Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis.

BACKGROUND: We investigated the clinical-pathological features and the prognosis of thrombotic thrombocytopenic purpura (TTP) in patients with lupus nephritis (LN). METHODS: A retrospective analysis was performed on the clinical-pathological data and prognosis in 8 patients with LN complicating with TTP. RESULTS: Thrombocytopenia and hemolytic anemia, neurologic symptoms, and renal dysfunction were the clinical manifestations in 8 patients. Six patients had fever. Eight patients presented with rapid progressive glomerulonephritis, and 1 patient with continuous gross hematuria. The histologic features of the 8 patients were thrombotic microangiopathy lesions. Immune-suppressive therapies were administrated in all patients, and blood purification therapy was applied in 7 patients. Three cases involved plasma exchange and/or immunoabsorption. Seven patients received a median follow-up of 12 months. One patient died, 3 cases received peritoneal dialysis, and 1 case failed to follow-up. During follow-up, 1 case was able to stop peritoneal dialysis, and 1 case changed to hemodialysis. The other 3 patients continued with stable renal function. CONCLUSION: The patients with LN with TTP have severe clinical-pathological changes. Active treatment including renal replacement therapy, plasma exchange, and immunoabsorption are promising.

Successful treatment of patients with lipoprotein glomerulopathy by protein A immunoadsorption: a pilot study.

BACKGROUND: No established therapy is available for patients with lipoprotein glomerulopathy (LPG). Protein A immunoadsorption has been proved to be effective in reducing proteinuria in patients with nephrotic syndrome. In this uncontrolled pilot study, we investigated the efficiency of immunoadsorption onto staphylococcal protein A as treatment for LPG. METHODS: Thirteen patients with renal biopsy-proven LPG were treated with staphylococcal protein A immunoadsorption. Immunoadsorption was administered for 10 cycles per session and 10 sessions as a course. A total of 30 l of plasma was regenerated in each course. RESULTS: Single immunoadsorption course led to a rapid decline in proteinuria from 4.01 +/- 3.09 g/24 h to 1.21 +/- 0.97 g/24 h (mean +/- SD) (n = 13, P = 0.001), along with a dramatic decline in apolipoprotein E (apo E) from 9.79 +/- 5.04 mg/dl to 6.20 +/- 2.22 mg/dl (P = 0.004). A repeated renal biopsy (n = 12) showed that intraglomerular lipoprotein thrombi almost disappeared. Six patients were enrolled in the investigation of long-term outcome, and proteinuria returned to baseline levels within 12 months. Four recurrent patients received repeat immunoadsorption treatment; proteinuria decreased from 5.02 +/- 1.85 g/24 h to 1.64 +/- 0.55 g/24 h at the end of the treatment, serum apo E decreased from 14.65 +/- 11.17 mg/dl to 7.90 +/- 1.72 mg/dl. No patients suffered from severe complications. CONCLUSION: Our observations suggest that immunoadsorption onto protein A might be an effective treatment for resolving intraglomerular thrombi and improving nephrotic syndrome in patients with LPG. Further studies are required to define the influence of immunoadsorption on long-term effects in LPG patients.

Spectrum of clinical features and type IV collagen alpha-chain distribution in Chinese patients with Alport syndrome.

BACKGROUND: Alport syndrome (AS) is a clinically and genetically heterogeneous nephropathy. The goal of the present study is to delineate clinical characteristics and the distribution of type IV collagen chains in Chinese AS patients and to identify any alpha(IV)-chain expression and clinical phenotype correlation. METHODS: A total of 126 biopsy-proven patients meeting immunofluorescence criteria for the diagnosis of AS were investigated retrospectively. RESULTS: Microscope haematuria associated with proteinuria was observed as the initial symptom in 77.8% of the patients; 59.8% showed hearing impairment and 22.9% had ocular abnormalities. Renal biopsies from 118 patients revealed mesangial proliferative glomerulonephritis (61.9%) and focal and segmental sclerosis glomerulonephritis (37.3%). Ten different distribution patterns for the type IV collagen alpha-chains were found in the kidney; six of these are presented here for the first time. Based on renal immunofluorescence findings, 113 patients (89.7%) were classified as X-linked dominant inherited AS (XLAS) and 13 (10.3%) as autosomal recessive AS (ARAS). The XLAS group was divided into typical and non-typical subgroups according to the expression patterns for the alpha3(IV)-chain. Clinical phenotypes were more severe in XLAS patients than in ARAS patients and the prognosis was poorer in typical XLAS patients than non-typical XLAS patients. CONCLUSION: In China, the incidence of XLAS is 89.7% and 10.3% for ARAS. Chinese patients with AS have various distribution patterns of type IV collagen alpha-chains. The distribution pattern of type IV collagen alpha-chains in the kidney may correspond to the severity of the clinical phenotype.