| Part 1 Clinical analysis of Ig A nephropathy with Anti-neutrophil CytoplasmicAntibody SeropositivityBackgroundIg A nephropathy(Ig AN) is the most common form of primary glomerulonephritis(GN) in the world and is also the majorcause of end-stage renal disease(ESRD) in patients with primary glomerulopathy. The clinical course of Ig AN is very variable and usually present with proteinuria, hematuria, hypertension, edema or renal insufficiency alone or in combination. The pathologic features of Ig AN are also very various, which vary from mesangial proliferation to severe formation of crescent and is charictered by the predominance of Ig A deposits in the glomerular mesangium and/or glomerular capillary loop.Anti-neutrophil cytoplasmic antibodies(ANCAs) are autoantibodies against myeloperoxidase(MPO) and proteinase 3(PR3), are used as a diagnostic marker which is strongly associated with a spectrum of autoimmune diseases(ANCA-associated vasculitis, AAV). The renal involvement is commonly character by pauci-immune necrotizing and crescentic GN with a very rapid decline in renal function. Pauci-immune necrotizing and crescentic GN is character with glomerular necrosis and crescent formation occur in the absence of significant cellular proliferation and lack of immune deposits in glomerular.Ig AN may also manifest as a crescent GN, but in contrast to ANCA- associated GN, it is characterized by the predominance of Ig A deposits, alone or with Ig G, Ig M, or both, in the glomerular mesangium. Interestingly, numbers of clinical observations showed that ANCAs are not only exclusive for AAV, but also can be detected in other diseases, such as membranous glomerulonephritis, lupus nephritis, and Ig AN. Several investigators observed the clinical presentation of rapidly deteriorating kidney function and heavy proteinuria accompanied with the histologic picture of crescentic glomerulonephritis in Ig AN patients with ANCAs. Of interest, some patients had extrarenal manifestations, with pulmonary involvement(hemoptysis/infiltrates) and sinusitis as a hallmark. These results suggest that Ig AN patients with ANCA seropositivity usually need immunosuppressant of active treatment. However,O‘Donoghue et al had reported that there were 2 patients with ANCA seropositivity in 100 Ig AN patients which presented slowly progressive renal failure and no crescents formation in glomorular. In this context, the role of ANCAs in Ig AN is remaining elusive. In this study, we detailed the clinical and histologic character of 14 Chinese patients with Ig AN with ANCA seropositivity and analyzed the renal outcome.MethodsA total of 787 Chinese renal biopsies showing Ig AN from the archives of the Renal Pathology Laboratory in Xinqiao Hospital of Third Military Medical University between November 2011 and November 2013 were enrolled. The degree of pathological changes was evaluated by Lee Classification of Ig AN. ANCA positivity was documented by EUROBlot kits and indirect immunofluorescence. Fourteen cases were found out that the Ig AN patients have ANCAs. The clinical and pathologic charactesr of 14 Ig AN patients with ANCA were analyzed retrospectively.Results:1. 787 Ig AN patients were analyzed retrospectively 14 patients presented ANCA positive and the rate was approximately 2%.2. 14 patients with Ig AN and ANCA, 4 men and 10 women with a mean age of 44.4±12.7 year. The results of EUROBlot kits were positive in 14 patients(12 MPO-ANCA, 2 PR3-ANCA). Indirect immuno-fluorescence was positive in 14 patients(12 P-ANCA, 2 C-ANCA).3. 3 of 14 Ig AN with ANCA positive patients had shown severe clinical manifestation, with crescents involved with mean 56% of glomeruli, including heavy proteinuria(24-hr urine protein 3.8 g/d), hematuria, and acute renal failure(creatinine 4.5±3.7 mg/dl). The remaining 11 patients with no crescents showed various degrees of proteinuria(24-hr urine protein 2.4±2.4 g/d), hematuria and serum creatinine levels(creatinine 1.4 ±1.5 mg/dl).4. 10 patients were available for follow-up with an average length of 14.0±11.2 months. Among the three patients with crescents who had been treated with steroids and cyclophosphamide, one patient became dialysis dependent at the time of biopsy and remains dialysis after treatment, another one died from acute heart failure, the last one showed improvement in renal function after treatment and had not develop end stage renal disease(ESRD) after 26 months renal biopsy. The remaining seven patients with no crescents were treated with steroids, cyclophosphamide, renin-angiotensin system inhibitors(RASI), and/or Traditional Chinese Medicine(TCM), six had stabilization or improvement in renal function, and other one progressed to ERSD with worsening renal function.Conclusions:1. These findings suggest that not all ANCA involved the pathology in Ig AN.2. The overlap of AAV and Ig AN may be the cause for poor prognosis of Ig AN patients with ANCA seropositivity.3. In patients with Ig AN and ANCAs, identification of pathogenic versus non-pathogenic ANCAs in combination with clinical and pathological features is recommended, which would help to guide the treatment on Ig AN patients with ANCAs seropositivity whether to need more active therapy. Part 2 Clinical analysis of left renal vein entrapment syndrome in combination withIg A nephropathyBackgroundLeft renal vein(LRV) entrapment syndrome(LRVES)refers to compression of theLRV most commonly between abdominal aorta and superior mesenteric artery. LRVES, also known as nutcracker syndrome(NCS), is the clinical manifestation of NCS characterized by a wide spectrum of symptoms, such as hematuria, orthostatic proteinuria, pain orgonadal vein syndrome, and varicoceles. Prevalence of LRVES is unknown. LRVES may be higher in male who are adolescents with a lank figure. LRVES is a benign disease that usually does not develop into uraemia, no treatment may be necessary if the symptoms can be tolerated.Ig A nephropathy(Ig AN) is the most common form of primary glomerulonephritis(GN) in the world and is also the most common cause of end-stage renal disease(ESRD) in patients with primary glomerular disease. Ig AN is character by the predominance of Ig A deposits, either alone or with Ig G, Ig M, or both, in the glomerular mesangium and/or glomerular capillary loop. Ig AN usually present with proteinuria, microscopic hematuria, or hypertension, alone or in combination during an upper respiratory or gastrointestinal illness. The KDIGO guideline suggests that Ig AN need treatment, if it presents hypertension, proteinuria or impairment of renal function.As imaging technology and renal biopsy technique advance, LRVES could exist with glomerulopathy such as Ig AN has been reported in clinical practice. Gross/or microscopic hematuria with or without proteinuria maybe involved in both LRVES and Ig AN, which easily leads to misdiagnose.There are few reports of LRVES in combination with Ig AN. In this study, we detail the clinical and histologic data of 6 Chinese patients with LRVES in combination with Ig AN. This data will facilitate to prompt diagnosis and treatment.MethodsIn our patients, LRVES was diagnosed by Doppler sonography. Ig AN was diagnosed by renal biopsy and the degree of pathological changes was evaluated by Lee Classification of Ig AN. The clinical and pathologic characters of 6 LRVES cases which complicated with Ig AN were analyzed retrospectively in Xinqiao Hospital of Third Military Medical University from January 2011 to January 2014.Results:1. 6 patients were male with a mean age of 16.5±4.3 years old. The body mass index(BMI) was 17.8±1.3 kg/m2.2. Clinical presentation included proteinuria, haematuria and loin pain. Pathologic evaluation revealed Lee Ⅰ-Lee Ⅲ Ig AN. One patient was Lee Ⅰ, four patients were Lee Ⅱ and one patient was Lee Ⅲ.Conclusions:1. Renal biopsy should be recommended in cases of LRVES with persistence of proteinuria, haematuria or abnormal morphology of urinary red blood cell.2. Supine/standing urinalysis combined with Doppler sonography should be considered in Ig AN patients who are adolescents with low BMI manifesting intermittent proteinuria and/or haematuria, which can be helpful for differential diagnosis and treatment to avoid over-treatment. |
PDF Full Text Request