A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy

An 18-year-old woman complained of fever and edema and was admitted to Showa University Hospital for treatment of thrombocytopenia and deteriorating renal function. Laboratory studies demonstrated the presence of lupus anticoagulant (LA), prolongation of prothrombin time, hemolytic anemia, a negative Coombs' test, the absence of antinuclear antibodies, and a normal fibrinogen level. Renal biopsy revealed mesangial hypercellularity, severe endocapillary cell damage, and double contour of the basement membrane walls. Immunofluorescence studies demonstrated focal, peripheral, and finely granular deposits for IgG, IgM, and IgA but were negative for fibrinogen. Electron microscopy showed glomerular capillary loops with subendothelial widening and subendothelial deposits, mesangiolysis, mesangial interposition, and marked luminal narrowing. Biopsy findings were consistent with thrombotic microangiopathy. The patient was treated with hemodialysis, methylprednisolone pulse therapy, and dipyridamole. After treatment, LA disappeared, the prothrombin time became normal, and renal function improved. The renal lesions in this patient were caused by primary antiphospholipid antibody syndrome. This case strongly suggests an important causal relationship between LA and renal lesions in thrombotic microangiopathy. We present this case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome. Copyright Copyright 1999 S. Karger AG, Basel     

Management of end-stage renal disease patients with antiphospholipid antibody syndrome

AIMS: End-stage renal disease (ESRD) patients with antiphospholipid antibody syndrome (APAS) remain at high risk for the development of posttransplant renal thrombosis without the benefit of anticoagulation therapy. This study describes the clinical management of these high-risk patients on anticoagulation therapy. METHODS: In this study period, 802 patients awaiting renal transplantation were screened for APAS. Twenty-seven of these patients (3%) had APAS. Of these 27, nine patients received cadaveric kidney transplants along with 409 patients who did not have APAS. Of the nine patients, seven were treated with coumadin and the remaining two were treated with heparin. RESULTS: Of the seven patients treated with coumadin, five did not have thrombotic complications posttransplant. However, three of these patients were taken off coumadin due to bleeding complications at 6 months to 1 year posttransplant. They all returned to dialysis shortly thereafter. The remaining two patients have maintained their allografts on coumadin therapy for 3 and 5 years posttransplants. The other two patients had posttransplant renal thrombosis within 24 hours of their transplant despite coumadin therapy. Of the two patients treated with heparin, one is doing well at 6 years posttransplant while the other had early allograft loss due to thrombosis. CONCLUSIONS: ESRD patients with APAS may benefit from anticoagulation therapy; however, early allograft loss and bleeding complication are two serious side effects of this therapy.     

Acute autoimmune cardiomyopathy in primary antiphospholipid antibody syndrome

We present a case of acute pulmonary oedema as the first presentation of autoimmune cardiomyopathy in primary antiphospholipid antibody syndrome in a patient who had no previous cardiac history. Five days of methylprednisolone at 500 mg/day followed by 100 mg/day for 10 days and then a weaning course of oral prednisone resulted in effective resolution of the acute diffuse cardiomyopathy. Her cardiac status became clinically and echocardiographically normal. We illustrate the effectiveness of immunosuppressive therapy as an adjunct to standard anti-failure measures in such presentations and we outline the association between antiphospholipid antibodies and cardiac dysfunction.     

Mitral insufficiency associated with primary antiphospholipid syndrome and chronic renal failure

We report a case of 52-year-old woman with primary antiphospholipid syndrome who developed mitral insufficiency and chronic renal failure. Continuous ambulatory peritoneal dialysis was started preoperatively due to thrombocytopenia that was aggravated by hemodialysis. Mitral annuloplasty was performed since the mitral valve was not severely damaged. Her postoperative hemodynamics were stable, and anticoagulant therapy was controlled easily. She recovered from severe thrombocytopenia while on continuous ambulatory peritoneal dialysis. Valvular heart disease is a well known feature of primary antiphospholipid syndrome, and there have been several reports about valve replacement in patients who had antiphospholipid syndrome with or without systemic lupus erythematosus. However, valve repair has been reported in only a few such patients. We believe that valve repair is better than valve replacement in patients with antiphospholipid syndrome because of its hypercoagulable tendency. In addition, it seems that continuous ambulatory peritoneal dialysis is a suitable method for the perioperative management of patients with antiphospholipid syndrome who suffer from chronic renal failure as well as thrombocytopenia, and require cardiac surgery under cardiopulmonary bypass.     

Catastrophic antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.     

Efficacy of anticoagulation therapy in end-stage renal disease patients with antiphospholipid antibody syndrome

BACKGROUND: End-stage renal disease (ESRD) patients with antiphospholipid antibody syndrome (APAS) remain at high risk for the development of renal thrombosis without the benefit of anticoagulation therapy. This study examines the efficacy of anticoagulation therapy in this high-risk patient population. METHOD: Of nine APAS renal-transplant patients, seven were treated with coumadin, whereas two were treated with heparin. RESULTS: Of the two patients treated with heparin, one had early allograft loss, whereas the other patient is doing fine at 5 years posttransplant. Of the seven 7 patients treated with coumadin, two patients are doing well at 2 and 3 years posttransplant, two had early allograft loss, the remaining three patients returned to dialysis after they were taken off of the coumadin at 6, 12, and 20 months posttransplant because of bleeding complications. CONCLUSIONS: Anticoagulation therapy is beneficial to some but not all APAS patients. In addition, bleeding complications are a serious side effect of this therapy.     

Asynchronous (heterochronic) bilateral renal infarction associated with primary antiphospholipid syndrome

We report the rare case of a 51-year-old man with asynchronous (heterochronic) bilateral renal infarction associated with primary antiphospholipid syndrome. He was treated for right renal infarction, but 2 months later, while under anticoagulant treatment, he had a recurrence on the other side of the renal infarction. The laboratory work-up confirmed antiphospholipid syndrome. Six months later the patient has not experienced any new thrombotic episodes and is receiving oral anticoagulants and antiplatelet therapy.     

Acute renal failure in a renal transplant donor due to primary antiphospholipid syndrome

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.     

Acute postoperative biventricular failure associated with antiphospholipid antibody syndrome

Antiphospholipid syndrome is probably the most common acquiredhypercoagulable state, but information on perioperative managementis sparse. Minor alterations in anticoagulant therapy, infection,or a surgical insult may trigger widespread thrombosis. Theperioperative course of a 31-yr-old woman with primary anticardiolipinantiphospholipid antibody syndrome requiring a mitral valvereplacement is described. Postoperatively, she developed acuteglobal biventricular failure requiring extracorporeal membraneoxygenation support and plasmapheresis. The management of anticoagulationand cardiac surgery in this condition is reviewed. Br J Anaesth 2004; 92: 748–54     

A case of antiphospholipid antibody syndrome with variable renal histological change despite of poor changes in urinalysis

We experienced a case of lupus nephritis with antiphospholipid antibody syndrome. A renal biopsy specimen from this patient showed various renal histological changes, but the results of urinalysis were almost normal. The patient was a 56-year-old woman diagnosed as having systemic lupus erythematosus with antiphospholipid antibody syndrome in 1983. In 1998, she had diarrhea and blood gas analysis showed metabolic acidosis. Therefore, she was admitted to our hospital and underwent renal function examination. The glomerular filtration rate was reduced(GFR: 40/ml/min), but urinalysis was almost normal. To examine her renal dysfunction, we performed open renal biopsy. Her renal tissues showed global glomerular sclerosis, mesangial cell proliferation and infiltration of cells in the tubulointerstitial area(WHO II). Furthermore, some arterioles showed organized thrombus formation and recanalization due to the antiphospholipid antibody syndrome. Renal biopsy of patients with lupus nephritis is useful not only for precise diagnosis, but also for the selection of appropriate treatment.     

The intrarenal vascular lesions associated with primary antiphospholipid syndrome

Even 10 yr after the identification of the antiphospholipid syndrome (APS), renal involvement in the course of APS is still relatively unrecognized, and is probably underestimated. The association of anticardiolipin antibodies and/or lupus anticoagulant with the development of a vaso-occlusive process involving numerous organs is now confirmed. In a multicenter study, 16 cases of "primary" APS (PAPS) were found and followed for 5 yr or more, all with renal biopsy. In all 16 cases of PAPS, there was a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries (12 patients), recanalizing thrombi in arteries and arterioles (six patients), and focal cortical atrophy (10 patients). In combination, these led to progressive destruction of the kidney, accelerated by acute glomerular and arteriolar microangiopathy in five patients. Focal cortical atrophy is a distinctive lesion, present in 10 biopsies, and likely represents the histologic and functional renal analogue to the multiple cerebral infarcts detected on imaging studies. The clinical hallmark of this vascular nephropathy in PAPS is systemic hypertension, only variably associated with renal insufficiency, proteinuria, or hematuria. The ensemble of histologic renal lesions defined in this study should aid in the separation of the lesions found in cases of secondary APS, especially systemic lupus erythematosus, into those lesions related to APS and those related to the underlying disease.     

Catastrophic antiphospholipid antibody syndrome following initiation of hemodialysis

Antiphospholipid syndrome (APS) is associated with arterial and venous thrombosis, pregnancy morbidity, and thrombocytopenia. Some APS patients develop rapid and disseminated microthrombosis and are known as having catastrophic APS or CAPS. We document here a case of CAPS in a patient who presented with various clinical symptoms and serious abnormalities of blood coagulation following initiation of hemodialysis after bilateral nephrectomy due to renal cancer. The patient developed multiple organ symptoms, including melena, visual disturbances, skin eruptions, lymph node swelling, urinary tract bleeding, backache, arteriovenous fistula occlusion, and chest pain. Based on the clinical course and serological and histological examinations, a diagnosis of CAPS was established. The patient recovered by following intensive anticoagulation and steroid therapy. Although CAPS is rare, once symptoms develop the condition deteriorates rapidly. Because of the associated high mortality, early diagnosis and prompt treatment are necessary.