Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS.

OBJECTIVE: Renal involvement in antiphospholipid syndrome (APS) has been poorly recognized. A renal small-vessel vasculopathy, defined as APS nephropathy, has recently been observed in small series of patients with primary APS (PAPS) and systemic lupus erythematosus (SLE)-APS. We examined the renal histologic, clinical, and laboratory characteristics of different groups of patients with APS including catastrophic APS (CAPS). METHODS: Our study included all CAPS (n=6), PAPS (n=8), and SLE-APS (n=23) patients with biopsy-proven renal involvement who were referred to our departments. The kidney biopsy specimens were retrospectively examined by the same renal pathologist. APS nephropathy was diagnosed as previously described. Demographic, clinical, and laboratory data were recorded. RESULTS: All patients with CAPS had acute and chronic renal vascular lesions compatible with diagnosis of APS nephropathy. Thrombotic microangiopathy (TMA), the acute lesion, was observed in all CAPS patients. Fibrous intimal hyperplasia of interlobular arteries (FIH) and focal cortical atrophy (FCA) were the most common chronic vascular lesions, occurring in 4 of 6 (66.7%) and 3 of 6 (50%) patients with CAPS, respectively. TMA was detected in 3 of 8 (37.5%) patients with PAPS and in 8 of 23 (35%) patients with SLE-APS, while FIH and FCA were found with similar frequencies in all 3 groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups. CONCLUSION: Acute and chronic APS nephropathy lesions were detected in all 3 APS groups. Acute lesions were more prominent in CAPS, while chronic lesions were found with similar frequencies in all groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups.

Reduction in glucocorticoid receptors in renal biopsies of patients with lupus nephritis.

OBJECTIVES: The first-line treatment for lupus nephritis is the administration of glucocorticoids (GC) that mediate their effects via the glucocorticoid receptor (GR). The aim of this study was to investigate the expression of GR protein in the cortical area of renal parenchyma of normal and diseased renal biopsies from treated and untreated patients. DESIGN AND METHODS: The immunohistochemical EnVision/HRP technique was performed on renal tissue to detect GR protein. Statistical analysis was performed by SAS (2001). RESULTS: The antigen was mainly detected in glomerular podocytes and in tubules. The number of GR-positive podocytes of the controls was significantly higher than in the untreated patients, which was accordingly higher than in patients who were under medication. CONCLUSIONS: The lower number of GR-positive cells in the diseased kidney compared to controls is possibly linked to tissue-specific GC resistance, whereas the decreased GR expression in podocytes of treated compared to untreated patients may be due to a down-regulation effect after GCs' administration.

Catastrophic antiphospholipid syndrome in a 14-year-old child.

Antiphospholipid syndrome (APS) is an autoimmune disease. Less than 1% of patients with APS present with life-threatening catastrophic APS (CAPS). We report here a case of CAPS in a young girl with cardiac, gastrointestinal and renal involvement. Although the management was complicated, the outcome was better than expected. We suggest that CAPS be included in the differential diagnosis of acute renal failure in children with multi-organ involvement and prolonged phospholipid-dependent coagulation time and promptly treated with immunomodulating agents and anticoagulants.

Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome.

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.

Tumor to tumor metastasis: report of two cases and review of the literature.

Tumor-to-tumor metastases are uncommon. The most frequent donor tumor is lung cancer, while renal cell carcinoma (RCC) is by far the most common recipient. In this report, a carcinoma of the uterine cervix metastasizing to an RCC and a urothelial carcinoma of the urinary bladder metastasizing to a solitary fibrous tumor of the pleura are described. No similar cases have been found in the accessible literature. These cases are discussed and the findings are correlated with the data of the literature.

Clinicopathologic predictors of death and ESRD in patients with pauci-immune necrotizing glomerulonephritis.

BACKGROUND: Pauci-immune necrotizing glomerulonephritis (PING) occurs in various settings and has a very variable prognosis. We investigated whether clinicopathologic findings at the time of renal biopsy may predict major disease outcomes. METHODS: We evaluated 72 consecutive patients with biopsy-documented PING. Kaplan-Meier curves and Cox models assessed event rates and risk factors for death, end-stage renal disease (ESRD), and death or new ESRD (after the renal biopsy). RESULTS: During a follow-up of 305 person-years, 11 patients died, 13 patients developed ESRD, and 16 patients died or developed new ESRD. Among patients first seen within 3 months of renal biopsy (incident cases), the 5-year mortality rate was 20%, whereas the death or new ESRD rate was 34%. In univariate analyses, older age, lower creatinine clearance, erythrocyte sedimentation rate, and percentages of abnormal glomeruli, glomeruli with fibrous crescents, and glomeruli with global sclerosis were significant predictors of mortality, whereas antineutrophil cytoplasmic autoantibodies with cytoplasmic staining conferred borderline protection. For ESRD, significant predictors included a greater creatinine level, lower hematocrit, interstitial fibrosis, tubular necrosis, greater C-reactive protein level, and percentages of abnormal glomeruli, glomeruli with extracapillary proliferation, cellular crescents, and global glomerulosclerosis. For death or new ESRD, predictors were fairly similar. Adjusting for baseline creatinine level, the risk for ESRD increased 1.78-fold (95% confidence interval [CI], 1.23 to 2.58) per each 10% increase in global sclerosis and 1.47-fold (95% CI, 1.05 to 2.07) per each 10% increase in glomeruli with cellular crescents. CONCLUSION: Global glomerulosclerosis and crescents in a renal biopsy are strong predictors of the long-term outcome of PING.

Diagnostic dilemmas in atypical postinfectious glomerulonephritis.

This study discusses the differential diagnosis of atypical postinfectious glomerulonephritis (PIGN) which may mimic a great variety of glomerular diseases. These include mild mesangial and/or endocapillary glomerulonephritis (GN) focal segmental glomerulosclerosis (FSGS) with diffuse IgM mesangial deposits, crescentic GN with C3 hump-like deposits, focal mesangiocapillary GN superimposed on endocapillary pattern, membranous GN with diffuse exudative changes, crescentic GN with microabscesses, and postinfectious glomerulonephritis with anti-GBM linear deposits.