APS - Nephrology Related
Menon RN, Bichile LS. Study of renal histopathological correlation with anticardiolipin antibody status in patients with systemic lupus erythematosus. Indian J Nephrol 2007;17:53-60. Conclusions: Hypocomplementemia is the only probable marker of renal morbidity in the laboratory follow-up of the SLE patients. aCL positivity does contribute to greater incidence of interstitial fibrosis, tubular atrophy and capillary wall thickening in biopsy specimens. APSN is an independent entity that must be specifically sought for on histological assessment.
Acta Paediatr. 1998 Aug;87(8):903-7.
Conclusion: High index of suspicion is required in the appropriate clinical setting with unexplained asymmetrical functioning of kidney.
Q J Med 2000; 93: 127-129 © 2000 Association of Physicians.
Q J Med 2000; 93: 127-129 © 2000 Association of Physicians
Am J Kidney Dis. 1992 Aug;20(2):150-8.
J Am Soc Nephrol 10:507-518, 1999 © 1999 American Society of Nephrology. This study allows us to approach the nephropathy of PAPS as an entity in its own right, characterized by small-vessel vaso-occlusive intrarenal pathology that likely evolves by repeated flares leading to a morphologic picture suggestive of its diagnosis even in the absence of history. This picture combines the lesions of TMA, FIH of the arteries and arterioles, and FCA. The clinical hallmark of this nephropathy is hypertension, only variably associated with renal insufficiency, protein-uria, or hematuria. Conversely, the recognition of such lesions on a biopsy performed for diagnostic purposes should lead to work-up for possible PAPS. However, we need to understand better the morbidity associated with this nephropathy to be able to define the indications for and modalities of treatment.
Rheumatology (Oxford). 2005 Mar;44(3):372-7. Epub 2004 Nov 30.
Transplant Proc. 1999 Feb-Mar;31(1-2):230-3.
Arthritis Rheum. 2004 Aug;50(8):2569-79. 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.
Am J Kidney Dis. 1992 Aug;20(2):150-8. Current studies indicate that a thrombotic microangiopathy (TMA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TMA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases.
Japanese Circulation Journal Vol. 64 (2000) , No. 7 541-543
Am J Nephrol. 2001 Jan-Feb;21(1):55-7. Primary antiphospholipid antibody (APA) syndrome, a common prothrombotic disorder, has been known in dialysis patients and renal transplant recipients. We report a case of primary APA syndrome presenting as a posttransplant complication in a renal transplant donor. A renal donor presented with acute, painless anuria due to renal artery thrombosis 6 years following renal transplant surgery, subsequent thrombosis of jugular catheter and arteriovenous fistula occurred, despite anticoagulation treatment, due to primary APA syndrome. This incident represents the most catastrophic complication reported in a renal donor due to primary APA syndrome. The validity of a prothrombotic assay in an organ donor workup to detect predilection to hypercoagulable disorders and to prevent such complications is open to question. The actual significance of APA in the blood is unclear; hence, the presence of APA in a potential renal donor would pose an ethical and practical dilemma.
Ren Fail. 2003 Nov;25(6):1043-9.
Ann Rheum Dis. 2003 Oct;62(10):999-1002. CONCLUSION: A significantly increased prevalence of RAS (26%) was found in patients with APS and hypertension, compared with relatively young (
Am J Kidney Dis. 2003 Jun;41(6):1205-11. The cases reported here represent a new aspect of the expanding spectrum of renal diseases encountered in association with APS.
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