Immunotactoid glomerulopathy - an enigmatic case in the setting of nodal marginal zone lymphoma and systemic sclerosis sine scleroderma.

BACKGROUND: Immunotactoid Glomerulopathy (ITG) is an exceedingly rare type of glomerulopathy characterised by distinctive electron microscopic features. ITG has been linked to lymphoproliferative or autoimmune disorders. The clinical manifestations are diverse including nephrotic syndrome (NS), haematuria, acute kidney injury and end stage renal failure (ESRD). We present a case with a stage 3 Nodal Marginal Zone Lymphoma (NMZL) and systemic sclerosis sine scleroderma (SSSS), where the evolution of ITG was documented in 2 renal biopsies 19 months apart. To the best of our knowledge, no cases have been reported linking ITG to NMZL. Furthermore, there is only one non-peer reviewed report linking ITG to scleroderma. We discuss the implications of our findings and highlight the satisfactory management of the case. CASE PRESENTATION: A 79-year-old female with history of systemic sclerosis sine scleroderma and stage 3 NMZL presented with acute kidney injury and NS on a background of chronic kidney disease. Her first kidney biopsy showed a diffuse proliferative glomerulonephritis and her serum protein electrophoresis showed no abnormalities. She was managed satisfactorily with conservative measures. She returned 19 months later with features of fluid overload, increasing proteinuria and rising serum creatinine. A repeat serum protein electrophoresis showed excess free kappa light chains and ITG was detected in the repeat kidney biopsy. Her kidney function and proteinuria showed a good and sustained response to rituximab administered after the second biopsy. CONCLUSION: ITG is a rare type of glomerulopathy, associated with underlying haematological malignancies and autoimmune disorders that may result in ESRD. Rituximab is one of the effective agents used in the management of ITG with haematological malignancies.

Sirolimus-based calcineurin inhibitor withdrawal immunosuppressive regimen in kidney transplantation: a single center experience.

BACKGROUND/AIM: This observational study was conducted to evaluate the safety and efficacy of the conversion from calcineurin inhibitors (CNIs) to sirolimus (SRL)-based immunosuppressive therapy in kidney transplantation. MATERIALS AND METHODS: Sixty-four kidney recipients of mean age 38.3 +/- 14.6 years were converted to SRL. The main reasons for conversion were elective in 45 (70.3%) and biopsy-proven chronic allograft nephropathy in 11 (17.2%). The primary CNI used was cyclosporine A in 51 patients. Mean time to conversion was 50.5 months. After conversion, 61 patients received mycophenolate mofetil. We evaluated the impact of conversion on renal function for 5 years post-conversion. The overall mean follow-up time was 72.8 months. RESULTS: The analysis showed significant improvement in renal function at month 3 post-conversion (P < 0.05) with stabilization thereafter. Lipid parameters and blood sugar levels were similar pre- and post-conversion. Abnormal liver function test was transient in 12.8%. Reasons for SRL discontinuation were nephrotic range proteinuria in two patients and mouth ulceration in one. We compared patients with serum creatinine <140 micromol/l and those with serum creatinine > or = 140 micromol/l, and found that serum creatinine was an independent risk factor for chronic allograft dysfunction (P = 0.02). Graft loss occurred in three patients because of cardiovascular death in two and an acute rejection episode in one. CONCLUSIONS: We concluded that conversion from CNIs to SRL is an option and of benefit without significant acute rejection episodes or chronic allograft dysfunction especially in well-selected kidney transplant recipients with good graft function.