Introduction
Background
Although renal vein thrombosis (RVT) has numerous etiologies, it occurs most commonly in patients with nephrotic syndrome (ie, > 3 g/d protein loss in the urine, hypoalbuminemia, hypercholesterolemia, edema).
The syndrome is responsible for a hypercoagulable state. The excessive urinary protein loss is associated with decreased antithrombin III, a relative excess of fibrinogen, and changes in other clotting factors; all lead to a propensity to clot. Numerous studies have demonstrated a direct relationship between nephrotic syndrome and both arterial and venous thromboses. Why the renal vein is susceptible to thrombosis is unclear.
The renal vein also may contain thrombus after invasion by renal cell cancer. Other less common causes include renal transplantation, Behçet syndrome, hypercoagulable states, and antiphospholipid antibody syndrome.
Pathophysiology
Hypercoagulability is the etiology for both arterial and venous thromboses. In the setting of malignant invasion of the vein by cancer, the presence of the tumor cells elicits thrombosis of the renal vein only.
Frequency
United States
Prevalence of RVT has been difficult to establish. Studies have shown a high degree of variability in the presence of RVT among patients with nephrotic syndrome, with reported rates of 5-62%.
Mortality/Morbidity
- The morbidity and mortality of RVT usually is secondary to the effects of nephrotic syndrome (including arterial thrombosis), renal dysfunction and/or failure, or the complications resulting from thromboembolism. If the etiology of the RVT is malignancy, morbidity and mortality are a result of either thromboembolism or the cancer itself. In the setting of transplantation, RVT may lead to loss of the graft. If the RVT eventuates from the other causes discussed, thromboembolism is the source of complications.
Race
- No race predilection exists.
Sex
- No specific numbers are available. However, theoretically, membranous nephropathy, the most commonly associated disease for RVT, has a male-to-female ratio of 2:1. Therefore, a male preponderance may exist.
Age
- Age is a factor in RVT only as associated with any age-related risk of glomerular disease. For example, membranous nephropathy, the lesion most associated with RVT, is the most common cause of nephrotic syndrome in adults, but it is rare in children. Membranous nephropathy peaks in the fourth through sixth decade, thus making RVT more likely in this specific age group. However, exact incidence or prevalence is not available.
- RVT from renal cell carcinoma occurs in older age groups.
Clinical
History
The presentation of RVT is variable, and patients may be asymptomatic. When RVT occurs as a result of malignancy, the signs of the renal malignancy (eg, hematuria, weight loss) predominate.
- The more common chronic form of RVT generally is covert.
- The less frequent acute form usually occurs in younger patients, with flank pain and macroscopic hematuria.
- Patients may present with thrombosis and/or embolism.
Physical
Observe for signs of nephrotic syndrome (edema or anasarca).
Causes
- In patients who are nephrotic, the most common underlying nephropathy associated with RVT is membranous nephropathy. For a renal biopsy of membranous nephropathy, see Image 1. The tumor association for RVT is renal cell carcinoma. However, most cases of membranous nephropathy are idiopathic.
- RVT also may be the result of nephrotic syndrome from membranoproliferative glomerulonephritis, minimal change disease, rapidly progressive glomerulonephritis, amyloid, focal sclerosis, or lupus nephritis. RVT is more common in patients with primary rather than secondary nephropathy.
- Findings relative to the causative disease may be present (eg, systemic lupus erythematosus [SLE]/antiphospholipid antibody syndrome, cancer).
- Theories for the putative relationship between nephrotic syndrome and RVT have evolved. Initially, nephrotic syndrome was believed to be a consequence of RVT. However, this presumed sequence was incorrect.
- Experimentally induced RVT causes only mild proteinuria.
- RVT in the absence of nephrotic syndrome has been reported in the surgical literature.
- Nephrotic patients with RVT who have undergone histologic evaluation show evidence of an identifiable glomerulopathy.
- RVT is known to occur after the onset of nephrotic syndrome. Thus, nephrotic syndrome is not a direct result of RVT but rather leads to RVT.
- SLE also has been associated with RVT.
- In general, patients with lupus and documented RVT have membranous lupus nephritis (World Heath Organization class V).
- Generally, thrombophlebitis and circulating anticoagulants (anticardiolipin antibodies) are believed to be much less important than nephrotic syndrome as predisposing factors of RVT in SLE.
- RVT is an uncommon but definite problem in neonates. A possible association exists between RVT and the factor V Leiden mutation in this age group.
- Other diseases or situations that have been associated with RVT include antithrombin III deficiency, protein C or S deficiency, antiphospholipid antibody syndrome, pregnancy or estrogen therapy (all hypercoagulable states), renal vein invasion by malignant cells, post–renal transplantation, Behçet syndrome, and extrinsic compression (eg, lymph nodes, tumor, retroperitoneal fibrosis, aortic aneurysm). Other than renal cell cancer, the other associations are uncommon.
- Trauma, ingestion of oral contraceptive agents, dehydration (infants), and steroid administration also have been associated with RVT.
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| References |
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References
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Further Reading
Keywords
renal vein thrombosis, RVT, nephrotic syndrome, hypercoagulable state, clotting, clot, hypoalbuminemia, hypercholesterolemia, arterial thrombosis, renal dysfunction, renal failure, thromboembolism
