Monoclonal gammopathies of renal significance.

The term monoclonal gammopathy of renal significance (MGRS) comprises a group of diseases pathogenetically characterised by proliferation of a B-cell or plasma cell clone that synthesises and secretes a monoclonal immunoglobulin or its components (light and/or heavy chains), that may deposit and cause glomerular, tubular, interstitial and/or vascular damage. The importance of differentiating the term MGRS from other monoclonal gammopathies lies in the fact that diagnostic and therapeutic procedures aimed at controlling monoclonal protein synthesis and secretion can be indicated, irrespective of the classic criteria based on malignant tumour expansion. Renal pathology associated with MGRS is highly heterogeneous, and therefore renal biopsy should be considered a key diagnostic tool. A precise diagnostic approach, however, must also identify the monoclonal protein in plasma and/or in urine, together with a complete haematological study in order to determine the nature and extension of cell clones. Recent advances in the understanding of these entities have resulted in significant improvements in clinical course and survival in several forms of MGRS, although more studies and clinical experience are needed in order to delineate more effective therapeutic strategies. In this review, we summarise the main clinical and pathological features of MGRS, highlighting the most appropriate diagnostic approach and current therapeutic options.