Table 3.

Literature review of Kaposi Sarcoma (KS) in other forms of systemic vasculitis

Author	Age, years/Sex	Diagnosis	Immunosuppression	Time to onset of KS	Areas affected by KS	HIV and HHV8 status	Treatment	Outcome
Klepp et al. [17]	79/F	Polymyalgia rheumatica	Glucocorticoids (oral prednisone)	7 Months	Skin lesions lower extremities and eyelid	HIV status not provided, HHV-8 status not provided	Radiotherapy	Regression of KS, patient died suddenly of unknown cause
Vincent et al. [33]	84/F	Polymyalgia rheumatica	Glucocorticoids (oral prednisone)	4 Months	Skin lesions on lower extremities	HIV status not provided, HHV-8 positive	Not provided	Not provided
Brambilla et al. [37]	72/F	Polymyalgia rheumatica	Glucocorticoids (oral prednisone)	4 Years (was on 4 mg daily for 4 years)	Skin lesions on trunk, upper and lower extremities, leg lymphedema	HIV negative, HHV-8 positive	Gradually discontinue prednisone, taxol	Partial regression of KS, developed Merkel cell carcinoma requiring additional treatment
Leung et al. [29]	70/F	Giant cell arteritis	Glucocorticoids (oral prednisone)	5 Months	Skin lesions upper and lower extremities, neck, lips, back	HIV status not provided, HHV-8 status not provided	Decrease in prednisone doses	Regression of KS, no flares of giant cell arteritis
Di Giacomo et al. [30]	69/M	Giant cell arteritis	Glucocorticoids (oral prednisone)	3 Months	Skin lesions lower extremities	HIV status not provided, HHV-8 status not provided	Decrease in prednisone, change to methyl-fluoro-prednisolone	Status of KS not available, flare of giant cell arteritis
Soria et al. [32]	45/F	Giant cell arteritis	Glucocorticoids (oral prednisone)	3 Years	Skin lesions upper and lower extremities, face, trunk	HIV status not provided, HHV-8 status not provided	Decrease in prednisone, vincristine, radiation therapy	Regression of KS, status of giant cell arteritis not provided
Kuttikat et al. [35]	79/F	Giant cell arteritis	Glucocorticoids (oral prednisolone)	6 Weeks	Skin lesions on trunk, lower extremities	HIV negative, HHV-8 positive	Taper of prednisone with discontinuation	Resolution of KS, no flares of giant cell arteritis
Kotter et al. [34]	29/M	Behcet’s disease	Glucocorticoids (oral prednisolone), cyclosporine A, azathioprine	3 Years	Skin, gastric mucosa, hard palate, pulmonary	HIV negative, HHV-8 positive	Discontinuation of azathioprine and cyclosporine A, taper prednisolone	Ocular disease flared requiring treatment with interferon alpha, both diseases in remission
Mezalek et al. [36]	44/M	Behcet’s disease	Glucocorticoids (IV followed by oral prednisolone), IV cyclophosphamide × 6 then azathioprine	10 Months	Skin lesions on lower extremities	HIV negative, HHV-8 positive	Discontinuation of azathioprine, decrease glucocorticoid dose	Ocular disease flared requiring treatment with interferon alpha, both diseases in remission
Schulhafer et al. [31]	61/M	IgA vasculitis	Glucocorticoids (intravenous prednisolone, oral prednisone), chlorpropamide	6 Months	Skin lesions trunk	HIV status not provided, HHV-8 status not provided	Decrease in prednisone	Regression of KS, IgA vasculitis flared requiring repeat prednisone treatment followed by discontinuation
Vincent et al. [33]	79/F	Leukocytoclastic vasculitis	Glucocorticoids (oral prednisone)	3 Months	Skin lesions on trunk, upper and lower extremities	HIV status not provided, HHV-8 positive	Not provided	Not provided

F Female, GPA granulomatosis with polyangiitis, HHV-8 human herpesvirus 8, HIV human immunodeficiency virus, IV intravenous, KS Kaposi sarcoma, M male, MPA microscopic polyangiitis, MPO myeloperoxidase, PR3 Proteinase 3