Glomerular Disease Associated with Takayasu Arteritis:6 Cases Analysis and Review of the Literature

Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively.None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy,accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period,were analyzed.All of them were females with a mean age of 35.5 ± 10.0 years.Four cases presented with lower extremity edema.Laboratory tests showed that one was nephrotic syndrome,three were nephrotic range proteinuria,and two of them had mild renal dysfunction.The other two patients were asymptomatic microscopic hematuria and proteinuria.Renal pathology revealed mild immunoglobulin A nephropathy in two cases,mild mesangial proliferative glomerulonephritis(GN),membranoproliferative GN,minimal change disease,and fibrillary GN in one case respectively.Five cases received glucocorticoids and cyclophosphamide therapy.Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases.The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment.Conclusions TA may induce glomerular disease as a part of its histological spectrum.Apart from ischemic glomerular disease,glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria,that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.

Postpartum Atypical Hemolytic Uremic Syndrome:an Unusual and Severe Complication Associated with Ig A Nephropathy

IGA nephropathy （IgAN） is one of the most commontypes of primary glomerulonephritis, which occursmore frequently in patients of reproductive age.Atypical hemolytic uremic syndrome （aHUS）without diarrhea is rare and has a poor prognosis. In theabsence of appropriate therapy, pregnancy-related aHUSis associated with high morbidity and mortality.

Non-Hodgkin's Lymphoma Primarily Presenting with Fanconi Syndrome and Acute Kidney Injury

KIDNEY involvement is common in non-Hodgkin＇s lymphoma （NHL） with incidence up to 30%-40% in autopsy studies. However, it us- ually occurs late in the course of the diseaseand is clinically silent. Clinically overt renal disease including acute kidney injury （AKI） as its primary manifestation is rarely reported, moreover, Fanconi syndrome （FS） is extremely rare as the main manifestation in NHL. In this report, we presented a case of NHL primarily presenting with FS and AKI due to diffuse interstitial infiltration of NHL cells and emphasized the important role of renal biopsy, especially renal immunohistochemical analysis in the diagnosis of renal diffuse lymphoma.

Underlying IgM heavy chain amyloidosis in treatment-refractory IgA nephropathy: A case report

BACKGROUND Monoclonal immunoglobulin can cause renal damage,with a wide spectrum of pathological changes and clinical manifestations without hematological evidence of malignancy.These disorders can be missed,especially when combined with other kidney diseases.CASE SUMMARY A 61-year-old woman presented with moderate proteinuria with normal renal function.She was diagnosed with IgA nephropathy combined with monoclonal gammopathy of undetermined significance after the first renal biopsy.Although having received immunosuppressive treatment for 3 years,the patient developed nephrotic syndrome.Repeated renal biopsy and laser microdissection/mass spectrometry analysis confirmed heavy chain amyloidosis.After nine cycles of bortezomib,cyclophosphamide and dexamethasone,she achieved very good partial hematological and kidney responses.CONCLUSION Renal injury should be monitored closely in monoclonal gammopathy patients without obvious hematological malignancy,especially in patients with other preexisting renal diseases.