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Anti-neutrophil cytoplasmic antibody vasculitis presenting with bilateral renal vein thrombosis.

Wlodek C, Robson MG - Clin Kidney J (2012)

Bottom Line: We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies.Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual.Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Transplantation, Guy's and St. Thomas' NHS Foundation Trust, London, UK.

ABSTRACT
We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies. Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual. Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

No MeSH data available.


Related in: MedlinePlus

Renal histology showing a representative glomerulus with a fibrocellular crescent.
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fig2: Renal histology showing a representative glomerulus with a fibrocellular crescent.

Mentions: His initial thrombophilia screen showed a lupus anticoagulant (LA) by dilute-activated partial thromboplastin time analysis with negative anti-cardiolipin antibodies (aCL). His activated partial thromboplastin time was normal at 1.1. The acute kidney injury was felt to be due to the renal vein thrombosis for which he was anticoagulated, initially with heparin and then with warfarin, and discharged. A month after his initial presentation, he was re-admitted with vomiting, possibly secondary to warfarin intolerance, and a subtherapeutic international normalised ratio requiring intravenous unfractionated heparin. His renal function was unchanged with a serum creatinine of 180 μmol/L, CRP was now <5 mg/L, urine PCR was 363 mg/mmol and serum albumin 32 g/L. A decision was made to perform a renal biopsy. There were 20 glomeruli of which 3 were obsolete. The remainder showed 1 active and 15 fibrocellular crescents. Tubular atrophy was in 10% of the sample and immunoperoxidase staining was negative for all immunoglobulin and complement components. There were no features suggestive of anti-phospholipid syndrome (APLS). Representative histology is shown in Figure 2.


Anti-neutrophil cytoplasmic antibody vasculitis presenting with bilateral renal vein thrombosis.

Wlodek C, Robson MG - Clin Kidney J (2012)

Renal histology showing a representative glomerulus with a fibrocellular crescent.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400499&req=5

fig2: Renal histology showing a representative glomerulus with a fibrocellular crescent.
Mentions: His initial thrombophilia screen showed a lupus anticoagulant (LA) by dilute-activated partial thromboplastin time analysis with negative anti-cardiolipin antibodies (aCL). His activated partial thromboplastin time was normal at 1.1. The acute kidney injury was felt to be due to the renal vein thrombosis for which he was anticoagulated, initially with heparin and then with warfarin, and discharged. A month after his initial presentation, he was re-admitted with vomiting, possibly secondary to warfarin intolerance, and a subtherapeutic international normalised ratio requiring intravenous unfractionated heparin. His renal function was unchanged with a serum creatinine of 180 μmol/L, CRP was now <5 mg/L, urine PCR was 363 mg/mmol and serum albumin 32 g/L. A decision was made to perform a renal biopsy. There were 20 glomeruli of which 3 were obsolete. The remainder showed 1 active and 15 fibrocellular crescents. Tubular atrophy was in 10% of the sample and immunoperoxidase staining was negative for all immunoglobulin and complement components. There were no features suggestive of anti-phospholipid syndrome (APLS). Representative histology is shown in Figure 2.

Bottom Line: We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies.Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual.Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Transplantation, Guy's and St. Thomas' NHS Foundation Trust, London, UK.

ABSTRACT
We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies. Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual. Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

No MeSH data available.


Related in: MedlinePlus