RESUMO
Membranous nephropathy (MN) is one of the most common causes of nephrotic syndrome in adults. The discovery of phospholipase A2 receptor (PLA2R) as a target antigen has led to a paradigm shift in the understanding and management of MN. At present, serum PLA2R antibodies are used for diagnosis, prognostication, and guiding treatment. Now, with the discovery of more than 20 novel target antigens, antigen mapping is almost complete. The clinical association of certain antigens provides clues for clinicians, such as the association of nerve epidermal growth factor-like 1 with malignancies and indigenous medicines. Serum antibodies are detected for most target antigens, except exostosin 1 and 2 and transforming growth factor-beta receptor 3, but their clinical utility is yet to be defined. Genome-wide association studies and studies investigating environmental factors, such as air pollution, shed more light on the underpinnings of MN. The standard therapy of MN diversified from cyclical cyclophosphamide and steroids to include rituximab and calcineurin inhibitors over the past decades. Here, we provide a cutting-edge review of MN, focusing on genetics, immune system and environmental factors, novel target antigens and their clinical characteristics, and currently available and emerging novel therapies in MN.
RESUMO
A known cerebral palsy young man presented with prolonged bouts of generalised body movements associated with high-grade fever without any localising signs of infection, requiring multiple hospital admissions over several months. All septic work-ups, including a lumbar puncture, were negative. Serum chemistry was consistent with rhabdomyolysis. Repeated electroencephalograms showed no epileptiform discharges. Cranial MRI with gadolinium contrast revealed left cerebral atrophy with hyperintensities at the left basal ganglia. Uncontrolled dystonia with concomitant rhabdomyolysis was considered. Subsequent aggressive hydration and administration of muscle relaxant afforded abrupt resolution of symptoms.
