A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exc...
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| Veröffentlicht in: | Internal Medicine 2018/06/01, Vol.57(11), pp.1617-1623 |
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| creator | Ono, Masafumi Ohashi, Naro Namikawa, Akio Katahashi, Naoko Ishigaki, Sayaka Tsuji, Naoko Isobe, Shinsuke Iwakura, Takamasa Sakao, Yukitoshi Tsuji, Takayuki Kato, Akihiko Fujigaki, Yoshihide Shimizu, Akira Yasuda, Hideo |
| description | A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS. |
| doi_str_mv | 10.2169/internalmedicine.0228-17 |
| format | Article |
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She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS.</description><identifier>ISSN: 0918-2918</identifier><identifier>EISSN: 1349-7235</identifier><identifier>DOI: 10.2169/internalmedicine.0228-17</identifier><identifier>PMID: 29434134</identifier><language>eng</language><publisher>Japan: The Japanese Society of Internal Medicine</publisher><subject>atypical hemolytic uremic syndrome ; Autoimmune diseases ; Biopsy ; Case Report ; eculizumab ; Health risk assessment ; Hemolytic uremic syndrome ; Internal medicine ; Lupus ; Lupus nephritis ; Nephritis ; pseudotubulization ; Shiga toxin ; Systemic lupus erythematosus ; Thrombotic microangiopathy</subject><ispartof>Internal Medicine, 2018/06/01, Vol.57(11), pp.1617-1623</ispartof><rights>2018 by The Japanese Society of Internal Medicine</rights><rights>Copyright Japan Science and Technology Agency 2018</rights><rights>Copyright © 2018 by The Japanese Society of Internal Medicine 2018</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c610t-7c48383652abc25b86f1081000b9b495de33838686b48fd0b488fe5d71f8bbfb3</citedby><cites>FETCH-LOGICAL-c610t-7c48383652abc25b86f1081000b9b495de33838686b48fd0b488fe5d71f8bbfb3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6028688/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6028688/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,1877,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29434134$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ono, Masafumi</creatorcontrib><creatorcontrib>Ohashi, Naro</creatorcontrib><creatorcontrib>Namikawa, Akio</creatorcontrib><creatorcontrib>Katahashi, Naoko</creatorcontrib><creatorcontrib>Ishigaki, Sayaka</creatorcontrib><creatorcontrib>Tsuji, Naoko</creatorcontrib><creatorcontrib>Isobe, Shinsuke</creatorcontrib><creatorcontrib>Iwakura, Takamasa</creatorcontrib><creatorcontrib>Sakao, Yukitoshi</creatorcontrib><creatorcontrib>Tsuji, Takayuki</creatorcontrib><creatorcontrib>Kato, Akihiko</creatorcontrib><creatorcontrib>Fujigaki, Yoshihide</creatorcontrib><creatorcontrib>Shimizu, Akira</creatorcontrib><creatorcontrib>Yasuda, Hideo</creatorcontrib><title>A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome</title><title>Internal Medicine</title><addtitle>Intern. Med.</addtitle><description>A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS.</description><subject>atypical hemolytic uremic syndrome</subject><subject>Autoimmune diseases</subject><subject>Biopsy</subject><subject>Case Report</subject><subject>eculizumab</subject><subject>Health risk assessment</subject><subject>Hemolytic uremic syndrome</subject><subject>Internal medicine</subject><subject>Lupus</subject><subject>Lupus nephritis</subject><subject>Nephritis</subject><subject>pseudotubulization</subject><subject>Shiga toxin</subject><subject>Systemic lupus erythematosus</subject><subject>Thrombotic microangiopathy</subject><issn>0918-2918</issn><issn>1349-7235</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><recordid>eNplksuO0zAUhiMEYsrAKyBLbNhksJ2bs0GqOsAgFaaCmbVlJyeNq8QOvoDCu_CuOGqpYNicszif_9_nkiSI4CtKyvqN0h6sFsMIrWqUhitMKUtJ9ShZkSyv04pmxeNkhWvCUhrDRfLMuQPGGatq-jS5oHWe5ZFcJb_W6IuwgDbCATId2oYpOPQZpt4qrxzaWXCgvdJ7JBy6660ZpfGqQZ9UY43Qe2Um4fsZ_VC-R9eq60IU2jkIrfFBhkH9FF4ZjXbGOSWHOTpFokVyRms_T6oRA7qB0QzzonpvYYzp66zb6ATPkyedGBy8OOXL5P79u7vNTbq9_fBxs96mTUmwT6smZxnLyoIK2dBCsrIjmBGMsaxlXhctZLHMSlbKnHUtjpF1ULQV6ZiUncwuk7dH3SnIONMmdmzFwCerRmFnboTi_1a06vnefOclplGWRYHXJwFrvgVwno_KNTAMQoMJjlOMSU1oUVQRffUAPZiwLHOhCpyRusRlpNiRilN2zkJ3_gzBfLkB_vAG-HIDnCwGL_9u5vzwz9IjcHsEDs6LPZwBYeMKBvhfuag4IUs8WZzJpheWg85-A_po1Ag</recordid><startdate>20180601</startdate><enddate>20180601</enddate><creator>Ono, Masafumi</creator><creator>Ohashi, Naro</creator><creator>Namikawa, Akio</creator><creator>Katahashi, Naoko</creator><creator>Ishigaki, Sayaka</creator><creator>Tsuji, Naoko</creator><creator>Isobe, Shinsuke</creator><creator>Iwakura, Takamasa</creator><creator>Sakao, Yukitoshi</creator><creator>Tsuji, Takayuki</creator><creator>Kato, Akihiko</creator><creator>Fujigaki, Yoshihide</creator><creator>Shimizu, Akira</creator><creator>Yasuda, Hideo</creator><general>The Japanese Society of Internal Medicine</general><general>Japan Science and Technology Agency</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20180601</creationdate><title>A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome</title><author>Ono, Masafumi ; Ohashi, Naro ; Namikawa, Akio ; Katahashi, Naoko ; Ishigaki, Sayaka ; Tsuji, Naoko ; Isobe, Shinsuke ; Iwakura, Takamasa ; Sakao, Yukitoshi ; Tsuji, Takayuki ; Kato, Akihiko ; Fujigaki, Yoshihide ; Shimizu, Akira ; Yasuda, Hideo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c610t-7c48383652abc25b86f1081000b9b495de33838686b48fd0b488fe5d71f8bbfb3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>atypical hemolytic uremic syndrome</topic><topic>Autoimmune diseases</topic><topic>Biopsy</topic><topic>Case Report</topic><topic>eculizumab</topic><topic>Health risk assessment</topic><topic>Hemolytic uremic syndrome</topic><topic>Internal medicine</topic><topic>Lupus</topic><topic>Lupus nephritis</topic><topic>Nephritis</topic><topic>pseudotubulization</topic><topic>Shiga toxin</topic><topic>Systemic lupus erythematosus</topic><topic>Thrombotic microangiopathy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ono, Masafumi</creatorcontrib><creatorcontrib>Ohashi, Naro</creatorcontrib><creatorcontrib>Namikawa, Akio</creatorcontrib><creatorcontrib>Katahashi, Naoko</creatorcontrib><creatorcontrib>Ishigaki, Sayaka</creatorcontrib><creatorcontrib>Tsuji, Naoko</creatorcontrib><creatorcontrib>Isobe, Shinsuke</creatorcontrib><creatorcontrib>Iwakura, Takamasa</creatorcontrib><creatorcontrib>Sakao, Yukitoshi</creatorcontrib><creatorcontrib>Tsuji, Takayuki</creatorcontrib><creatorcontrib>Kato, Akihiko</creatorcontrib><creatorcontrib>Fujigaki, Yoshihide</creatorcontrib><creatorcontrib>Shimizu, Akira</creatorcontrib><creatorcontrib>Yasuda, Hideo</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ono, Masafumi</au><au>Ohashi, Naro</au><au>Namikawa, Akio</au><au>Katahashi, Naoko</au><au>Ishigaki, Sayaka</au><au>Tsuji, Naoko</au><au>Isobe, Shinsuke</au><au>Iwakura, Takamasa</au><au>Sakao, Yukitoshi</au><au>Tsuji, Takayuki</au><au>Kato, Akihiko</au><au>Fujigaki, Yoshihide</au><au>Shimizu, Akira</au><au>Yasuda, Hideo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. Med.</addtitle><date>2018-06-01</date><risdate>2018</risdate><volume>57</volume><issue>11</issue><spage>1617</spage><epage>1623</epage><pages>1617-1623</pages><issn>0918-2918</issn><eissn>1349-7235</eissn><abstract>A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS.</abstract><cop>Japan</cop><pub>The Japanese Society of Internal Medicine</pub><pmid>29434134</pmid><doi>10.2169/internalmedicine.0228-17</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Internal Medicine, 2018/06/01, Vol.57(11), pp.1617-1623 |
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| source | J-STAGE Free; PubMed Central; PubMed Central Open Access |
| subjects | atypical hemolytic uremic syndrome Autoimmune diseases Biopsy Case Report eculizumab Health risk assessment Hemolytic uremic syndrome Internal medicine Lupus Lupus nephritis Nephritis pseudotubulization Shiga toxin Systemic lupus erythematosus Thrombotic microangiopathy |
| title | A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome |
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