Kaposi sarcoma in anti-neutrophil cytoplasmic antibody-associated vasculitis: a case-based review - PubMed (original) (raw)

Review

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

Benedict K Tiong et al. Rheumatol Int. 2021 Jul.

Abstract

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.

Keywords: Anti-neutrophil cytoplasmic antibody-associated vasculitis; Granulomatosis with polyangiitis; Kaposi sarcoma; Microscopic polyangiitis; Prednisone; Vasculitis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no conflicts of interest/competing interests.

Figures

Fig. 1

Fig. 1

a Multiple violaceous, coalescent, nodular lesions on the foot and ankle. b Histologic sections of skin from biopsy of a thigh lesion show dermis filled with irregular, somewhat jagged blood-filled vascular spaces adhering to collagen bundles and surrounding native blood vessels (so-called ‘promontory sign’, see arrows). Hematoxylin and eosin, 200x. c Performed CD34 immunohistochemistry strongly highlights irregular, infiltrative vascular spaces. CD34 immunohistochemistry, 200x. d Performed HHV8 immunohistochemistry highlights tumor endothelial cell nuclei and confirms the diagnosis of Kaposi’s sarcoma. HHV8 immunohistochemistry, 200x

References

    1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11. doi: 10.1002/art.37715. -DOI -PubMed
    1. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117:39–50. doi: 10.1016/j.amjmed.2004.02.030. -DOI -PubMed
    1. Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369:417–427. doi: 10.1056/nejmoa1213277. -DOI -PMC -PubMed
    1. Vangipuram R, Tyring SK. Epidemiology of Kaposi sarcoma: review and description of the nonepidemic variant. Int J Dermatol. 2019;58:538–542. doi: 10.1111/ijd.14080. -DOI -PubMed
    1. Antman K, Chang Y. Kaposi’s sarcoma. N Engl J Med. 2000;342:1027–1038. doi: 10.1056/NEJM200004063421407. -DOI -PubMed

Publication types

MeSH terms

Substances

LinkOut - more resources