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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 3  |  Page : 549-552
Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, A. P., India

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Date of Web Publication24-Apr-2013


In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS) is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

How to cite this article:
Vellanki VS, Parvathina S, Gondi S, Yadla M, Chenu KK, Vishnubhotla S. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus. Saudi J Kidney Dis Transpl 2013;24:549-52

How to cite this URL:
Vellanki VS, Parvathina S, Gondi S, Yadla M, Chenu KK, Vishnubhotla S. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2020 May 29];24:549-52. Available from: http://www.sjkdt.org/text.asp?2013/24/3/549/111064

   Introduction Top

Pregnancy-related acute renal failure (PRAF) is common in developing countries, with signi­ficant maternal and fetal mortality. [1],[2] Anti-phospholipid syndrome (APS) is characterized by the presence of venous and arterial throm­bosis, recurrent fetal losses and thrombocytopenia in the presence of elevated antiphospholipid antibodies (APA). In the presence of sys­temic lupus erythematosus (SLE) or related autoimmune disorders, it is termed as secon­dary antiphospholipid syndrome (SAPS). Preg­nancy-related renal failure due to SAPS is rarely reported in the literature. [3] We present a case of bilateral renal cortical necrosis due to SAPS in late pregnancy and the need to be vi­gilant toward this uncommon cause of PRARF under appropriate clinical settings is emphasized.

   Case Report Top

A 24-year-old primigravida, a house wife with no previous comorbid ailments such as SLE or vascular thrombosis, who was on regular antenatal follow-up elsewhere presented to our center with toxemia of pregnancy at 34 weeks gestation with an intrauterine dead fetus. She was referred to us on the second post-partum day with a complaint of anuria and generalized convulsions after an induced delivery for intra-uterine dead fetus. On examination, she was hemodynamically stable with well-contracted uterus and no retained products of conception. She continued to be anuric and was found to have severe renal failure with a fluid overload state requiring hemodialysis support.

Laboratory parameters revealed hemoglobin 8.5 gm/dL, leukocyte count 15,700/mm 3 , polymorphs 82%, lymphocytes 12%, eosinophils 3%, monocytes 3%, platelet count 48,000/mm 3 , sedimentation rate 78 mm, prothrombin time 14 s with a control of 12 s and activated partial thromboplastin time (APTT) 36.5 s with a con­trol of 31.8 s. Serological tests were positive for SLE and SAPA. Antinuclear antibodies and anti-double-stranded DNA antibodies were positive by ELISA, anticardiolipin antibodies-IgM were positive by ELISA, complement activity C3 120 mg/dL (90-180 mg/dL) C4: 20 mg/dL (10-40 mg/dL) and VDRL: negative; CPK: 180 IU/L (normal <300 IU/L) and LDH 610 IU/L (normal <800 IU/L); peripheral smear showed no schistozytes or abnormal cells. Urine microscopic examination: albumin ++, sugar - nil, RBC 2-3, WBC 12-14, no casts, and epithelial cells occasional; blood urea 180 mg/dL, serum creatinine 4.2 mg/dL. Cultures of the blood, urine and vaginal swabs were negative.

Ultrasound of the kidneys revealed normal-sized kidneys and contrast CT scan was suggestive of bilateral renal cortical necrosis [Figure 1]. Both the renal arteries were patent, ECG showed normal sinus rhythm and echo-cardiography was normal. A subsequent renal biopsy was deferred in view of poor general condition and thrombocytopenia.
Figure 1. Contrast-enhanced CT scan of the abdomen. The white arrows show bilateral "tram track" appearance of the kidneys, suggestive of bilateral renal cortical necrosis.

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The patient received 20 sessions of hemodialysis support and her urine output partially improved. She continued to be dialysis depen­dent with severe renal failure, and was even­tually lost to follow-up.

   Discussion Top

Acute renal failure in pregnancy in the modern era is considered as a rare compli­cation in developed countries, with an estimated incidence of less than 1 in 20,000 preg­nancies. In the developing countries, however, it is on the decline, with an estimated inci­dence of 4.24-9.06%. [1],[2] Obstetric-related acute renal failure, although on the decline, still constitutes about 25% of the referrals for dialysis in the developing world with a high risk of maternal mortality of 9-55%. [1],[2]

Two distinctive frequency peaks have been reported in obstetric acute renal failure. The first peak occurs during the 7-8 th weeks of ges­tation, with sepsis and post-abortion- related complications playing a major role, while the second peak occurs during the 32-36 th weeks, wherein toxemia of pregnancy, anti-partum/ post-partum hemorrhage, microangiopathy and acute fatty liver of pregnancy are largely res­ponsible, all of which could result in subse­quent renal cortical necrosis. [4],[5]

Renal cortical necrosis is defined as a con­dition of destruction of the renal cortex except for a thin tissue rim under the capsule and usually a thicker layer under the cortico-medullary junction owing to disturbed blood flow into the interlobular and afferent arterioles. In practice, the diagnosis is usually made without the aid of biopsy based on protracted or often irreversible renal shutdown along with characteristic imaging findings on CT scan/angiography. It is an uncommon en­tity, accounting for only 2% of all cases of acute renal failure. Pregnancy bears a high risk of bilateral renal cortical necrosis owing to the well-described entity of Schwartzman phenomenon. [6] Its incidence has been reported to range from 14.28% to 23.8% in India as com­pared with 1.5% in the developed countries. [2],[7]

SLE occurs nine times more frequently in women, and pregnancy is not an infrequent occurrence in SLE. The incidence of pre-eclampsia is as high as 66%. The disease flares and exacerbation of glomerulonephritis may occur especially in the 3 rd trimester and in the post-partum period. [6]

The APA syndrome (APS) describes a clin­ical entity with recurrent thrombosis, fetal loss, thrombocytopenia in the presence of lupus anticoagulant and/or antibodies to cardiolipin. A patient with APS must manifest at least one of two criteria (vascular thrombosis or preg­nancy morbidity) and at least one of three laboratory criteria lupus anticoagulants (LAs), anticardiolipin antibodies (aCLs) and/or anti-β2-glycoprotein I antibodies (anti-β2GPI). Labo­ratory criteria must be met on two or more occasions, at least more than 12 weeks but less than 5 years apart. APS has traditionally been classified as primary if found in isolation and as secondary (SAPS) if associated with connec­tive tissue disorders. SLE has been the most common disease with which SAPS occurs. The frequency of antiphospholipid antibodies (aPLs) among patients with SLE is about 30-40%. The lupus APS may be associated with pro­minent renal vascular disease in the absence of proliferative glomerular lesions. [6],[8]

It has been suggested that aPLs interfere with or modulate the function of phospholipid-binding proteins involved in the regulation of coagulation along with activation of endothe­lial cells with increased expression of adhesion molecules through possible modulation of Toll­like receptor 4 (TLR4) and leading to a pro-thrombotic microenvironment. [8] Renal manifestations of APS have been reported although infrequently possibly due to synthesis of large amounts of nitric oxide (NO) by mesangial cells and synergy between prostacyclin (PGI2) and NO in their platelet inhibitory action, which may contribute to a lower incidence of thrombosis in the glomerular capillaries. [9] Va­rious manifestations of APA include renal artery thrombosis, thrombotic microangiopa­thy and renal vein thrombosis, progressive re­nal failure, systemic hypertension, renal cort­ical necrosis and variable proteinuria. [6],[8]

Our patient presented with typical signs of renal cortical necrosis as anuria and severe renal failure requiring dialysis support follo­wing evacuation of the intrauterine dead fetus in the third trimester of pregnancy. Bilateral cortical necrosis was diagnosed with contrast CT scan and she fulfilled the American College of Rheumatology (ACR) criteria for SLE. [5],[6] The presence of positive anti-nuclear anti­bodies and anti-double-stranded DNA strongly suggested the possibility of SLE, in addition to the anti-cardiolipin antibodies, which sugges­ted the possibility of secondary antiphospholipid syndrome.

Obstetric complications related to septicemia and hypotensive shock are responsible for a vast majority of acute bilateral renal cortical necrosis in developing countries. [2],[7] Thrombotic occlusion most frequently has been des­cribed in context with major veins in SAPS, while arterial involvement is uncommon. [9],[10],[11] The ominous outcome in this patient was attributed probably to a combination of abnor­malities such as lupus, SAPS, toxemia of preg­nancy and intrauterine death.

   References Top

1.Kumar KS. Krishna CR, Siva Kumar V. Preg­nancy related acute renal failure. J Obstet Gynecol India 2006;56:308-10.  Back to cited text no. 1
2.Goplani KR, Shah PR, Gera DN, et al. Preg­nancy -related acute renal failure: A single­center experience. Indian J Nephrol 2008;18: 17-21.  Back to cited text no. 2
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3.Jha R, Sinha S, Kumar J, Bansal D, Gupta PC. Renal infarction in antiphospholipid syndrome. Indian J Nephrol 2005;15:17-21.  Back to cited text no. 3
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4.Chugh KS. Etiopathogenesis of acute renal failure in the tropics. Ann Natl Acad Med Sci (India) 1987;23:88-9.  Back to cited text no. 4
5.Maikranz P, Katz Al. Acute renal failure in pregnancy. Obstet Gynecol Clin North Am 1991;18:333-43.  Back to cited text no. 5
6.West SG, Gregory A, Achenbach, Edelstein CL. Renal involvement in systemic lupus erythematosus. In: Schrier RW, ed. Diseases of the Kidney and urinary tract. 8 th ed. Phila­delphia: Lippincott Williams & Wilkins; 2007. p. 1673-99.  Back to cited text no. 6
7.Prakash J, Tripathi K, Pandey LK, Gadela SR, Usha. Renal Cortical Necrosis in pregnancy-related acute renal failure. J Indian Med Assoc 1996;94:227-9.  Back to cited text no. 7
8.Fischer MJ, Rauch J, Levine JS. The Antiphospholipid syndrome. Semin Nephrol 2007;27:35-46.  Back to cited text no. 8
9.Kitta T, Takeyama Y, Seki T, Togashi M. Harada H. Asynchronous (heterochronic) bila­teral renal infarction associated with primary antiphospholipid syndrome. Int J Urol 2001;8: 631-3.  Back to cited text no. 9
10.Asherson RA, Hughes GR, Derksen RH. Renal infarction associated with antiphospholipid antibodies in systemic lupus erythematosus & lupus like disease. J Urol 1988;140:1028.   Back to cited text no. 10
11.Moss KE, Isenberg DA. Comparison of renal disease severity and outcome in patients with primary antiphospholipid syndrome, antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE) and SLE alone. Rheumatology (Oxford) 2001;40:863-7.  Back to cited text no. 11

Correspondence Address:
Sivakumar Vishnubhotla
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, A. P. 517507
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DOI: 10.4103/1319-2442.111064

PMID: 23640629

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This article has been cited by
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