AI Article Synopsis
- Systemic mastocytosis (SM) is a condition characterized by the abnormal accumulation of mast cells in various tissues, commonly due to mutations in the KIT gene, leading to their survival and proliferation.
- Although the kidneys are rarely directly affected by SM, there are increasing instances of indirect involvement, particularly associated with various kidney conditions such as immune-mediated glomerulonephritis.
- The treatment with new anti-neoplastic agents for advanced SM may lead to kidney dysfunction, and kidney injuries have also been reported in cases where SM is linked to plasma cell disorders.
Article Abstract
Systemic mastocytosis (SM) is a disorder of excessive mast cell accumulation in tissues due to a somatic gain-of-function mutation, commonly in the KIT gene, which prevents apoptosis of mast cells. Whereas bone marrow, skin, lymph nodes, spleen and gastrointestinal tract are commonly involved, kidneys are rarely involved directly by SM. However, there are increasing reports of indirect kidney involvement in patients with SM. Novel anti-neoplastic agents to treat advanced forms of SM include non-specific tyrosine kinase inhibitors, which are reported to be associated with kidney dysfunction in some patients. SM is also associated with immune-mediated glomerulonephritis (GN) such as mesangioproliferative GN, membranous nephropathy and diffuse proliferative GN. Kidney injury, in the form of monoclonal deposition disease and primary light chain amyloidosis, is reported in SM associated with plasma cell dyscrasia. In this narrative review we discuss the various ways kidneys (and the urinary tract) are involved in patients with SM.
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| http://dx.doi.org/10.1093/ndt/gfad081 | DOI Listing |
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