Systemic autoimmune and autoinflammatory disease are associated with all types of diffuse kidney disease, that is, glomerulonephritis (including rapidly progressive), thrombotic microangiopathy, tubulointerstitial nephritis, and AA-amyloidosis. However, the occurrence and types of nephropathies differ significantly in patients with various systemic immunemediated inflammatory diseases. For example, glomerulonephritis is one of the leading manifestation of systemic lupus erythematosus and small-vessels vasculitis, thrombotic angiopathy occurs frequently in patients with both primary and secondary antiphosholipid syndrome, tubulointerstitial nephritis is frequently found in patients with Sjogren syndrome and IgG4-associated disease, whereas AA-amyloidosis can complicate rheumatoid arthritis, ankylosing spondilytis, psoriatic arthritis, familial Meditarranean fever, and certain other monogenic autoinflammatory diseases. Over last decades, renal survival in patients with autoimmune and autoinflammatory diseases improved significantly due to earlier diagnosis and development of effective immunosuppressive and anti-inflammatory treatments. However, a proportion of patients still present with progressive impairment of kidney function that can be unrelated to activity of underlying diseases.