Kaposi sarcoma in anti-neutrophil cytoplasmic antibody-associated vasculitis: a case-based review

• Published in: Rheumatology international Vol. 41; no. 7; pp. 1357 - 1367
• Main Authors: Tiong, Benedict K., Singh, Arun S., Sarantopoulos, G. Peter, Kermani, Tanaz A.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.07.2021; Springer Nature B.V
• Subjects: Aged; Case Based Review; Cyclophosphamide - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Kaposis sarcoma; Male; Medicine; Medicine & Public Health; Microscopic Polyangiitis - complications; Microscopic Polyangiitis - drug therapy; Microscopic Polyangiitis - pathology; Neutrophils; Prednisone - therapeutic use; Remission Induction; Rheumatology; Sarcoma, Kaposi - complications; Sarcoma, Kaposi - pathology; Granulomatosis with polyangiitis; Kaposi sarcoma; Microscopic polyangiitis; Prednisone; Vasculitis; Anti-neutrophil cytoplasmic antibody-associated vasculitis
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-021-04810-w

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.