Renal biopsy reports in nephritic syndrome:Update

BACKGROUND Nephritic syndrome(NiS)is a major indicator of serious renal diseases necessitating kidney biopsies for histopathological evaluations,but due to the lack of comprehensive reviews in the literature,the current understanding of the syndrome and its significance is limited.AIM To collect all the evidence retrievable from the literature on the diagnoses made on the renal biopsies performed for NiS as the indication to the procedure.METHODS A literature search was conducted to find studies reporting final diagnoses on renal biopsies in NiS patients.Data were pooled and analyzed with stratifications on age and regions.Meta-analyzes were performed using Stata v.9.RESULTS Overall,26414 NiS patients from the total number of 96738 kidney biopsy diagnoses reported by 47 studies from 23 countries from all continents(except sub-Saharan Africa)were found and analyzed.NiS was the indication for renal biopsy in 21%of the patient populations across the reviewed studies.Immunoglobulin A(IgA)nephropathy was the single most frequent diagnosis in these patients(approximately 38%)followed by lupus nephritis(approximately 8%)and Henoch Schönlein purpura(approximately 7%).IgA nephropathy was the most frequent diagnosis reported for the NiS patients from the East Asia,comprising half of all the cases,and least prevalent in South Asia.Considering the age subgroups,adult(vs pediatric or elderly)patients were by far the most likely age group to be diagnosed with the IgA nephropathy.A myriad of such regional and age disparities have been found and reported.CONCLUSION As the indication for renal biopsy,NiS represents a very distinctive epidemiology of final renal disease diagnoses compared to the other major syndromes.

Immunoglobulin A glomerulonephropathy:A review

In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.

Paraneoplastic focal segmental glomerulosclerosis associated with gastrointestinal stromal tumor with cutaneous metastasis: A case report

BACKGROUND Gastrointestinal stromal tumor(GIST)with cutaneous metastasis is very rare.As a result,cutaneous GISTs have not been well characterized.Focal segmental glomerulosclerosis(FSGS)is also a rare symptom among paraneoplastic nephritic syndromes(PNS).CASE SUMMARY In this case report,we describe a patient with cutaneous metastatic GIST accompanied by nephrotic syndrome occurring as a malignancy-associated PNS,for whom symptomatic treatment was ineffective,but clinical remission was achieved after surgery.Moreover,the patient has a missense mutation in NPHP4,which can explain the occurrences of GIST and FSGS in this patient and indicates that the association is not random.CONCLUSION This is the first reported case of a GIST with cutaneous metastasis accompanied by nephrotic syndrome manifesting as a PNS.

Management and outcomes of acute post-streptococcal glomerulonephritis in children

Acute post-streptococcal glomerulonephritis(APSGN)is the major cause of acute glomerulonephritis among children,especially in low-and middle-income countries.APSGN commonly occurs following pharyngitis due to the activation of antibodies and complements proteins against streptococcal antigens through the immune-complex-mediated mechanism.APSGN can be presented as acute nep-hritic syndrome,nephrotic syndrome,and rapidly progressive glomerulonephritis,or it may be subclinical.The management of APSGN is mainly supportive in nature with fluid restriction,anti-hypertensives,diuretics,and renal replacement therapy with dialysis,when necessary,as the disease is self-limiting.Congestive heart failure,pulmonary edema,and severe hypertension-induced encephalopathy might occur during the acute phase of APSGN due to hypervolemia.APSGN generally has a favorable prognosis with only a small percentage of patients with persistent urinary abnormalities,persistent hypertension,and chronic kidney disease after the acute episode of APSGN.Decreased complement levels,increased C-reactive protein,and hypoalbuminemia are associated with disease severity.Crescent formations on renal biopsy and renal insufficiency on presentation may be the predictors of disease severity and poor outcomes in APSGN in children.

Efficacy of rituximab therapy in children with refractory nephrotic syndrome:a prospective observational study in Shanghai

Background:Idiopathic nephrotic syndrome is the most common glomerular disease in children.This study was undertaken to observe the efficacy and side-effects of rituximab(RTX)in treating children with different types of refractory primary nephrotic syndrome.Methods:Twelve patients with steroid dependent nephrotic syndrome(SDNS),frequently relapsing nephritic syndrome(FRNS),and steroid resistant nephrotic syndrome(SRNS)were enrolled in our study.There were obvious drug side-effects,and proteinuria remained difficult to control.RTX was administered at a dose of 375 mg/m2 body surface area,once or twice weekly.Results:The male to female ratio was 3:1,and the onset age was 1.6–8.9 years.There were 9 patients with steroid sensitive nephrotic syndrome(SDNS or FRNS),and 3 patients with SRNS.There were 7 patients with minimal change disease(MCD),3 patients with focal segmental glomerular sclerosis(FSGS),1 with focal proliferative glomerulonephritis,and 1 without renal biopsy.The total effective treatment rate of RTX was 91.67%,and for 77.78%of the patients,steroid dosage could be reduced.Six months before and after RTX infusion,the mean steroid dosage was significantly decreased(P=0.014)and the recurrence number was significantly reduced(P<0.001).The results were better in MCD patients than in FSGS patients(P=0.045).There was no significant difference between FRNS/SDNS and SRNS patients(P=0.175).During RTX administration,3 patients developed skin rashes,1 developed hypotension,and 1 developed a fever.One patient experienced a persistent decrease in serum immunoglobulin level but without serious infection.Conclusion:RTX was effective in the treatment of refractory nephrotic syndrome,and it could significantly reduce the use of steroid and immunosuppressants.