Her laboratory results continued to show low platelet and haemoglobin levels and elevated creatinine concentrations. has been an evolving area of nephrology. The understanding of the role GSK-923295 of complement system dysregulation in kidney disease has led BBC2 to reclassification of old disease entities like membranoproliferative glomerulonephritis (MPGN). This has also led to the discovery of effective therapies for these conditions previously described as idiopathic and not effectively treated. Patients often times present first to family physicians, general internists and emergency room physicians before they are managed by nephrologists. It is important to shed light on this old disease entity but with updated classification and treatment approaches as timely diagnosis and management improves patients survival and renal outcomes. Case presentation Our patient, a 36-year-old woman who was previously healthy, except for a history of hearing loss since childhood, presented with anasarca and was found to have hypertension and proteinuria and subsequently developed generalised tonicCclonic seizures. She was transferred to the intensive care unit (ICU) where she was intubated and received antiseizure medications. A review of her medical condition showed that she had neither infectious or connective tissue disease symptoms nor prior evidence of GSK-923295 deep vein thrombosis or abortions. She did not receive any medications nor did she take oral contraceptives. She had no family history of a similar illness, and her blood pressure was 200/110?mm?Hg. Her blood results are shown in table 1. MRI of the brain showed parenchymal changes presumably related to posterior reversible encephalopathy syndrome. An electroencephalogram (EEG) showed no epileptiform discharges, and a lumbar puncture excluded a central nervous system infection. The new onset of hypertension, active urine sediment and progressive elevation of creatinine levels from an unknown baseline were all suggestive of a glomerular pathology; hence, a kidney biopsy was performed. She was treated with three doses of intravenous methylprednisolone; subsequently, her dosage was revised to 50?mg of oral prednisone daily. Her seizures resolved, and she was discharged from the hospital. Her biopsy findings revealed eight glomeruli on light microscopy with an MPGN pattern, no evidence GSK-923295 of global or segmental sclerosis, no crescents or necrosis, a single focus GSK-923295 of possible fibrin thrombus and no tubular atrophy or interstitial fibrosis. Congo red staining for amyloid fibrils was negative. The immunofluorescence results showed seven glomeruli with moderate granular staining for IgG and C3 in the capillary loops, trace staining for kappa, lambda and C1q and negative IgA and fibrin staining. Electron microscopy showed one hypercellular glomerulus with subendothelial and mesangial but no subepithelial dense deposits compatible with an immune complex-mediated MPGN (figure 1). In the nephrology outpatient clinic, the patient was clinically healthy with controlled blood pressure (table 1). Her prednisone dosage was decreased to 25?mg daily. One month later, follow-up blood work showed severe haemolytic anaemia and thrombocytopenia, which prompted hospital admission and additional laboratory tests (table 1). During her hospital stay, her seizures recurred. She appeared to have thrombotic microangiopathy (TMA) after a recent diagnosis of immune complex-mediated MPGN. Her oral prednisone dosage was increased to 70?mg daily, and plasma exchange (PLEX) was initiated, which was increased to twice a day and subsequently three times GSK-923295 per day. 1 She received a total of 13 exchanges of 5 litres each of cryosupernatant and fresh frozen plasma. She failed to respond to treatment and exhibited persistent lactate dehydrogenase (LDH) elevation. Her laboratory results continued to show low platelet and haemoglobin levels and elevated creatinine concentrations. The normal level of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13?(ADAMTS 13) and failure to respond to PLEX and conventional therapy for her renal impairment were consistent with a diagnosis of atypical haemolytic uremic syndrome (aHUS). She was administered a meningococcal vaccine and eculizumab at an initial dose of 900? mg intravenously weekly for the first 4 weeks, followed by 1200?mg for the fifth dose 7 days later and then 900? mg intravenously every 14 days thereafter. While management continued, genetic analysis was performed to screen for predisposing aHUS risk factors..