Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Cecere, Fred; Yoshinoya, S; Pope, Richard M.

doi:10.1002/art.1780240316

Cited by 26 publications

(8 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…TMA is characterized by fibrin thrombi in the glomeruli and/or small arterioles in the absence of vascular immune deposits or inflammatory cells. The appearance of TMA lesions is similar to the changes observed in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome, entities occasionally occurring in the context of SLE (25, 26). When making a diagnosis of APS nephropathy, the clinical context must be considered rather than relying only on biopsy criteria.…”

Section: Discussionsupporting

confidence: 65%

Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long‐term outcome

Tektonidou¹,

Sotsiou²,

Nakopoulou³

et al. 2004

Arthritis & Rheumatism

221

163

View full text Add to dashboard Cite

Objective. To evaluate the prevalence, clinical associations, and outcome of antiphospholipid syndrome (APS) nephropathy in patients with systemic lupus erythematosus (SLE) and antiphospholipid antibodies (aPL) and in SLE patients without aPL.Methods. Kidney biopsy specimens obtained from 81 patients with aPL (18 of whom had APS) and 70 patients without aPL were retrospectively examined for the presence of APS nephropathy. Clinical and serologic data obtained at the time of kidney biopsy and during a mean followup of 7 years were recorded. In cases for which serial kidney biopsy specimens were available, the evolution of APS nephropathy was examined.Results. APS nephropathy existed in 39.5% of patients with aPL, compared with only 4.3% of patients without aPL. APS nephropathy was associated with both lupus anticoagulant and anticardiolipin antibodies. Among aPL-positive SLE patients, APS nephropathy was found in two-thirds of those with APS and in one-third of those without APS. A strong association between APS nephropathy and the presence of arterial thrombosis and livedo reticularis was noted. Patients with APS nephropathy had a higher frequency of hypertension and elevated serum creatinine levels at the time of kidney biopsy but did not have a higher frequency of renal insufficiency, end-stage renal disease, or death at the end of followup. Serial kidney biopsy specimens were available from 11 patients and showed progression of APS nephropathy lesions. During followup, manifestations of APS (especially arterial thromboses) developed more frequently in the SLE/non-APS patients with APS nephropathy than in those without APS nephropathy.Conclusion. Among patients with SLE, APS nephropathy occurs almost exclusively in those with aPL, suggesting an important role of aPL in the pathogenesis of APS nephropathy. Patients with APS nephropathy develop hypertension, raised serum creatinine levels, and progression of histologic lesions, all of which are associated with a poor renal outcome. Manifestations of APS also tend to develop in these patients. APS nephropathy should be included in the APS classification criteria, and the use of appropriate anticoagulant therapy should be tested.

show abstract

Section: Discussionsupporting

confidence: 65%

Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long‐term outcome

Tektonidou¹,

Sotsiou²,

Nakopoulou³

et al. 2004

Arthritis & Rheumatism

221

163

View full text Add to dashboard Cite

show abstract

“…The appearance of TMA is similar to the renal lesions observed in other microangiopathies such as thrombotic thrombocytopenic purpura, haemolytic-uremic syndrome, malignant hypertension, postpartum renal failure, preeclampsia, diabetic nephropathy, cyclosporin therapy, or chemotherapy [89,90]. Thus, when making a diagnosis of APS nephropathy, the clinical context must be considered and patients with clinical and laboratory characteristics compatible with the above entities, often occurring in SLE patients, should be excluded.…”

Section: Aps Nephropathy In Slementioning

confidence: 96%

Renal Involvement in the Antiphospholipid Syndrome (APS)—APS Nephropathy

Tektonidou

2008

Clinic Rev Allerg Immunol

View full text Add to dashboard Cite

Although the kidney represents a major target organ in antiphospholipid syndrome (APS), renal involvement in APS was poorly recognized until recently. The most well-recognized renal manifestations of APS are the renal artery thrombosis/stenosis, renal infarction, hypertension, renal vein thrombosis, end-stage renal disease, increased allograft vascular thrombosis, some types of glomerular disease, and a small-vessel vaso-occlusive nephropathy, recently defined as APS nephropathy. APS nephropathy was first described in primary APS patients, characterized by acute thrombotic lesions in glomeruli and/or arterioles (thrombotic microangiopathy) and chronic vascular lesions such as fibrous intimal hyperplasia of arterioles and interlobular arteries, organized thrombi with or without recanalization, and fibrous arterial and arteriolar occlusions or focal cortical atrophy. APS nephropathy was also detected in further studies including patients with systemic lupus erythematosus (SLE)-related APS and SLE/non-APS patients with positive antiphospholipid antibodies, independently of lupus nephritis. The same histologic lesions, especially thrombotic mictroangiopathy, were also observed in patients with catastrophic APS. The most frequent clinical and laboratory characteristics of APS nephropathy in all the above groups of patients are hypertension (often severe), proteinuria (ranging from mild to nephrotic range), hematuria, and acute or chronic renal insufficiency.

show abstract

“…TTP has been described in association with SLE (1,9,11). It has also been reported to occur during pregnancy, with a few patients having been successfully treated by plasma exchange or plasma infusion ( I ) .…”

mentioning

confidence: 99%

Thrombotic thrombocytopenic purpura developing during pregnancy in A C2‐deficient patient with a history of systemic lupus erythematosus

Dixit

Krieg

Atkinson

1985

Arthritis & Rheumatism

View full text Add to dashboard Cite

Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Cited by 26 publications

References 21 publications

Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long‐term outcome

Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long‐term outcome

Renal Involvement in the Antiphospholipid Syndrome (APS)—APS Nephropathy

Thrombotic thrombocytopenic purpura developing during pregnancy in A C2‐deficient patient with a history of systemic lupus erythematosus

Contact Info

Product

Resources

About