain BF. Cholesterol ble for many of the systemic manifestations embolism: a pseudovasculitic syndrome. Semin nine. Cultures of blood and pleural eVusion Arthritis Rheum 1989;18:240–6. exudate were negative. PSL 15 mg/day was such as fever, , , leuco- 4 Thadani R, Camargo C Jr, Xavier R, Fang L, started. However, acute progression of renal cytosis, , and a raised ESR. Thus Bazari H. Atheroembolic renal failure after failure required haemodialysis. cholesterol embolism is referred to as both invasive procedures. Natural history based on The patient was transferred to our hospital. vasculitis look-alikes2 and pseudovasculitic 52 histologically proved cases. Medicine (Balti- 3 more) 1995;74:350–8. Physical examination showed a temperature syndrome. The prognosis is poor, particu- 5 Peat DS, Mathieson PW. Cholesterol emboli of 38.0°C and of 178/98 larly in the presence of acute renal failure.4 may mimic systemic vasculitis. BMJ 1996;313: mmHg. Cyanosis was noted in both heels and Three ANCA positive cases56 of choles- 546–7. terol embolism have been described. Peat and 6 Kaplan-Pavlovcic S, Vizjak A, Vene N, Ferluga all toes with necrosis and ulcers at the tips of D. Antineutrophil cytoplasmic autoantibodies the fifth toes. He had an increased erythro- Mathieson reported an ANCA positive pa- in atheroembolic disease. Nephrol Dial Trans- cyte sedimentation rate (ESR) of 82 mm/1st tient with dyspnoea and haemoptysis after plant 1998;13:985–7.

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