Andreas Kronbichler, Austria
Medical University Innsbruck Internal Medicine IV (Nephrology and Hypertension)Presenter of 2 Presentations
RITUXIMAB IN ADULT MINIMAL CHANGE DISEASE AND FOCAL SEGMENTAL GLOMERULOSCLEROSIS - WHAT IS KNOWN AND WHAT IS STILL UNKNOWN?
Abstract
Background and Aims
Primary forms of minimal change disease and focal segmental glomerulosclerosis are rare podocytopathies and clinically characterized by nephrotic syndrome.
Methods
Glucocorticoids are the cornerstone of the initial immunosuppressive treatment in these two entities. Especially among adults with minimal change disease or focal segmental glomerulosclerosis, relapses, steroid dependence or resistance are common and necessitate re-initiation of steroids and other immunosuppressants.
Results
Effective steroid-sparing therapies and introduction of less toxic immunosuppressive agents are urgently needed to reduce undesirable side effects, in particular for patients whose disease course is complex. Rituximab, a B cell depleting monoclonal antibody, is increasingly used off-label in these circumstances. While a growing body of evidence supports the use of rituximab in membranous nephropathy, data are less clear for adult patients with minimal change disease and focal segmental glomerulosclerosis.
Conclusions
Hence, critical questions concerning drug-safety, long-term efficacy and the optimal regimen for rituximab-treatment remain unanswered. Evidence in the form of large, multicenter studies and randomized controlled trials are urgently needed to overcome these limitations.
CLINICAL ASSOCIATIONS OF RENAL INVOLVEMENT IN ANCA-ASSOCIATED VASCULITIS
Abstract
Background and Aims
Renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is associated with significant morbidity and higher mortality rates. This study examined clinical manifestations associated with renal involvement in ANCA-associated vasculitis within a large, international cross-sectional cohort.
Methods
Univariate and multivariate analyses were performed to identify clinical factors associated with renal disease, which was defined as i) a serum-creatinine > 30% above normal and a fall in creatinine-clearance > 25%; or ii) haematuria attributable to active vasculitis.
Results
The study cohort include 1230 patients from 31 countries; 723 (58.8%) presented with renal involvement: microscopic polyangiitis (82.2%), granulomatosis with polyangiitis (58.6%), and eosinophilic granulomatosis with polyangiitis (26.4%). The following clinical and laboratory factors were more common among patients with renal disease: age (p=0.001), fever (p<0.001), fatigue (p=0.005), weight loss (p=0.001), polyarthritis (p=0.036), petechiae/purpura (p=0.022), pulmonary haemorrhage (p=0.014), gastrointestinal symptoms (p=0.002), seizures (p=0.016), lower serum albumin (p<0.001), higher CRP (p=0.038), low serum C3 at baseline (p=0.015), myeloperoxidase- (p<0.001) and proteinase 3-ANCA (p=0.020). The following clinical factors were less common among patients with renal disease: mononeuritis multiplex (p=0.041), proptosis/exophthalmos (p=0.001), nasal polyps, septal defect/perforation (p<0.001 each), respiratory distress/pulmonary fibrosis/asthma (p<0.001), and wheeze/obstructive airway disease (p<0.001).
Conclusions
In this large international study, several clinical and laboratory factors were identified as associated with renal involvement in ANCA-associated vasculitis.