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Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed immunosuppressive treatments. This is particularly challenging when organ dysfunction, i.e., end-stage kidney failure, is present. In this case report, we present the unique case of a 43-year-old female patient with rapidly progressive diffuse systemic sclerosis who underwent aHSCT despite end-stage renal failure as consequence of SSc-renal crisis. Therefore, conditioning chemotherapy was performed with melphalan instead of cyclophosphamide with no occurrence of severe adverse events during the aplastic period and thereafter. After aHSCT, early disease progression of the skin occurred and was successfully treated with secukinumab. Thereby, to the best of our knowledge, we report the first case of successful aHSCT in a SSc-patient with end-stage kidney failure and also the first successful use of an IL-17 inhibitor to treat early disease progression after aHSCT., Competing Interests: PPS received speaker’s fees and travel grants from Janssen-Cilag Galapagos, Eli Lilly, Boehringer/Ingelheim and AbbVie (less than $10,000 each) as well as research funding from Chugai (25000$). HL received travel grants from UCB, Boehringer Ingelheim, Pfizer, and Abbvie as well as compensations for consulting activity from Pfizer and for lecturing activities from Janssen. LKN received travel grants from Abbvie, UCB and Medac. JP received travel grants from CSL Behring, Galapagos, AbbVie. MF received speaker’s fees, travel grants or compensation for board memberships from AbbVie, Novartis, Janssen, and Eli Lilly. MG received travel grants, compensation for advisory boards or speaker’s fees from AbbVie, Chugai, Eli Lilly, Hexal, Janssen, Novartis, Pfizer, Takeda. MS received speaker’s fees, travel grants, research funding, or compensation for consultancies or board memberships from AbbVie, Actelion, AstraZeneca, BMS, Boehringer/Ingelheim, Celgene, Chugai/Roche, Eli Lilly, Genzyme, Gilead, Hexal/Sandoz, Janssen-Cilag, MSD, Novartis, Pfizer, Sanofi Pasteur, Takeda (Shire), UCB (less than $ 10,000 each)., (Copyright © 2023 Strunz, Labinsky, Nagler, Portegys, Froehlich, Gernert and Schmalzing.)