{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T22:03:33Z","timestamp":1792015413988,"version":"4.4.0"},"reference-count":16,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,11,29]],"date-time":"2023-11-29T00:00:00Z","timestamp":1701216000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To describe a 41\u2010year\u2010old woman with a history of neonatal onset multisystem inflammatory disease, on treatment with daily subcutaneous injections of 600 mg of recombinant interleukin\u20101 receptor antagonist (IL\u20101Ra) protein, anakinra, since the age of 28, who presented with golf\u2010ball size nodules at the anakinra injection sites, early satiety, new onset nephrotic syndrome in the context of normal markers of systemic inflammation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical history and histologic evaluation of biopsies of skin, gastric mucosa, and kidney with Congo\u2010red staining and proteomic evaluation of microdissected Congo red\u2013positive amyloid deposits by liquid chromatography\u2010tandem mass spectrometry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The skin, stomach, and kidney biopsies all showed the presence of Congo red\u2013positive amyloid deposits. Mass spectrometry\u2010based proteomics demonstrated that the amyloid deposits in all sites were of AIL1RAP (IL\u20101Ra protein)\u2010type. These were characterized by high spectral counts of the amyloid signature proteins (apolipoprotein AIV, apolipoprotein E, and serum amyloid P\u2010component) and the amyloidogenic IL\u20101Ra protein, which were present in Congo red\u2013positive areas and absent in Congo red\u2013negative areas. The amino acid sequence identified by mass spectrometry confirmed that the amyloid precursor protein was recombinant IL\u20101Ra (anakinra) and not endogenous wild\u2010type IL\u20101Ra.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the first report of iatrogenic systemic amyloidosis due to an injectable protein drug, which was caused by recombinant IL1Ra (anakinra).<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42664","type":"journal-article","created":{"date-parts":[[2023,7,25]],"date-time":"2023-07-25T01:45:55Z","timestamp":1690249555000},"page":"100-106","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Anakinra\u2010Associated Systemic Amyloidosis"],"prefix":"10.1002","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6531-6108","authenticated-orcid":false,"given":"Sara","family":"Alehashemi","sequence":"first","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surendra","family":"Dasari","sequence":"additional","affiliation":[{"name":"Mayo Clinic  Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anvitha","family":"Metpally","sequence":"additional","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kat","family":"Uss","sequence":"additional","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie A.","family":"Castelo\u2010Soccio","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theo","family":"Heller","sequence":"additional","affiliation":[{"name":"National Institute of Diabetes and Digestive and Kidney Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Kellman","sequence":"additional","affiliation":[{"name":"National Heart, Lung, and Blood Institute, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus Y.","family":"Chen","sequence":"additional","affiliation":[{"name":"National Heart, Lung, and Blood Institute, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Ahlman","sequence":"additional","affiliation":[{"name":"Clinical Center, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeff","family":"Kim","sequence":"additional","affiliation":[{"name":"National Institute on Deafness and Other 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