Recurrent or de novo IgA nephropathy with crescent formation after renal transplantation

IgA nephropathy is the most common glomerular disease in China, accounting for 38.8% of primary glomerular disease. It has been reported that 20.8% patients of IgA nephropathy had a different degree of crescent formation. From January 1995 to December 2004, 1000 patients had undergone cadaveric renal transplantation, and 1742 allograft renal biopsies were reviewed in the Department of Nephrology at Jinling Hospital, Nanjing University. Among them, 18 cases were found with crescent formation, in which 10 patients were diagnosed as recurrent or de novo IgA nephropathy because their immunofluorescence showed strong IgA deposition in mesangial area and capillary. The initial treatment protocol was CsA+Azp+Pred, except in two cases of CsA+MMF+Pred. There were 8 males and 2 females, with ages from 25 to 69 (mean of 37.1) years old. All of them showed progressive renal dysfunction with increasing level of serum creatinine ranged from 1.48 to 6.25 mg/dL. Seven cases presented edema with an increasing level of proteinuria (1.36 to 3.58 g/24hr), and nine cases presented with hematuria ranging from 50 to 1250 x 10(4)/mL (one showed gross hematuria). In pathological examinations, they showed mesangial proliferation and matrix expansion with 10% to 66.7% crescents (mean of 37.5%) in their allograft renal biopsy's samples. All patients changed their immunosuppressive regimens; however, nine of them eventually advanced to ESRD and returned to hemodialysis after 6 to 36 months. Two cases received second renal transplantation after six months to five years, and one kept stable renal function with 2.5 mg/dL of serum creatinine after three years of follow-up. IgA nephropathy with crescentic formation was not rare in renal allografts or native glomerulonephritis in Chinese patients. These patients showed rapidly progressive renal dysfunction, and most of them lost graft function and needed hemodialysis therapy.     

伴与不伴节段性新月体形成的IgA肾病临床病理比较

新月体型IgA肾病(IgAN)常伴有袢坏死、血栓形成等,病情进展迅速[1-3].但我们在临床上也观察到一部分IgAN患者病理上虽然有新月体形成,但新月体的数目较少,且许多新月体是节段性的小新月体,肾小球的包曼囊壁并不断裂.伴有节段性新月体的IgAN的临床和病理是否具有特殊性,目前尚无定论.本研究比较伴与不伴节段性新月体形成的IgAN患者在临床和病理等方面的差异,以期明确这类IgAN患者的特点.     

中等剂量甲泼尼龙冲击治疗伴新月体形成的IgA肾病

我们自2006年12月至2008年7月应用中等剂最甲泼尼龙冲击治疗伴部分新月体形成的IgA.肾病,取得满意疗效,现报道如下.     

A comparison of corticosteroid and warfarin therapy in IgA nephropathy with crescent formation: preliminary trial

Background. No satisfactory treatment exists for IgA nephropathy (IgAN), especially in patients with severe histologic damage. Several trials using steroids combined with other therapies such as warfarin have demonstrated unremarkable results. We investigated the renoprotective effects of warfarin and steroids in IgAN patients with crescent formation. Methods. Fifteen Japanese patients with IgAN were followed for up to 3 years. Crescent formation was recognized in over half of their glomeruli from renal biopsy specimens. Treatments consisted of either 0.5 mg/kg per day of prednisolone, or warfarin monotherapy. Blood pressure was controlled with long-acting calcium channel blockers and alpha-beta blockers. Serum creatinine and urinary protein excretion were evaluated at least every 2 months for 36 months. Results. Ten of the 15 patients completed the study. The serum creatinine levels had increased in both groups by 3 years, but significantly more so in the group treated with warfarin. However, they were not significantly different between the two groups as measured at the beginning and end of the study. Blood pressure for all patients in the study was maintained below 130/85 mmHg. Excluded from the study were 5 patients who experienced either peptic ulcers (n = 2, steroid group) or bleeding problems (n = 3, warfarin group). Conclusions. These results suggest that corticosteroid therapy may assist in preventing deterioration of renal function in patients with IgAN accompanied by crescent formation. However, further study would be required to decide its usefulness. Received: October 3, 2001 / Accepted: October 29, 2002 Correspondence to:H. Suzuki     

Idiopathic IgA nephropathy presenting as malignant hypertension

Three cases are reported of idiopathic IgA nephropathy (Berger's disease) presenting as malignant hypertension, with no data suggesting the underlying glomerulopathy, which was uncovered only after renal biopsy was performed. Comments are made on the validity of the pathological diagnosis, the possible pathogenetic sequence of the association, and on the eventual risks and benefits derived from performing renal biopsy in such patients.     

Idiopathic IgA nephropathy with segmental necrotizing lesions of the capillary wall

BACKGROUND: Segmental glomerular necrosis has been described in the biopsy material in a minority of patients with idiopathic IgA nephropathy in the oldest studies on this disease, but this marker of active capillaritis has received little attention in the subsequent literature, and its significance and relevance for the clinical outcome is still unknown. METHODS: Thirty-five out of 340 patients (10.3%) biopsied in our division at the San Carlo Hospital since 1974 showed active segmental necrotizing lesions. The morphological features and the natural history of this group of patients were compared with those of a control group of 229 patients who had comparable serum creatinine and extent of glomerular sclerosis, but who lacked active segmental necrosis. RESULTS: Patients with the necrotic variant showed a significantly more marked extracapillary proliferation and interstitial accumulation of monocytes and T lymphocytes and, in the segmental areas of necrotizing and extracapillary lesions, infiltration of monocytes, deposition of fibrinogen, and expression of the adhesion molecule vascular cell adhesion molecule-1. No difference was found in the presenting clinical syndrome. The clinical course was frequently characterized by acute flare ups, and the progression to end-stage renal failure was more frequent, although actuarial renal survival was not significantly worse (P = 0.07). The aggressive treatment with steroids and cyclophosphamide, carried out in 20 of the 35 patients, has probably been beneficial, justifying the multicenter controlled trial that recently has been initiated. CONCLUSIONS: Vasculitic lesions of the glomerular capillaries, with histologic and immunohistological features similar to those of Henoch-Sch?nlein purpura and antineutrophil cytoplasmic antibody-positive renal vasculitis, were found in 10% of patients with idiopathic IgAN. Clinical features at presentation did not differ from those of the other patients with IgAN, and despite of the more frequent occurrences of recurrent acute flare ups, rapid progression to end-stage renal failure was a rare phenomenon, even in untreated patients.     

弥漫性泛细支气管炎继发IgA肾病一例

弥漫性泛细支气管炎(DPB)是以两肺弥漫性呼吸性细支气管及其周围的慢性炎性反应为特征的独立性疾病,临床表现为持续性咳嗽、咳痰及活动时呼吸困难等,并发肾损害少见.我们收治DPB并发IgA肾病1例,并随访1年半,报告如下.     

Efficacy of prednisolone and mizoribine therapy for diffuse IgA nephropathy

OBJECTIVE: There have been only a few studies concerning oral prednisolone and mizoribine therapy for diffuse IgA nephritis (IgAN). We evaluated the efficacy of prednisolone and mizoribine therapy for diffuse IgAN. METHODS: We enrolled 34 patients who had been diagnosed as having diffuse IgAN with severe proteinuria during the period from 1992 to 1999. Following diagnostic renal biopsy, the patients were treated with prednisolone, mizoribine, warfarin and dilazep dihydrochloride. The clinical features, laboratory data and pathological findings between pre- and post-therapy were investigated. RESULTS: The mean urinary protein excretion after 6 months of treatment had decreased significantly compared to pre-therapy. The incidence of hematuria in post-therapy was lower than that of pre-therapy. The grading index decreased significantly from 4.8 +/- 2.1 at the first biopsy to 2.3 +/- 1.7 at the second biopsy (p < 0.001) and the staging index decreased significantly from 4.1 +/- 1.9 at the first biopsy to 2.7 +/- 2.4 at the second biopsy (p < 0.05). Macrophage infiltration and alpha-smooth muscle actin-positive cells in the glomerulus and interstitial region decreased significantly in post-therapy compared with pre-therapy. At the most recent follow-up, none of the 34 patients had renal insufficiency. CONCLUSIONS: Our study suggested that prednisolone and mizoribine therapy is effective for those patients with the risk of progression of IgAN.     

Clinicopathological features and prognosis of IgA nephropathy with crescentic formation in children

Objective To observe the clinical, pathological features and prognosis of IgA nephropathy in children with crescent formation. Methods A total of 38 cases of children with IgA nephropathy diagnosed by renal biopsy at Qilu Hospital of Shandong University were retrospectively analyzed. According to whether there were crescent formation, they were divided into crescents formation group (crescent formation≥10%, n=18) and control group consisting of children with complete clinical data and matching age, gender and course of disease (no crescent formation, n=20). The clinical, pathological features and prognosis of the two groups were compared. Results Fourteen cases (77.78%) of crescents formation group were associated with nausea hematuria, and 12 cases (66.67%) had hypertension, 8 patients in control group (40%) had gross hematuria, and 6 cases (30%) had high blood pressure. The differences were statistically significant (P＜0.05). In crescents formation group, Alb and eGFR were significantly lower than those of control group. 24 h urine protein and Scr were significantly higher than those of control group and there were significant statistically differences between two groups (P＜0.05). Nephropathological changes in crescent formation group were mainly in grade Ⅱ and grade Ⅲ and were usually combined with tubular interstitial damages. In addition to IgA immunoglobulin deposition, they often accompanied by IgG and IgM immunoglobulin deposition. In control group, nephropathological changes were mainly in grade Ⅰ and grade Ⅱand tubular interstitial damages were rare. IgA was the main form of deposition in control group. After 12 weeks steroid treatment and (or) immunosuppressive therapy, urinary protein turned negative in 7 cases in crescent formation group, 24 h urinary protein level declined by half than before in 7 cases and no obvious change in 4 cases. In control group, urinary protein turned negative in 12 cases, 6 cases showed a more than 50% decline in urinary protein level and 2 cases had no obvious change. During the 1-3 years of follow-up, renal function of 14 cases and 19 cases was normal in the crescent formation group and control group respectively, serum creatinine of 3 cases and 1 case increased mildly in the crescent formation group and control group respectively, 1 case needed dialysis treatment in the crescent formation group and the one in the control group who had slightly increasement of Scr did not reach the standard of dialysis treatment. Conclusions The clinical and pathological manifestations of children with IgA nephropathy with crescent formation are more severe and more likely to manifest renal insufficiency, suggesting that crescent formation is one of the indicators of poor prognosis.     

Idiopathic carpotarsal osteolysis with nephropathy

Idiopathic carpotarsal osteolysis is characterized by gradual lysis and resorption of bones, occurring mainly on hands and feet. It may be sporadic or hereditary and either form can manifest renal involvement. Nephropathy is seen more frequently and is more severe in the sporadic form. We present herein two new cases of unrelated boys with the sporadic form associated with nephropathy. One of the patients had focal segmental glomerulosclerosis. The other patient revealed isolated proteinuria. Received: 24 March 2000 / Revised: 21 September 2000 / Accepted: 23 September 2000