Clinical and Prognostic Factors in Patients with IgG4-Related Kidney Disease.

Background: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined.

Methods: We conducted an observational cohort study using data from 35 sites in two European countries.

Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy.

Immunotactoid glomerulopathy is a rare disease defined by glomerular microtubular immunoglobulin deposits. Since management and long-term outcomes remain poorly described, we retrospectively analyzed results of 27 adults from 21 departments of nephrology in France accrued over 19 years. Inclusion criteria were presence of glomerular Congo red-negative monotypic immunoglobulin deposits with ultrastructural microtubular organization, without evidence for cryoglobulinemic glomerulonephritis.

[A systemic disease mimicking a rheumatoid arthritis].

Introduction: Rare systemic diseases such as amyloidosis can mimic inflammatory rheumatic diseases. Because of their poor prognosis, physicians should rule them out at the onset of inflammatory rheumatism. We report a case of AL amyloidosis misdiagnosed as rheumatoid arthritis.

Acute Renal Colic Due to Immunoglobulin Free Light Chain Kidney Stones: A Case Report of an Unusual Complication of Multiple Myeloma.

Kidney failure is common in patients with a monoclonal gammopathy, most frequently due to hypercalcemia or myeloma cast nephropathy. Immunoglobulin crystallization is an uncommon phenomenon that also results in kidney injury. We report the case of a 74-year-old man with recurrent renal colic and acute kidney injury.

Randall-type monoclonal immunoglobulin deposition disease: novel insights from a nationwide cohort study.

Monoclonal immunoglobulin deposition disease (MIDD) is a rare complication of B-cell clonal disorders, defined by Congo red negative-deposits of monoclonal light chain (LCDD), heavy chain (HCDD), or both (LHCDD). MIDD is a systemic disorder with prominent renal involvement, but little attention has been paid to the description of extrarenal manifestations. Moreover, mechanisms of pathogenic immunoglobulin deposition and factors associated with renal and patient survival are ill defined.

Severe tubulointerstitial nephritis: tracking tuberculosis even in the absence of renal granuloma.

Extra-pulmonary tuberculosis is frequently located in the kidneys and, in such cases, could be associated with a granulomatous interstitial nephritis. Granulomas are not always detected, especially in human immunodeficiency virus (HIV)-positive patients. We report here a case of tubulointerstitial nephritis without granulomas in an HIV-negative patient.

[Pulmonary embolism as a presentation of nephrotic syndrome].

Background: Nephrotic syndrome (NS) in adults is defined by proteinuria>3g/24h or 50mg/kg/d, hypoproteinemia<60g/24h and hypoalbuminemia<30g/L. The final diagnosis is guided by the histopathology evidence when a renal biopsy is possible. The consequences of NS are multiple: high blood pressure, undernutrition, infections and a hypercoagulable state.

New insights into renal toxicity of the B-RAF inhibitor, vemurafenib, in patients with metastatic melanoma.

Purpose: Vemurafenib (VMF) is a B-RAF inhibitor used in the treatment of B-RAF-V600-mutant metastatic melanomas. Reports of acute kidney injury (AKI) in patients treated with VMF are scarce.

Methods: To investigate the incidence and severity of AKI, we conducted a retrospective, observational, monocentric study in the Lyon Sud Hospital University, France, which included 74 patients with metastatic B-RAF-mutated melanomas treated with VMF, between June 2011 and August 2014.