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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 3  |  Page : 566-570
Unusual presentation of renal vein thrombosis with pulmonary artery embolism

Department of Nephrology and Internal Medicine, Bahrain Specialist Hospital, Manama, Kingdom of Bahrain

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Date of Web Publication24-Apr-2013


A young 23-year-old male patient presented with a two-day history of right flank pain. He had no history of any significant illnesses in the past. His investgations showed nephrotic range proteinuria with hypoalbuminemia. The patient developed cough and shortness of breath after having a left kidney biopsy. He did not respond to regular respiratory tract infection treat­ment. The kidney biopsy revealed membranoproliferative glomerulonephritis. Further investigations for the cough showed thromboembolism of the posterior and lateral basal segments of the right lower lobe. Moreover he was found to have thrombosis of the right upper pole renal vein. The patient was started on full anticoagulation along with three days pulse steroid, followed by 1 mg/kg oral steroid. Clinical improvement was noticed within 48 h. After eight weeks the proteinuria decreased from 8.5 gm/day to 1.1 gm/day. The kidney function was normal with eGFR 145 mL/min through the course of the disease. This case represent one of the unusual presentation of nephrotic syndrome with pulmonary and renal vascular thromboembolic events. The response to the combination of anticoagulation and steroid was remarkable.

How to cite this article:
Mzayen K, Al-Said J, Nayak-Rao S, Catacutan MT, Kamel O. Unusual presentation of renal vein thrombosis with pulmonary artery embolism. Saudi J Kidney Dis Transpl 2013;24:566-70

How to cite this URL:
Mzayen K, Al-Said J, Nayak-Rao S, Catacutan MT, Kamel O. Unusual presentation of renal vein thrombosis with pulmonary artery embolism. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2022 Sep 25];24:566-70. Available from: https://www.sjkdt.org/text.asp?2013/24/3/566/111068

   Introduction Top

Thromboembolic episodes are one of the most serious complications in patients with nephrotic syndrome, with an overall reported inci­dence of more than 25%. [1] Venous thrombosis, including renal vein thrombosis (RVT), pulmonary thrombosis and deep vein thrombosis (DVT), are common. Arterial thrombosis is less common, but is a serious complication with significant morbidity. Unusual sites of thrombosis had been reported, such as portal circulation, cerebral and coronary arteries. We would like to present this case of a young gentleman with nephrotic syndrome secondary to membranoproliferative glomerulonephritis. He presented with a clinical picture of only right flank pain. On investigation, he was found to have RVT and pulmonary embolism. His clinical condition improved within one week of using anticoagulant and steroids. One month later, the proteinuria decreased from 8.5 g to 1.1 g/24 h and the circulation was restored back to normal in the kidney and the lung.

   Case Report Top

Mr. Y.J. was a healthy 23-year-old man. He had an earlier admission to another hospital for having dull right flank pain of two days' du­ration with no radiation and no associated symptoms. It was for the first time that he experienced this symptom. He had no abnor­mal urinary or bowel symptoms. He did not have any history of leg swelling, facial puffiness or hematuria. There was no history of skin rash. He did not have any joint pain or swelling. He had no previous upper respiratory tract or skin infection. His laboratory tests revealed hemoglobin of 124 g/L (12.4 g/dL), hematocrit 0.379 L/L and white blood cell (WBC) count 8200 cells/ mm 3 with normal differential count. Urine analysis showed +++ protein, 10-12 red blood cells (RBC)/HPF, 8-10 WBC/HPF and no casts. Urine culture was negative. His serum creatinine was 61.9 μmol/ L (0.7 mg/dL) and the estimated glomerular fil­tration rate (eGFR) was 145 mL/ min. Fasting blood sugar was normal. The initial diagnosis was renal colic. Further investigations were carried out, because he did not improve, and they showed normal liver function test. Total serum protein was 61 g/L (6.1 g/dL) and se­rum albumin was 20 g/L (2.0 g/dL). The 24-h urine protein was 8.5 g/24 h. Renal ultrasound was normal with no urinary calculi. Because of the significant proteinuria and negative evi­dence for any stones or infection, a left renal biopsy was performed and it revealed membranoproliferative glomerulonephritis. Two days later, the patient started to have fever and right-sided chest pain, with mild dyspnea and hemoptysis. He was diagnosed and treated as a case of respiratory tract infection.

The patient did not improve and continued to have fever and right-sided pleuritic lower chest pain with hemoptysis. Physical examination at that point revealed BP of 150/80 mmHg, pulse 100/min regular, RR 27/min and tempe­rature 38.2°C. Chest exam revealed decreased air entry in the right base with inspiratory crackles. Mild right hypochondriac tenderness was found and the liver was felt 2 cm below the costal margin with no splenomegaly. He did not have ascites or lower extremity edema. Further investigations showed high serum fibrinogen >23 μmol/L (>800 mg/ dL), cholesterol of 5.9 mmol/L (230 mg/dL), triglycerides 2.25 g/L (225 mg/dL) with LDL of 3.3 mmol/L (128 mg/dL). WBC this time was 14200 cells/mm 3 with normal differential count. Urine analysis showed pH of 7, SG 1.025, ++ protein, 7-10 RBC/HPF and 12-15 WBC/HPF with no casts. Serum creatinine was 70.7 μmol/L (0.8 mg/dL), urea was 5.3 mmol/L (15 mg/dL) with e-GFR 145 mL/min/ 1.73 m 2 , creatine phosphorkinase (CPK) was 41 IU/L, creatine phosphokinase-MB (CPK-MB) was 1 IU/L, LDH was 191 IU/L, amylase was 45 IU/L, total serum protein was 61 g/L (6.1 g/dL) with 20 g/L (2.0 g/dL) albumin, ESR was 40 mm/h, CRP was 185 mg/L, ALT was 39 IU/L and alkaline phosphatase was 88 IU/L. Chest X-ray showed infiltrates in the right lower lung zone with blunting of the costophrenic angle. A work-up for secondary causes of membranoproliferative glomerulonephritis was performed, including hepatitis B surface antigen (HbsAg), anti- hepatitis C (HCV) antibody, human immunodeficiency virus (HIV) antibody, anti-nuclear antibodies(ANA), anti-double-stranded DNA (Anti-Ds DNA) antibody, anti-phospholipid antibody and complement C3 and C4 levels. All serological tests were nor­mal. C3 was 2.16 g/L (216 mg/dL) and C4 was 0.4 g/L (40 mg/dL). Cultures of the blood, urine and stool were also negative. Ultrasound of the kidneys was repeated and it revealed that the right kidney was 12.9 cm in length with 1.9 cm parenchymal thickness. There was normal echogenicity, no hydronephrosis and no masses. The left kidney was 13.4 cm in length with 2.4 cm parenchymal thickness with normal echogenicity. There were no masses and no obstruction. Contrast computerized tomogram (CT) scan for the chest and upper abdomen [Figure 1], [Figure 2] and [Figure 3] revealed thromboembolism of the right lower pulmonary artery branches with collapse of the posterior and lateral basal segments of the right lower lobe. Thrombosis of the right upper pole renal vein with perfusion defect was also noticed.
Figure 1: CT scan of the kidneys before therapy. Arrow at the thrombus.

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Figure 2: CT scan of the kidney before therapy. Arrow at the thrombus

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Figure 3: Chest CT scan before therapy. Arrow at the thrombus.

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Heparin was given as IV drip after a bolus dose of 5000 IU. The dose was titrated accor­ding to the partial thromboplastin time (PTT). Pulse steroid was also given in a dose of 250 mg IV/day for three doses followed by oral steroids at 1 mg/kg. Subsequently, warfarin was started and the dose was adjusted to maintain an international normalized ratio (INR) between 2.0 and 3.0. Perindopril 10 mg and torvastatin 10 mg were added few days later. The patient improved clinically after 48 h. Eight weeks later, the patient was stable. A CT scan of the abdomen and pelvis showed resolution of the RVT and the pulmonary embolization with restored normal filling and wash out of the contrast from both organs. His labo­ratory tests showed significant improvement. The proteinuria decreased to 1.1 g/24 h, CRP decreased to 1.61 mg/L and ESR dropped to 18 mm/first hour. His renal function remained normal.

   Discussion Top

Venous thrombosis has been recognized as one of the complications of nephrotic syn­drome since 1840. [1] Although it was initially thought to be the cause rather than the effect of nephrotic syndrome, today, it is clear that the hypercoagulable state is the result of several pathophysiological alterations that occur in nephrotic syndrome, favoring a pro-thrombotic status. These include (1) increased hepatic synthesis of fibrinogen, lipoprotein and coa­gulation factors like factor V, VII and VIII, (2) urinary losses of clotting inhibitors, zymogens and plasminogens, (3) increased platelet aggre­gation, (4) hemoconcentration due to decreased plasma oncotic pressure and loss of fluid from the intravascular compartment, (5) drugs as diuretics and steroids, [2],[3],[4],[5] (6) hypoalbuminemia increases the availability of free arachidonic acid, normally albumin bound, resulting in in­creased formation of pro-aggregatory agent thromboxane A2 in platelets causing platelet aggregation and (7) markedly elevated levels of LDL cholesterol may increase the in vitro platelet aggregation, as observed in patients with familial hypercholesterolemia.

Both arterial as well as venous thromboses are described. The incidence of RVT ranges from 5% to 62%, and pulmonary thrombosis can occur in as many as 50% of the affected patients. [2],[3],[4],[5],[6] Uncommon sites of venous throm­bosis are the portal veins and cerebral veins in children. [7],[8],[9] Arterial thromboembolism, although less common, has been described in the coronary, mesenteric and femoral arteries. [6],[10] Thromboembolism is seen in minimal change disease, membranoproliferative-glomerulonephritis and amyloidosis. Initially, it was thought to be present only in membranous nephropathy.

RVT with pulmonary embolism in nephrotic syndrome was described in earlier reports. [11] What makes our case unique is that the symp­toms that the patient presented with are only flank pain. He did not have any clue in the history or physical exam that would suggest having nephrotic syndrome. With this presen­tation, it could easily be diagnosed as a renal colic case in a young patient. A similar pre­vious presentation was published by Decoster et al. [12] The association of biochemical picture of nephrotic syndrome and negative work-up for urinary tract infection or renal calculi drew suspicion to some other underlying renal dis­ease. Predictors of increased risk of throm­bosis include low serum albumin (<20 g/L), high rates of protein excretion (>10 g/24 h), high fibrinogen levels and low anti-thrombin III levels (<75%). [13] Increased incidence of thrombo-embolic complications in nephrotic syndrome has raised the argument regarding the use of anticoagulation prophylaxis and therapy.

In our case, the pulmonary embolism is most probably secondary to dislodged thrombus from the renal vein. The likelihood of primary pulmonary thrombosis however cannot be ruled out. A retrospective study of venous thrombo-embolism in patients hospitalized with nephrotic syndrome by Kayali et al [14] reported that out of more than 9000 patients diagnosed to have nephrotic syndrome from 1979 to 2005, 1.5% had evidence of deep venous thrombosis, 0.5% had pulmonary embolism and <0.5% had RVT. The authors reported that pulmonary embolism, if it occurs, was more likely to be due to deep venous thrombosis and not due to RVT. This study, however, reported clinically documented episodes of thromboembolism.

The published data did not show additional advantage in terms of survival or kidney func­tion when we compared thrombolysis or thrombectomy over anticoagulants alone. Thrombolytic therapy is recommended in cases of: bilateral DVT with acute renal failure, large clot size with high risk of acute embolic events and in recurrent pulmonary embolism in the absence of thrombolytic contraindications. [15]

The use of steroids and their effect on throm­bosis in patients with nephrotic syndrome, who have high levels of proteinuria, is contro­versial. Because of the elevation in the inflam­matory markers, nephrotic range proteinuria and the kidney biopsy result for this patient, we elected to start pulse steroids and continue steroids on oral regimen in addition to the anticoagulation therapy. We believe that part of the patient's improvement, that was noticed clinically and biochemically, is related to the steroids. The beneficial effect of adding ste­roid to the anticoagulation was also noticed in a previous published case with nephrotic syn­drome and RVT. [16] Randomized controlled trials are required to test the role of steroids in similar cases.

It was found that the recurrence rate for RVT was 1.0/100 patient years. [17] It is less than the recurrence rate for DVT patients. The prog­nosis of patients with nephrotic syndrome who develop RVT is unclear. There are no large studies that have addressed this issue. Laville et al [18] followed-up 27 patients with documen­ted RVT to evaluate their long-term prognosis. Eleven patients (40%) died within six months mainly from hemorrhagic complications or severe sepsis. Survivors were followed-up to 19 years and their renal function did not change throughout the follow-up period. The main prognostic factors reported were initial renal function and the cause of nephropathy. Patients with membranous glomerulopathy had a signi­ficantly better prognosis. [18]

The basic conclusion in this case is that in pa­tients with loin pain with no signs of infection on urine analysis and no stones on imaging, if presenting with significant proteinuria, the diagnosis of RVT should be kept in mind. The second point that we would like to emphasize is the significant clinical improvement after using a combination of pulse steroid with full therapeutic anticoagulation. This observation was noticed in an earlier case report as well. To confirm the benefit of steroid in RVT needs further studies.

   References Top

1.Keusch G. Thrombotic complications in the nephrotic syndrome. Schweiz Med Wochenschr 1989;119:1080-5.  Back to cited text no. 1
2.Cameron JS. The nephrotic syndrome and its complications. Am J Kidney Dis 1987;10:157-71.  Back to cited text no. 2
3.Kendall AG, Lehmann RC, Dossetor JB. Nephrotic syndrome: A hypercoagulable state. Arch Intern Med 1971;127:1021-7.  Back to cited text no. 3
4.Llach F. Thromboembolic complications in nephritic syndrome. Coagulation abnormalities, renal vein thrombosis and other conditions. Postgrad Med 1984;76:111-4.  Back to cited text no. 4
5.Llach F. Hypercoagulability, renal vein throm­bosis and other thrombotic complications of nephritic syndrome. Kidney Int 1985;28:429-39.  Back to cited text no. 5
6.Schwartz JC, Wyrzykowski AD, Dente CJ, Nicholas JM. The nephrotic syndrome: An unusual cause of multiple embolic events. Vasc Endovascular Surg 2009;43:207-10.  Back to cited text no. 6
7.Sun L, Xu C. Portal vein thrombosis as the first sign of nephrotic syndrome. Nat Clin Pract Nephrol 2008;4:342-5.  Back to cited text no. 7
8.Varghese J, Mathew A, Seethalekshmy NV, Kurian G, Unni VN. Isolated portal vein thrombosis in nephrotic syndrome. Indian J Nephrol 2007;17:26-8.  Back to cited text no. 8
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9.Al Fakeeh KN, Al Rasheed SA. Cerebral venous thrombosis in the nephrotic syndrome. Saudi J Kidney Dis Transpl 2000;11:59-63.  Back to cited text no. 9
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10.Fahal IH, Mc Clelland P, Hay CR, Bell GM. Arterial thrombosis in the nephrotic syndrome. Postgrad Med J 1994;70:905-9.  Back to cited text no. 10
11.Kutcher R, Cohen JR, Gordon DH. Glomerulonephritis and nephrotic syndrome complicated by renal vein thrombosis and Pulmonary Emboli: Report of two cases. AJR Am J Roentgenol 1977;128:447-9.  Back to cited text no. 11
12.Decoster T, Schwagten V, Hendricks J, Beaucourt L. Renal colic as the first symptom of acute renal vein thrombosis resulting in the diagnosis of nephrotic syndrome. Eur J Emerg Med 2009;16:170-1.  Back to cited text no. 12
13.Sarasin FP, Schifferli JA. Prophylactic oral anticoagulation in nephrotic patients with idiopathic membranous nephropathy. Kidney Int 1994;45:578-85.  Back to cited text no. 13
14.Kayali F, Najjar R, Aswad F, Matta F, Stein PD. Venous thromboembolism in patients hospitalized with nephrotic syndrome. Am J Med 2008;121:226-30.  Back to cited text no. 14
15.Markowitz GS, Brignol F, Burns ER, Koenigsberg M, Folkert VW. Renal vein thrombosis treated with thrombolytic therapy: Case report and brief review. Am J Kidney Dis 1995;25:801-6.  Back to cited text no. 15
16.Al-Said J, Kamel O. Changes in renal cortical and medullary perfusion in a patient with renal vein thrombosis case report. Saudi J Kidney Dis Transpl 2009;20:123-7.  Back to cited text no. 16
17.Wysokinski WE, Gosk-Bierska I, Greene EL, Grill D, Wiste H, McBane RD 2nd. Clinical characteristics and long term follow-up of patients with renal vein thrombosis. Am J Kidney Dis 2008;51:224-32.  Back to cited text no. 17
18.Laville M, Aguilera D, Maillet PJ, Labeeuw M, Madonna O, Zech P. The prognosis of renal vein thrombosis: A re-evaluation of 27 cases. Nephrol Dial Transplant 1988;3:247-56.  Back to cited text no. 18

Correspondence Address:
Jafar Al-Said
Department of Nephrology and Internal Medicine, Bahrain Specialist Hospital, P. O. Box 10588, Manama
Kingdom of Bahrain
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.111068

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