A case of Behçet's disease with pulmonary arterial aneurysm and secondary amyloidosis

K. Aksu, H. Koçanaogullari, G. Keser, V. Inal, Y. Kabasakal, F. Oksel, G. Gümüsdis and E. Doganavsargil

Department of Internal Medicine, Division of Rheumatology, Ege University School of Medicine, Bornova, I·zmir, Turkey

SIR, Behçet's disease (BD) was first defined by Hulusi Behçet after he had examined patients with oral and genital ulcers and eye involvement. During the following years, BD was accepted as a multisystemic vasculitis involving locomotor, neurological, gastrointestinal and vascular systems. Venous involvement is more common than arterial involvement, which is rarely reported. Secondary amyloidosis is reported to occur rarely during the progression of BD [1]. It has been reported that vascular involvement and arthritis are the strongest predictors of amyloidosis in BD [2]. Supporting this view, we present a Behçet's disease patient with vascular involvement (asymptomatic pulmonary arterial aneurysm) and secondary amyloidosis.

A 35-yr-old male patient with no established disease or symptoms had recurrent oral aphthae at the age of 27 yr followed in the next year by genital ulcerations. He also had an attack of iridocyclitis and thrombophlebitis after being diagnosed as having BD with positivity for the pathergy reaction. Six years after his first symptoms appeared, he was admitted to Ege University Hospital with swelling of both legs. Pretibial oedema (++) and scrotal scars were observed in the physical examination. Laboratory analyses revealed the following: erythrocyte sedimentation rate 74 mm/h; white blood cell count 8100/mm3; haematocrit 42%; platelet count 207000/mm3; urea concentration 8.2 mmol/l; creatinine concentration 70.7 mmol/l; albumin concentration 26 g/l; globulin concentration 29 g/l; total cholesterol concentration 7.2 mmol/l; triglyceride concentration 3.1 mmol/l. Daily protein excretion was 4.2 g/day. Renal parenchymal echogenicity was mildly increased (grade I) on ultrasonography.

The findings of peripheral oedema, proteinuria and hyperlipidaemia suggested the presence of nephrotic syndrome. Secondary amyloidosis was thought to be the possible cause of nephrotic syndrome, and abdominal biopsy of subcutaneous fat tissue was performed. Positivity with Congo red was determined histologically. A renal biopsy was not performed for two reasons: first, there was a high risk of bleeding in patients with amyloidosis [3]; secondly, our experience supported the increased incidence of aneurysmal changes following invasive procedures in Behçet's patients, especially those with pathergy positivity.

Moreover, increased density in the left hilar region was revealed on the plain chest X-ray (Fig. 1Go). Computed tomography (CT) with a contrast angiogram was performed and an aneurysm of the left pulmonary artery, 2 cm in diameter, was detected. Treatment comprised pulsed intravenous cyclophosphamide 1 g monthly for 6 months, then 1 g every 2 months. Colchicine 1.5 mg/day and acetyl salicylic acid 100 mg/day were also started. No increase in the diameter of the aneurysm was detected on the angio-CT image obtained 20 months after the start of therapy. However, at 20 months we found that the patient's proteinuria had progressed.



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FIG. 1.  Vascular opacity (arrow) in the left hilar region.

 
Secondary amyloidosis has been observed in chronic inflammatory diseases, such as ankylosing spondylitis, rheumatoid arthritis and osteomyelitis, and it may also be seen in BD [1, 2]. In the series of Filali-Ansary [1], amyloidosis was reported in only one Behçet's disease patient out of 162. Among our 184 Behçet's disease patients, there are three with amyloidosis. Melikoglu et al. [2] found that the incidence of amyloidosis in BD was 14/4000 (0.35%) in their larger series. They suggested that peripheral and pulmonary artery involvement and arthritis were the strongest predictors of amyloidosis in BD [2]. The coexistence of amyloidosis and pulmonary arterial involvement in our patient supports this view.

The incidence of vascular involvement in BD is 25% in the data of the Istanbul Behçet's Disease Research Institute. The rate of vascular involvement is 37% (68/184) at our centre. The most common symptom of pulmonary arterial aneurysm is haemoptysis. Fortunately, the diagnosis of pulmonary artery aneurysm in our patient was made very early, before haemoptysis occured. The patient responded very well to immunosuppressive treatment, and remained well at the 20-month follow-up.

In conclusion, despite the presence of concurrent amyloidosis, pulmonary artery aneurysm was treated successfully in this patient, probably because of the early diagnosis.

Notes

Correspondence to: K. Aksu, 80 Sokak No. 27/3, 35040 Bornova, Izmir, Turkey. Back

References

  1. Filali-Ansary N, Tazi-Mezalek Z, Mohattane A et al. Behcet's disease. 162 cases. Ann Med Interne1999;150:178–88.[ISI][Medline]
  2. Melikoglu M, Altiparmak MR, Fresko I et al. A reappraisal of amyloidosis in Behçet's syndrome. Rheumatology2001;40:212–5.[Abstract/Free Full Text]
  3. Yood RA, Skinner M, Rubinow A, Talarico, Cohen AS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc1983;249:1322–4.[Abstract]
Accepted 12 February 2002