Long-standing and intractable ascites involved in renal vein thrombosis of a patient with systemic lupus erythematosus

S. YAMASAKI, Y. KAWABE, H. NAKAMURA, H. ISHIKAWA, A. KAWAKAMI, K. MIGITA, A. KURATA and K. EGUCHI

The First Department of Internal Medicine, Nagasaki University School of Medicine, Sakamoto 1-7-1, Nagasaki 852–8501, Japan

SIR, A 34-yr-old man was referred to Nagasaki University Hospital for long-standing intractable ascites in 1998. In 1985, he developed pulmonary congestion with peripheral oedema, malar rash, and arthralgia. Laboratory study showed positive antinuclear antibodies, and decreased amounts of serum protein and complements with proteinuria (4.5 g/day). He was diagnosed as having systemic lupus erythematosus (SLE) because he satisfied four of 11 American Rheumatism Association (ARA) criteria for SLE. He was also diagnosed as having nephrotic syndrome because of massive proteinuria and decreased serum albumin level. Renal biopsy revealed lupus nephritis defined as WHO class IVb. Computed tomography showed left renal vein obstruction. He was treated with intravenous methylprednisolone (1 g daily) for 3 successive days, and then per os prednisolone (60 mg/daily for 6 months and tapered), cyclophosphamide (100 mg daily for 9 months and tapered). His symptoms disappeared and there has been remission in SLE activity since 1991.

In 1994, he developed insidious ascites. Extensive examination showed only left renal vein thrombosis (RVT) with more collateral vessels than previously demonstrated 9 yr earlier. He was treated with daily 5 mg of prednisolone at that time. As he complained of anorexia and diarrhoea, the drainage of 5 l ascites twice a week was necessary. This procedure was continued for 4 yr.

On admission, the patient was a slender man with markedly demonstrable abdominal fluid waves. His blood pressure was 102/66 mmHg. His breath and heart sounds were normal. There was no peripheral oedema. Total urine protein was 60 mg/day, haemoglobin 14.6 g/dl, white blood cell count 4900/µl with 23% lymphocytes. Mild elevation of liver enzymes including AST (30 IU/l) and ALT (34 IU/l) was found. Serum albumin was 3.7 g/dl. The blood urea nitrogen level was 20.0 mg/dl and the creatinine level was 1.0 mg/ldl. Creatinine clearance was 39 ml/min. An extensive coagulation test revealed a normal coagulation including lupus anticoagulant and anticardiolipin antibody. No autoantibodies to nuclear antigens and double-stranded DNA were detected. The concentrations of serum complements were within normal ranges. The ascites fluid was transudatory and extensive cultures were negative.

Computed tomography and angiography (Fig. 1Go) revealed occlusion of the left renal vein accompanied by collateral vessels around the left kidney. There were no obstructions in other veins or inferior vena cava. Laparoscopic examination showed a normal liver, and biopsy specimens of the peritoneum failed to prove peritonitis. As there were no findings for active SLE, the dose of prednisolone was not increased.



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FIG. 1. (A) Right renal angiography showing normal venous flow. (B) Left renal angiography showing severe obstruction of the left renal vein with collateral vessels around a hypertrophic kidney. Delayed blood return from the left renal vein to the inferior vena cava was detected as compared with the right renal vein.

 
Finally, we chose a Denver shunt operation [1] to bring the ascites to superior vena cava. After the Denver peritoneovenous shunt (DPVS) was inserted, urine volume increased and the patient's abdominal girth decreased without ascites drainage. Creatinine clearance increased from 39 ml/min to 46 ml/min. The abdominal symptoms of the patient disappeared for 1 yr.

The prevalence of ascites in SLE has been reported to be 11% [2]. When it does occur, it usually reflects kidney involvement with nephrotic syndrome or congestive heart failure. Our patient's ascites was transudatory and extensive examination revealed only an occlusion of the left renal vein. These suggest that his ascites was complicated by RVT.

RVT has been found in 22% of nephrotic syndrome [3]. It has been reported that RVT is rarely complicated by ascites. Multiple factors might be indicated, including nephrotic syndrome [4], hypercoagulation state, and anticardiolipin antibody [5]. Antecedent thrombophlebitis has been considered to be a high risk for RVT in SLE [3]. However, Lai and Lan have reported that nephrotic syndrome could be a distinct risk factor for RVT in SLE patients [6]. Our SLE patient had nephrotic syndrome, but no history of thrombophlebitis and positive anticardiolipin antibody. These results suggest that nephrotic syndrome might have been a distinct risk factor for RVT in our patient. Harrison et al. [7] described two groups of patients with RVT. The first group had a sudden onset of the lumbar or frank pain, while the second group had only nephrotic syndrome without symptoms. It seems likely that our patient belongs to the second group.

As his ascites was suspected of being complicated with RVT, we did not increase the dose of prednisolone, administer immunosuppressive drugs or anticoagulant drugs which have been reported to be received only when extensive thrombosis was found, or with repeated episodes of pulmonary thromboembolism in RVT [3]. The effectiveness of a peritoneovenous shunt in relieving ascites refractory to conventional treatment has been established [8, 9]. We believe that the risk of the DPVS for our patient's ascites was low because he had mild renal dysfunction and no coagulation abnormality [10]. But it was necessary to protect him against infection and shunt failure due to coagulation.

Notes

Correspondence to: K. Eguchi. Back

References

  1. Hober PC, Turner WW, Valentine RJ. Successful use of the Denver peritoneovenous shunt in patients with nephrogenic ascites. Surgery1987;101:161–4.[ISI][Medline]
  2. Dubois EL, Tuffanelli DL. Clinical manifestation of systemic lupus erythematosus. Computer analysis of 520 cases. J Am Med Assoc1964;190:104–11.[ISI]
  3. Mintz G, Acevedo-Vazquez E, Gutierrez-Espinosa G, Avelar-Garnica F. Renal vein thrombosis and inferior vena cava thrombosis in systemic lupus erythematosus. Frequency and risk factors. Arthritis Rheum1984;27:539–44.[ISI][Medline]
  4. Appel GB, Williams GS, Meltzer JI, Pirani CL. Renal vein thrombosis, nephrotic syndrome, and systemic lupus erythematosus. Ann Int Med1966;85:310–7.
  5. Averbuch M, Levo Y. Budd-Chiari syndrome as the major thrombotic complication of systemic lupus erythematosus with the lupus anticoagulant. Ann Rheum Dis1986;45:435–7.[Abstract]
  6. Lai NS, Lan JL. Renal vein thrombosis in Chinese patients with systemic lupus erythematosus. Ann Rheum Dis1997;56:562–4.[Abstract/Free Full Text]
  7. Harrison CV, Milne MD, Steiner RE. Clinical aspect of renal vein thrombosis. Q J Med1956;25:285–98.
  8. Holm A, Halpern NB, Aldrete JS. Peritoneovenous shunt for intractable ascites of hepatic, nephrogenic, and malignant causes. Am J Surg1989;158:162–6.[ISI][Medline]
  9. Berkowitz HD, Mullen JL, Miller LD, Rosato EF. Improved renal function and inhibition of renin and aldosterone secretion following peritoneovenous (LeVeen) shunt. Surgery1978;84:120–6.[ISI][Medline]
  10. Smith RE, Nostrant TT, Erchauser FE, Wilson JP, Knol JA, Strodel WE. Patient selection and survival after peritoneovenous shunting for nonmalignant ascites. Am J Gastroenterol1984;79:659–62.[ISI][Medline]
Accepted 22 October 1999





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