BACKGROUND: Streptococcus A infections have been associated with immunemediated sequelae including acute glomerulonephritis, acute rheumatic fever, thrombocytopenia, hemolytic anemia, Henoch-Schönlein purpura, arthritis, uveitis, guttate psoriasis and erythema nodosum. Available reviews do not report the occurrence of acute poststreptococcal glomerulonephritis in association with one of the mentioned conditions. METHODS: We performed a systematic review of the literature on extrarenal immune-mediated disorders associated with acute poststreptococcal glomerulonephritis. The principles recommended by the Economic and Social Research Council guidance on the conduct of narrative synthesis and on the Preferred Reporting Items for Meta-Analyses and Systematic Reviews were used. RESULTS: We identified 41 original articles, published after 1965, which reported on 52 patients (34 males and 18 females aged from 1.7 to 57 years, median 9) affected by acute poststreptococcal glomerulonephritis associated with a further post-streptococcal disease: 29 cases with rheumatic fever (17 males and 12 females aged 3.0 to 57, median 17 years), 16 with hematologic diseases such as thrombocytopenia or hemolytic anemia (13 males and 3 females aged 1.8 to 13, median 6.0 years) and seven with Henoch-Schönlein syndrome, reactive arthritis or uveitis (4 males and 3 females aged 1.7 to 14, median 7.0 years). Patients affected by acute poststreptococcal glomerulonephritis associated with acute rheumatic fever were on the average older (P<0.05) than patients with acute poststreptococcal glomerulonephritis associated with thrombocytopenia, hemolytic anemia, Henoch-Schönlein syndrome, reactive arthritis or uveitis. Five large case series describing 2058 patients affected by acute poststreptococcal glomerulonephritis did not mention its occurrence in association with further immune-mediated disorders. CONCLUSION: This review points out that acute poststreptococcal glomerulonephritis can be associated, albeit rarely, with rheumatic fever, thrombocytopenia, hemolytic anemia, Henoch-Schönlein syndrome, reactive arthritis or uveitis.