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Most forms of this syndrome respond to corticosteroids at standard doses and are, therefore, defined as steroid-sensitive nephrotic syndrome (SSNS). Immunological mechanisms and subsequent podocyte disorders play a pivotal role in SSNS and have been studied for years; however, the precise pathogenesis remains unclear. With recent advances in genetic techniques, an exhaustive hypothesis-free approach called a genome-wide association study (GWAS) has been conducted in various populations. GWASs in pediatric SSNS peaked in the human leukocyte antigen class II region in various populations. Additionally, an association of immune-related <jats:italic>CALHM6\/FAM26F<\/jats:italic>, <jats:italic>PARM1<\/jats:italic>, <jats:italic>BTNL2<\/jats:italic>, and <jats:italic>TNFSF15<\/jats:italic> genes, as well as <jats:italic>NPHS1<\/jats:italic>, which encodes nephrin expressed in podocytes, has been identified as a locus that achieves genome-wide significance in pediatric SSNS. However, the specific mechanism of SSNS development requires elucidation. This review describes an updated view of SSNS pathogenesis from immunological and genetic aspects, including interactions with infections or allergies, production of circulating factors, and an autoantibody hypothesis.<\/jats:p>","DOI":"10.1007\/s00467-021-05401-4","type":"journal-article","created":{"date-parts":[[2022,1,10]],"date-time":"2022-01-10T14:02:48Z","timestamp":1641823368000},"page":"1957-1965","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["An updated view of the pathogenesis of steroid-sensitive nephrotic syndrome"],"prefix":"10.1007","volume":"37","author":[{"given":"Tomoko","family":"Horinouchi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kandai","family":"Nozu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0378-8174","authenticated-orcid":false,"given":"Kazumoto","family":"Iijima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,1,10]]},"reference":[{"key":"5401_CR1","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1016\/s0140-6736(18)30536-1","volume":"392","author":"DG Noone","year":"2018","unstructured":"Noone DG, Iijima K, Parekh R (2018) Idiopathic nephrotic syndrome in children. 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