肾脏病学与肾脏疾病杂志

Primary Membranous Nephropathy: Non-invasive diagnosis and evidence based management

Akbar Mahmood

This presentation is a clinical scenario of a young adult who presented with nephrotic syndrome complicated with pulmonary embolism, resistant oedema and pleural effusion associated with constitutional symptoms. Keeping in view the fact that nephrotic syndrome is not a diagnosis per se in-fact a manifestation of a unique underlying condition. Efforts must be put in to hunt for the culprit cause which needs to be treated in its own right while managing complications and symptoms. Extended evaluation was conducted in this particular case to find the cause which revealed the presence of anti-phospholipase receptor antibodies (aPLA2R). Furthermore age appropriate cancer screening was reassuring that aPLA2R is consistent with the diagnosis of primary membranous nephropathy in this scenario. Detection of aPLA2R has diagnostic and prognostic value. Serum levels are considered a guiding tool to monitor treatment. We confidently diagnosed idiopathic membranous nephropathy based on the antibody titre without the kidney biopsy and managed it with rituximab uneventfully by confirming the periodic decline in titre. Aim of sharing our experience through this clinical case is to highlight two important points. First, patients can be saved from kidney biopsy which is an invasive procedure associated with procedure related risks and morbidity. Secondly, opting rituximab for induction and maintenance of remission as a first choice drug which is highly evidence based strategy in terms of tolerability, ease of administration, side effect profile and less relapse rates of the disease. Landmark trials and research work has been reviewed extensively during the management of this complex case. Critical appraisal of relevant literature has been discussed. After academic discussion based on historical treatment modalities, rituximab has been opted as a standard of care. Management strategies for refractory and resistant cases has also been highlighted by comparatively discussing role of cyclophosphamide, cyclosporine and steroid use. Future treatment has also been summarized.