{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,23]],"date-time":"2025-08-23T00:07:18Z","timestamp":1755907638910,"version":"3.44.0"},"reference-count":10,"publisher":"SMC Media","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnosis excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD. We present the case of a 64-year-old man with iMCD (HIV and HHV-8 negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratorial remission was achieved and maintained at 2-year follow-up.<\/jats:p>","DOI":"10.12890\/2021_002831","type":"journal-article","created":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T05:08:14Z","timestamp":1634706494000},"source":"Crossref","is-referenced-by-count":1,"title":["A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient"],"prefix":"10.12890","volume":"8","author":[{"given":"Lu\u00eds","family":"Landeiro","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Hospital da Luz, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Carolina","family":"Freitas","sequence":"additional","affiliation":[{"name":"Hematology Department, Instituto Portugu\u00eas de Oncologia Francisco Gentil, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margarida","family":"Proen\u00e7a","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Hospital da Luz, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9","family":"Cabe\u00e7adas","sequence":"additional","affiliation":[{"name":"Anatomic Pathology Department, Instituto Portugu\u00eas de Oncologia Francisco Gentil, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Albertina","family":"Nunes","sequence":"additional","affiliation":[{"name":"Hematology Department, Instituto Portugu\u00eas de Oncologia Francisco Gentil, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandra","family":"Bay\u00e3o Horta","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Hospital da Luz, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2021,10,20]]},"reference":[{"key":"10.12890\/2021_002831_ref1","doi-asserted-by":"crossref","unstructured":"<li>Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman\u2019s disease: a systematic literature review. <em>Lancet Haematol <\/em>2016;<strong>3<\/strong>(4):e163\u2013e175. <\/li>","DOI":"10.1016\/S2352-3026(16)00006-5"},{"key":"10.12890\/2021_002831_ref2","doi-asserted-by":"crossref","unstructured":"<li>Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative\/idiopathic multicentric Castleman disease. <em>Blood<\/em> 2017;<strong>129<\/strong>:1646\u20131657. <\/li>","DOI":"10.1182\/blood-2016-10-746933"},{"key":"10.12890\/2021_002831_ref3","doi-asserted-by":"crossref","unstructured":"<li>Naddaf E, DIspenzieri A, Mandrekar J, Mauermann ML. Clinical spectrum of Castleman disease-associated neuropathy. <em>Neurology<\/em> 2016;<strong>87<\/strong>(23):2457\u20132462. <\/li>","DOI":"10.1212\/WNL.0000000000003405"},{"key":"10.12890\/2021_002831_ref4","doi-asserted-by":"crossref","unstructured":"<li>El Karoui K, Vuiblet V, Dion D, Izzedine H, Guitard J, Frimat L, et al. Renal involvement in Castleman disease. <em>Nephrol Dial Transplant<\/em> 2011;<strong>26<\/strong>(2):599\u2013609. <\/li>","DOI":"10.1093\/ndt\/gfq427"},{"key":"10.12890\/2021_002831_ref5","doi-asserted-by":"crossref","unstructured":"<li>Chan KL, Lade S, Prince HM, Harrison SJ. Update and new approaches in the treatment of Castleman disease. <em>J Blood Med<\/em> 2016;<strong>7<\/strong>:145\u2013158. <\/li>","DOI":"10.2147\/JBM.S60514"},{"key":"10.12890\/2021_002831_ref6","doi-asserted-by":"crossref","unstructured":"<li>Dispenzieri A. POEMS syndrome: 2017 update on diagnosis, risk stratification, and management. <em>Am J Hematol<\/em> 2017;<strong>92<\/strong>(8):814\u2013829. <\/li>","DOI":"10.1002\/ajh.24802"},{"key":"10.12890\/2021_002831_ref7","doi-asserted-by":"crossref","unstructured":"<li>Sugimoto T, Ito J, Takeda N, Gasyu I, Okazaki T, Sakaguchi M, et al. A case of Castleman\u2019s disease complicated with nephrotic syndrome due to glomerulopathy mimicking membranoproliferative glomerulonephritis. <em>Am J Med Sci<\/em> 2008;<strong>335<\/strong>(6):495\u2013498. <\/li>","DOI":"10.1097\/MAJ.0b013e3181571f7e"},{"key":"10.12890\/2021_002831_ref8","unstructured":"<li>Tazi I, Rachid M, Quessar A, Benchekroun S. A rare association of Castleman\u2019s disease and nephrotic syndrome. <em>Saudi J Kidney Dis Transpl<\/em> 2011;<strong>22<\/strong>(1):116\u2013119. <\/li>"},{"key":"10.12890\/2021_002831_ref9","doi-asserted-by":"crossref","unstructured":"<li>Mauermann ML. The peripheral neuropathies of POEMS syndrome and Castleman disease. <em>Hematol Oncol Clin North Am<\/em> 2018;<strong>32<\/strong>(1):153\u2013163. <\/li>","DOI":"10.1016\/j.hoc.2017.09.012"},{"key":"10.12890\/2021_002831_ref10","doi-asserted-by":"crossref","unstructured":"<li>Ide M, Kawachi Y, Izumi Y, Kasagi K, Ogino T. Long-term remission in HIV-negative patients with multicentric Castleman\u2019s disease using rituximab. <em>Eur J Haematol <\/em>2006;76:119\u2013123.<\/li>","DOI":"10.1111\/j.1600-0609.2005.00570.x"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2831\/2858","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2831\/2859","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2831\/2859","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,22]],"date-time":"2025-08-22T12:25:12Z","timestamp":1755865512000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/2831"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,20]]},"references-count":10,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2021]]}},"URL":"https:\/\/doi.org\/10.12890\/2021_002831","relation":{"has-manifestation":[{"id-type":"other","id":"1-78-2831-g2858","asserted-by":"subject"},{"id-type":"other","id":"1-78-2831-g2859","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-78","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"type":"electronic","value":"2284-2594"}],"subject":[],"published":{"date-parts":[[2021,10,20]]},"article-number":"6"}}