AA amyloidosis in Takayasu's arteritis—long-term survival on maintenance haemodialysis

Yoshifumi Wada, Hidemi Nishida, Keisuke Kohno, Osamu Tamai, Masahisa Fujisawa, Seiya Katoh, Minoru Morimatsu and Seiya Okuda

Department of Internal Medicine III, Department of Pathology Kurume University School of Medicine, Kurume, Fukuoka, Japan

Correspondence and offprint requests to: Yoshifumi Wada, MD, Department of Internal Medicine III, Kurume University School of Medicine 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan.

Keywords: AA amyloidosis; long-term survival; Takayasu's arteritis; haemodialysis; regression



   Introduction
 Top
 Introduction
 Case
 Discussion
 References
 
AA amyloidosis occurs in association with chronic inflammatory disorders, chronic infection and, occasionally, neoplastic diseases. The median survival time from diagnosis of AA amyloidosis varies from 30 to 60 months [1,2]. We observed a patient with renal AA amyloidosis secondary to Takayasu's arteritis. There was no progression of, and potential recovery from, systemic amyloidosis after the induction of maintenance haemodialysis. The patient survived for 16 years on maintenance haemodialysis.



   Case
 Top
 Introduction
 Case
 Discussion
 References
 
A 23-year-old woman was admitted for proteinuria and hypertension. She had suffered from toxaemia of pregnancy during her first pregnancy a few months before admission. On admission, her body temperature was 36.6°C and blood pressure 150/84 mmHg. Physical examination was unremarkable. The patient did not have goitre or macroglossia, but did have hepatosplenomegaly. Urinalysis showed 3+ proteinuria. Neither erythrocytes nor casts were found in the urinary sediment. A 24-h urine collection contained 6 g of urinary protein. The results of laboratory investigations were haemoglobin 9.7 g/dl, haematocrit 35%, white blood cells count 7700/µl, serum creatinine 1.4 mg/dl, total protein 5.8 g/dl, albumin 3.3 g/dl, total cholesterol 110 mg/dl and erythrocyte sedimentation rate (1 h) 24 mm. C-reactive protein, antinuclear antibody and rheumatoid factor were negative. No abnormal serum proteins were detected by immunoelectrophoresis. No signs of cardiac disorder were found by electrocardiography and echocardiography. Renal biopsy revealed amyloid deposition in all glomeruli and tubular basement membranes by Congo red and periodic acid–Schiff staining (Figure 1Go). The amyloid deposits disappeared upon treatment of Congo red-stained kidney sections with permanganate. These deposits were positive for AA protein by immunostaining. Electron micrography revealed non-branching amyloid fibrils disposed in random array in the glomeruli (Figure 2Go). Amyloid deposits were also detected in the small vessels of the gastric mucosa. No amyloid deposits were found in skin biopsy or in bone marrow aspirate. The cause of the amyloidosis could not be identified despite extensive examination.



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Fig. 1. Renal biopsy specimen, showing extensive amyloid deposition in most of glomeruli and a part of the tubules (periodic acid–Schiff stain x400).

 


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Fig. 2. Electron micrograph study showing non-branching amyloid fibrils arranged in a random array in the glomerulus (x100 000).

 
The patient gradually developed renal failure and was admitted for maintenance haemodialysis 7 years after the diagnosis. On admission, a bruit was audible in the neck and subclavicular area. Marked intimal thickening and narrowing of both carotid arteries were documented by echogram and computed tomography (Figure 3Go). Similarly, magnetic resonance angiography showed narrowing of the carotid arteries on both sides (Figure 4Go). These findings were compatible with Takayasu's disease. The patient did not complain of any symptoms pointing to this diagnosis such as fever, neck pain or chest pain, and did not show any abnormal laboratory findings including leukocytes, erythrocyte sedimentation rate and C-reactive protein at that time. She was left without treatment and has been doing well on maintenance haemodialysis for 9 years (Figure 5Go). Recently, no amyloidosis deposits were detected in the gastric mucosa. Although we do not have a biopsy at baseline, this finding would be compatible with regression of systemic AA amyroidosis.



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Fig. 3. Computed tomography, showing the narrowing and wall thickening of the bilateral common carotid arteries.

 


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Fig. 4. Magnetic resonance angiography showing a narrowing of the bilateral common carotid and left subclavian arteries.

 


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Fig. 5. Clinical course.

 


   Discussion
 Top
 Introduction
 Case
 Discussion
 References
 
Our patient suffered from AA amyloidosis, but a primary disease causing amyloidosis could not be identified initially. At the time when maintenance haemodialysis was started, a bruit was audible in the neck and subclavicular area, and the angiographic findings fulfilled the criteria for Takayasu's arteritis. The primary disease seemed to be Takayasu's arteritis, although this was apparently no longer active at that time.

Takayasu's arteritis is an inflammatory and stenotic disease involving large and medium size arteries. Typically, the aortic arch and major branches are affected. It is an uncommon disease affecting mainly young women in South East Asia. Glomerular lesions associated with Takayasu's arteritis include mesangial proliferative, membranoproliferative and crescentic glomerulonephritis [3,4] and, rarely, amyloidosis [57]. AA amyloidosis is infrequently associated with Takayasu's arteritis. Remission of nephrotic syndrome was reported after use of prednisolone and dimethylsulfoxide in a patient with renal amyloidosis associated with Takayasu's arteritis [8]. As AA amyloidosis is evidently a dynamic process, progression to secondary amyloidosis may be arrested (or possibly even reversed) by treatment of the underlying disease [9]. It is now clear that aggressive anti-inflammatory therapy can improve survival and preserve organ function in AA amyloidosis associated with inflammatory conditions. Triger and Joekes found evidence of clinical regression of renal amyloidosis in five out of 31 patients with secondary amyloidosis after adequate treatment of the underlying chronic infection or chronic inflammation [1].

Recent advances in haemodialysis and transplantation therapy have increased the life expectancy of amyloidotic patients. Get et al. reported that the median survival time of dialysis patients with primary systemic amyloidosis was 8.2 months [10]. The 3-year survival rate was 37% for patients with secondary amyloidosis on dialysis, as reported by Ylinen et al. [11]. In contrast, Martinez-Vea reported a much better median survival of 52 months for dialysis patients with systemic amyloidosis, and no difference between patients with primary or secondary amyloidosis [12].

The prognosis of patient with AA amyloidosis who are on dialysis depends on the activity of the primary disease and the involvement of organs other than the kidney. Common problems in amyloidosis are persistent hypotension and intolerance to ultrafiltration, problems in the creation and maintenance of arteriovenous fistulae, and a high mortality rate due to cardiac complications. This patient has survived for 9 years already on haemodialysis without such problems. Her cardiac and endocrine functions have been normal during these 9 years.



   References
 Top
 Introduction
 Case
 Discussion
 References
 

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Received for publication: 13. 4.99
Accepted in revised form: 28. 5.99





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