RESUMO
Lupus-like glomerulnephritis in patients with negative lupus serologies and no extra-renal manifestations of lupus can create a diagnostic dilemma. We describe a 53-year-old gentleman with chronic hepatitis C virus (HCV) infection who presented with dialysis-requiring renal failure, renal histologic findings of "full-house" immunofluorescence label and tubuloreticular inclusions on electronic microscopy, but no extra-renal or laboratory signs of systemic lupus erythematosis. Attempted treatment with cyclophosphamide and corticosteroids was limited by cyclophosphamide hypersensitivity. The patient remained dialysis-dependent over 18 months of observation and did not develop extra-renal clinical or biological manifestations of lupus. Mimics of seronegative lupus with isolated renal involvement can include HCV-related autoimmunity. Treatment of acute glomerulonephritis may be similar initially, but other concerns in patients with HCV infection include excluding cryoglobulinemia, the potential impact of immunosuppression therapy on liver disease and consideration of subsequent use of antiviral therapy. Given the increasing prevalence of HCV globally, the recognition of extra-hepatic autoimmune manifestations of HCV infection will likely increase.
RESUMO
Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is considered a "pauci-immune" disease, characterized by absent or mild glomerular tuft staining for immunoglobulin and/or complement. We describe a 72-year-old man with progressive renal failure over five months who was found to have P-ANCA associated crescentic glomerulonephritis. Renal biopsy also revealed immunofluorescence staining for Immunoglobulin G and C3. Treatment comprised corticosteroids, cyclophosphamide, and plasmapheresis but unfortunately kidney function did not recover, likely due to substantial interstitial fibrosis at diagnosis. This case illustrates that serologic evaluation for ANCAs should not be discounted when immune deposits are present. Prompt diagnosis is warranted.
RESUMO
BACKGROUND: Clinical practice guidelines for management of chronic kidney disease (CKD) have been developed within the Kidney Disease Outcomes Quality Initiative (K/DOQI). Adherence patterns may identify focus areas for quality improvement. METHODS: We retrospectively studied contemporary CKD care patterns within a private health system in the United States, and systematically reviewed literature of reported practices internationally. Five hundred and nineteen patients with moderate CKD (estimated GFR 30-59 ml/min) using healthcare benefits in 2002-2005 were identified from administrative insurance records. Thirty-three relevant publications in 2000-2006 describing care in 77,588 CKD patients were reviewed. Baseline demographic traits and provider specialty were considered as correlates of delivered care. Testing consistent with K/DOQI guidelines and prevalence of angiotensin converting enzyme inhibitor/angiotensin receptor blocker (ACEi/ARB) medication prescriptions were ascertained from billing claims. Care descriptions in the literature sample were based on medical charts, electronic records and/or claims. RESULTS: KDOQI-consistent measurements of parathyroid hormone (7.1 vs. 0.6%, P = 0.0002), phosphorus (38.2 vs. 1.9%, P < 0.0001) and quantified urinary protein (23.8 vs. 9.4%, P = 0.008) were more common among CKD patients with versus without nephrology referral in the administrative data. Nephrology referral correlated with increased likelihood of testing for parathyroid hormone and phosphorus after adjustment for baseline patient factors. Use of ACEi/ARB medications was more common among patients with nephrology contact (50.0 vs. 30.0%; P = 0.008) but appeared largely driven by higher comorbidity burden. The literature review demonstrated similar practice patterns. CONCLUSIONS: Delivery of CKD care may be monitored by administrative data. There is opportunity for improvement in CKD guideline adherence in practice.
