+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Renal thrombotic microangiopathy and antiphospholipid syndrome nephropathy in a patient with lupus nephritis



Renal thrombotic microangiopathy and antiphospholipid syndrome nephropathy in a patient with lupus nephritis



Nihon Jinzo Gakkai Shi 58(1): 45-54



The patient was a 48-year-old Japanese woman diagnosed as having systemic lupus erythematosus at the age of 21 years when she presented with fever and an erythematous skin rash on her face and extremities. Prednisolone was initiated at that time. Thirteen days before admission to our hospital, she was referred to us by her family physician. Upon admission, blood tests showed pancytopenia, hypocomplementemia, and renal dysfunction, as well as the presence of lupus anticoagulant. Urinalysis showed abundant proteinuria and heavy microscopic hematuria. After performing a renal biopsy, we initiated immunosuppressive therapy and an anticoagulant. On the 22nd hospital day, microangiopathic hemolytic anemia appeared with the progression of thrombocytopenia and renal failure, and the patient subsequently underwent ten sessions of plasma exchange. After the commencement of the plasma exchange, her general condition improved. Her renal dysfunction, however, continued to progress, and hemodialysis was started on the 36th hospital day. The light microscopy showed severe endo- and extra-capillary proliferative glomerulonephritis with abundant crescents, and massive thrombi in the capillary lumen of the glomeruli. The arterioles contained occlusive hyaline materials. An immunofluorescence study showed granular staining of immunoglobulins and complements along the glomerular capillary wall. An electron microscopy examination revealed the presence of electron-dense deposits in the subepithelial and intramembranous areas of the glomeruli, but subendothelial deposits were absent. For cases with lupus nephritis (LN), immunosuppressive therapy based on corticosteroid remains the mainstay of treatment. However, immunosuppression alone may be insufficient when antiphospholipid antibody syndrome and thrombotic microangiopathy (TMA) are also present, and other treatment modalities including antiplatelet therapy, anticoagulation, and plasma exchange are likely to be necessary, as illustrated by the present case. Although the mechanism responsible for LN remains uncertain, we report a case of LN suggesting that TMA is associated with renal dysfunction.

Please choose payment method:






(PDF emailed within 1 workday: $29.90)

Accession: 058738771

Download citation: RISBibTeXText

PMID: 26950981


Related references

Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. American Journal of Kidney Diseases 20(2): 150-158, 1992

Thrombotic microangiopathy and poor renal outcome in lupus patients with or without antiphospholipid syndrome. Clinical and Experimental Rheumatology 33(4): 503-508, 2015

Renal thrombotic microangiopathy in a patient with rheumatoid arthritis and antiphospholipid syndrome: successful treatment with cyclophosphamide pulse therapy and anticoagulant. Internal Medicine 33(8): 484-487, 1994

A 3-year follow-up of a patient with acute renal failure caused by thrombotic microangiopathy related to antiphospholipid syndrome: case report. Lupus 26(7): 777-782, 2017

Antiphospholipid syndrome nephropathy (APSN) in patients with lupus nephritis: a retrospective clinical and renal pathology study. Rheumatology International 34(4): 535-541, 2014

The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Research and Therapy 15(1): R12, 2013

Antiphospholipid syndrome and renal thrombotic microangiopathy. JN Journal of Nephrology 8(3): 123-125, 1995

Plasmapheresis Is Associated With Better Renal Outcomes in Lupus Nephritis Patients With Thrombotic Microangiopathy: A Case Series Study. Medicine 95(18): E3595, 2016

Reversible renal failure due to the antiphospholipid antibody syndrome, pre-eclampsia and renal thrombotic microangiopathy. Clinical Nephrology 44(4): 271-273, 1995

Renal Thrombotic Microangiopathy in Proliferative Lupus Nephritis: Risk Factors and Clinical Outcomes: A Case-Control Study. Journal of Clinical Rheumatology 22(5): 235-240, 2016

Primary antiphospholipid syndrome paps associated with renal thrombotic microangiopathy tma report of 4 cases. Journal of the American Society of Nephrology 2(3): 267, 1991

De novo thrombotic microangiopathy in renal transplant recipients: a comparison of hemolytic uremic syndrome with localized renal thrombotic microangiopathy. American Journal of Kidney Diseases 41(2): 471-479, 2003

Catastrophic antiphospholipid syndrome presenting with renal thrombotic microangiopathy and diffuse proliferative glomerulonephritis. Clinical and Experimental Rheumatology 24(1): 110, 2006

Secondary thrombotic microangiopathy in systemic lupus erythematosus and antiphospholipid syndrome, the role of complement and use of eculizumab: Case series and review of literature. Seminars in Arthritis and Rheumatism 49(1): 74-83, 2019

Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature. World Journal of Gastroenterology 12(44): 7206-7209, 2006