PMID- 33620515 OWN - NLM STAT- MEDLINE DCOM- 20220111 LR - 20220111 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 41 IP - 7 DP - 2021 Jul TI - Kaposi sarcoma in anti-neutrophil cytoplasmic antibody-associated vasculitis: a case-based review. PG - 1357-1367 LID - 10.1007/s00296-021-04810-w [doi] AB - Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are systemic necrotizing vasculitides associated with significant morbidity and mortality. Given the immunosuppression used to manage these conditions, it is important for clinicians to recognize complications, especially infectious ones, which may arise during treatment. Kaposi sarcoma (KS) is a lymphoangioproliferative neoplasm caused by human herpes virus 8 (HHV-8). Its cutaneous manifestations can mimic vasculitis. We describe a 77-year-old man with microscopic polyangiitis with pulmonary-renal syndrome treated with prednisone and intravenous cyclophosphamide who developed KS (HHV-8 positive) after 2 months of treatment. Cyclophosphamide was discontinued and prednisone gradually lowered with improvement and clinical stabilization of KS lesions. This comprehensive review includes all published cases of KS in patients with AAV, with a goal to summarize potential risk factors including the clinical characteristics of vasculitis, treatment and outcomes of patients with this rare complication of immunosuppressive therapy. We also expanded our literature review to KS in other forms of systemic vasculitis. Our case-based review emphasizes the importance of considering infectious complications of immunosuppressive therapy, especially glucocorticoids, and highlights the rare association of KS in systemic vasculitis. FAU - Tiong, Benedict K AU - Tiong BK AUID- ORCID: 0000-0001-5556-3335 AD - Division of Rheumatology, David Geffen School of Medicine, University of California, Los Angeles, 2020 Santa Monica Boulevard, Suite 540, Santa Monica, CA, 90404, USA. BTiong@mednet.ucla.edu. FAU - Singh, Arun S AU - Singh AS AD - Division of Hematology and Oncology, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA. FAU - Sarantopoulos, G Peter AU - Sarantopoulos GP AD - Department of Pathology and Laboratory Medicine, UCLA Medical Center, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA. FAU - Kermani, Tanaz A AU - Kermani TA AUID- ORCID: 0000-0002-7335-7321 AD - Division of Rheumatology, David Geffen School of Medicine, University of California, Los Angeles, 2020 Santa Monica Boulevard, Suite 540, Santa Monica, CA, 90404, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210223 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - VB0R961HZT (Prednisone) SB - IM MH - Aged MH - Cyclophosphamide/*therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Microscopic Polyangiitis/*complications/drug therapy/pathology MH - Prednisone/*therapeutic use MH - Remission Induction MH - Sarcoma, Kaposi/*complications/pathology PMC - PMC8164621 OTO - NOTNLM OT - Anti-neutrophil cytoplasmic antibody-associated vasculitis OT - Granulomatosis with polyangiitis OT - Kaposi sarcoma OT - Microscopic polyangiitis OT - Prednisone OT - Vasculitis COIS- The authors declare that they have no conflicts of interest/competing interests. EDAT- 2021/02/24 06:00 MHDA- 2022/01/12 06:00 PMCR- 2021/02/23 CRDT- 2021/02/23 12:15 PHST- 2020/12/08 00:00 [received] PHST- 2021/02/06 00:00 [accepted] PHST- 2021/02/24 06:00 [pubmed] PHST- 2022/01/12 06:00 [medline] PHST- 2021/02/23 12:15 [entrez] PHST- 2021/02/23 00:00 [pmc-release] AID - 10.1007/s00296-021-04810-w [pii] AID - 4810 [pii] AID - 10.1007/s00296-021-04810-w [doi] PST - ppublish SO - Rheumatol Int. 2021 Jul;41(7):1357-1367. doi: 10.1007/s00296-021-04810-w. Epub 2021 Feb 23.