Abstract
Background: IgA nephropathy was a syndrome of uniform morphology, diverse clinical features and uncertain prognosis, presence of crescents around blood vessels indices the unfavorable long-term prognosis, if the proportion of crescent in glomeruli exceeding ratio of 50%, we give the active treatment of prednisone pulse therapy, but in most condition, IgA nephropathy was diagnosed with a few of the proportion of crescent, these patients are the precondition of crescent IgA nephropathy or a special group of disease? Steroid therapy is suitable for these patient, especially for the patient with bulk fibrous crescent in renal tissue? How about the prognosis of IgA nephropathy with a few quantity of crescent formation, so we extended our original study to aim directly at above assumption. In this study, we report the results of an extended 2-year follow-up of our original randomized cohort of IgAN subjects who had crescentic formation using either steroid therapy alone or steroid pulse one kind of immunodepressant. Objective: To observe the clinical and pathological characteristics of IgA nephropathy ( IgAN) with a few quality crescent formation in 63 patients. Methods: Clinic pathological data of 63 patients with IgAN accompanied by crescents were analyzed. These patients were accepted in the group and were renal biopsied according to the percentage of glomeruli affected by crescents less than 50% during 2007-2008, and their clinical and laboratory data were collected. Results: (1) Clinical features: all the patients aged 28.3 ± 5.6 years had hematuria and proteinuria and gross hematuria (28.6%) and large mounts of proteinuria were also common, protein excreted in urine was more than 3.5 g per day in 14.2% of the patients. The patient's accompained by hypertension was 60.3% and five patients present with malignant hypertension, and acute renal insufficiency were found by 7.9%. 38 patients present with increased serum level of IgA immuglobin. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 47%.Most crescents were cellular. All the cases had a diffuse mesangial proliferation and all the patients presented deposition of IgA, IgM and C3 in mesangial area. There were 9 specimens combined with the deposition of IgA around capillary. 18 patients were given steroid therapy with renal function ameliorated, serum creatinine of 3 patients with fibrous crescent formation were obviously decreased after large amount of steroid invention. Conclusion: The main clinical features of IgAN with crescent formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria and some patients with AKI. However, the severity of clinical features and number of crescent was not positively relative. The hematuria, elevated plasma IgA and tonsils edema were independent risk factors of crescent formation in renal tissue. The steroid therapy for the patient with fibrous crescent in renal tissue showed a promising clinical effect, especially for the patients with fibrous crescent formation, so the clinical manifestation combined with pathological injury of renal tissue may contribute to the decision of therapy protocol.
Highlights
Clinical observations of patients who have undergone renal transplantation have provided strong support for the notion that IgA nephropathy is a systemic disease
IgA nephropathy was a syndrome of uniform morphology, diverse clinical features and uncertain prognosis, especially for the pathological type of crescent formation in the renal tissue, some patient of this type manifest with only mild microscopic hematuria, but with rather quatity of crescent in glomerular, except for renal biopsy, the clinical and experimental index establishment of judgement for the pathological changes degree are in demand
The energetic treatment for patient with crescent formation not more than 50% are invariably ignored by therapy decision maker, What is more, the clinical effect of steroid therapy for the patient with fibrous crescent in renal tissue especially for the patients with fibrous crescent formation is disputed, whether the long-term therapy of small dose of steroid is needed for the patient with mild laboratory change but with crescent formation are due to be identified
Summary
Clinical observations of patients who have undergone renal transplantation have provided strong support for the notion that IgA nephropathy is a systemic disease. IgA nephropathy was a syndrome of uniform morphology, diverse clinical features and uncertain prognosis, especially for the pathological type of crescent formation in the renal tissue, some patient of this type manifest with only mild microscopic hematuria, but with rather quatity of crescent in glomerular, except for renal biopsy, the clinical and experimental index establishment of judgement for the pathological changes degree are in demand. IgA nephropathy was a syndrome of uniform morphology, diverse clinical features and uncertain prognosis, presence of crescents around blood vessels indices the unfavorable long-term prognosis, if the proportion of crescent in glomeruli exceeding ratio of 50%, we give the active treatment of prednisone pulse therapy, but in most condition, IgA nephropathy was diagnosed with a few of the proportion of crescent, these patient are the precondition of crescent IgA nephropathy or a special group of disease? Objective: To observe the clinical and pathological characteristics of IgA nephropathy ( IgAN) with a few quality crescent formation in 63 patients
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