{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T22:35:57Z","timestamp":1764714957572,"version":"3.40.5"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,2,25]],"date-time":"2021-02-25T00:00:00Z","timestamp":1614211200000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Scleroderma renal crisis (SRC) is a rare but feared complication with high morbidity and mortality. Its aetiopathogenesis is unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To investigate epidemiological, serologic and clinical features of all patients with SRC listed on the population\u2010based South Australian Scleroderma Register and to examine possible factors in aetiopathogenesis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A case note review was performed on all SRC patients with relevant data extracted to determine incidence, clinical phenotype, presence of autoantibodies and survival. Possible precipitating and aetiopathogenic factors were also examined. Data from the South Australian Scleroderma Register and Australia Bureau of Statistics was sourced for comparative purposes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Over the 34\u2010year period (1985\u20132018), 30 patients (21 females, 9 males) presented with SRC giving a South Australian mean annual incidence of 0.58\/million\/year (95% CI 0.39\u20130.89). Twenty\u2010eight of these patients had diffuse cutaneous scleroderma and two with limited cutaneous scleroderma. The mean age at first symptom of scleroderma was 51.2 \u00b1\u200915.9 (mean \u00b1 SD) years with SRC occurring 4.6\u2009years later (median = 3.0\u2009years, range 0.1\u201320\u2009years). Possible precipitating factors for SRC included high dose steroids in five patients. Twelve patients were anti\u2010 RNA polymerase3 (RNAPol3) positive and two were anti\u2010topoisomerase 1 (Topo1) positive. Renal outcome was poor with 13 patients requiring renal replacement therapy and two proceeding to renal transplantation. The mean age at death was 61.2 \u00b1\u200911.6\u2009years with SRC patient survival being significantly shorter than patients with diffuse scleroderma without renal involvement (<jats:italic>P<\/jats:italic> =\u20090.002). There was no significant difference in survival between the 1985\u20132002 and the 2003\u20132018 SRC cohorts (<jats:italic>P<\/jats:italic> =\u20090.2). Nailfold capillaroscopy performed in 10 patients revealed extensive microvascular damage with prominent capillary drop out.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>SRC is a rare occurrence with an incidence of 0.58\/million\/year in South Australia. This frequency has not changed over time. It continues to have a severe adverse outcome with frequent requirement for renal replacement therapy and poor survival. Nailfold capillaroscopy revealed evidence of extensive capillary damage. No improvement in survival was observed over the 34\u2010year study period.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.14772","type":"journal-article","created":{"date-parts":[[2020,2,11]],"date-time":"2020-02-11T10:01:01Z","timestamp":1581415261000},"page":"235-242","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Scleroderma renal crisis: observations from the South Australian Scleroderma Register"],"prefix":"10.1111","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8475-4376","authenticated-orcid":false,"given":"Yassmin","family":"Khadra","sequence":"first","affiliation":[{"name":"Department of Immunology Flinders Medical Centre  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8197-4565","authenticated-orcid":false,"given":"Jennifer G.","family":"Walker","sequence":"additional","affiliation":[{"name":"Department of Immunology Flinders Medical Centre  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8179-0566","authenticated-orcid":false,"given":"Paul","family":"Hakendorf","sequence":"additional","affiliation":[{"name":"Department of Immunology Flinders Medical Centre  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3046-9884","authenticated-orcid":false,"given":"Susanna","family":"Proudman","sequence":"additional","affiliation":[{"name":"Rheumatology Unit Royal Adelaide Hospital  Adelaide South Australia Australia"},{"name":"Discipline of Medicine University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maureen","family":"Rischmueller","sequence":"additional","affiliation":[{"name":"Rheumatology Unit Queen Elizabeth Hospital  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8289-4922","authenticated-orcid":false,"given":"Catherine L.","family":"Hill","sequence":"additional","affiliation":[{"name":"Rheumatology Unit Royal Adelaide Hospital  Adelaide South Australia Australia"},{"name":"Discipline of Medicine University of Adelaide  Adelaide South Australia Australia"},{"name":"Rheumatology Unit Queen Elizabeth Hospital  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7417-7691","authenticated-orcid":false,"given":"Samuel","family":"Whittle","sequence":"additional","affiliation":[{"name":"Discipline of Medicine University of Adelaide  Adelaide South Australia Australia"},{"name":"Rheumatology Unit Queen Elizabeth Hospital  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5211-7417","authenticated-orcid":false,"given":"Eliza","family":"Pontifex","sequence":"additional","affiliation":[{"name":"Department of Immunology Flinders Medical Centre  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4330-6398","authenticated-orcid":false,"given":"Peter J.","family":"Roberts\u2010Thomson","sequence":"additional","affiliation":[{"name":"Department of Immunology Flinders Medical Centre  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,2,25]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2016.124"},{"key":"e_1_2_7_3_1","first-page":"425","volume-title":"Systemic Sclerosis","author":"Steen V","year":"1996"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197906213002505"},{"key":"e_1_2_7_5_1","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/S0307-742X(21)00059-X","article-title":"Progressive systemic sclerosis: kidney involvement","volume":"5","author":"Kahaleh MB","year":"1979","journal-title":"Clin Rheum Dis"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(74)90757-8"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1445-5994.2001.00048.x"},{"key":"e_1_2_7_8_1","first-page":"202","article-title":"Scleroderma (systemic sclerosis). 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