Table 2.

Summary of literature review of ANCA-associated vasculitis with Kaposi sarcoma

Author	Age, years/sex	Diagnosis	AAV Organ involvement	ANCA type	Immunosuppression	Time to onset of KS	Areas affected by KS	HIV and HHV-8 status	Treatment	Outcome
Our case	77/M	Drug-associated MPA	Pulmonary-renal syndrome	p-ANCA, MPO	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	6 Weeks	Skin lesions on upper and lower extremities	HIV negative, HHV-8 positive	Withdrawal of cyclophosphamide, lower prednisone, imiquimod topical	Regression KS, vasculitis in remission
Fatma et al.[20]	72/F	MPA	Pulmonary-renal syndrome	Positive p-ANCA, MPO	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	5 Months	Skin lesions on trunk, lower extremities, face, neck	HIV negative, HHV-8 positive	Withdrawal of immunosuppression	Regression KS, relapse vasculitis with alveolar hemorrhage
Biricik et al.[27]	71/M	MPA	Pulmonary-renal syndrome	p-ANCA positive; MPO/PR3 not tested	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	3 Months	Skin lesions on lower extremities	HIV status not provided, HHV-8 positive	Decrease glucocorticoid dose, cyclophosphamide discontinued, radiation therapy	Regression KS, vasculitis status not provided
Erban and Sokas[21]	78/M	GPA	Pulmonary-renal syndrome, chronic sinusitis, arthralgia	Not tested	Glucocorticoids (oral methylprednisolone), oral cyclophosphamide	10 Weeks	Skin lesions on trunk, upper and lower extremities	HIV negative, HHV-8 status not provided	Glucocorticoid discontinued, cyclophosphamide continued, proton beam radiation to the feet	Regression KS, death from cardiogenic shock during cardiac bypass procedure
Deschenes et al.[22]	54/M	GPA	Sinusitis, cavitary pulmonary lesions	c-ANCA, PR3	Glucocorticoids (IV then oral prednisone), oral cyclophosphamide	8 Weeks	Skin lesions on trunk, upper and lower extremities	HIV negative, HHV-8 status not provided	Glucocorticoids tapered off, cyclophosphamide reduced then discontinued after 20 months	Regression KS, vasculitis in remission
Hoff and Rødevand[19]	46/M	GPA	Cranial neuropathies, sinusitis, arthritis, lung nodules	Negative c-ANCA, p-ANCA MPO/PR3 not tested	Glucocorticoids, IV cyclophosphamide (stopped due adverse effects), methotrexate	~ 19 Years	Skin lesion on ear	HIV and HHV-8 status not provided	None	Died of bladder cancer, vasculitis improved
Bouattar et al.[23]	50/F	GPA	Glomerulonephritis, L nasal ulceration	c-ANCA positive; MPO and PR3 not tested	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	18 Weeks	Skin lesions on trunk, upper and lower extremities	HIV negative, HHV-8 positive	Discontinuation of cyclophosphamide, decrease glucocorticoid dose	Regression KS followed by recurrence, worsening renal function requiring dialysis, death from DIC
Saxena et al.[25]	66/F	GPA	Not provided	Not provided	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	5 Months	Skin lesions on trunk and upper and lower extremities	HIV status not provided, HHV-8 positive	Cyclophosphamide continued for another month then switched to azathioprine, prednisone gradually tapered, azathioprine stopped for worsening KS, IV doxorubicin	Regression KS, vasculitis in remission
Kılıç et al. [26]	70/F	GPA	Nasal septal perforation, glomerulonephritis, pulmonary nodules	c-ANCA positive; MPO/PR3 not tested	Glucocorticoids (IV followed by oral prednisone), IV cyclophosphamide	0 (Present at diagnosis but worse at 12 weeks)	Skin lesions on left lower extremity	HIV negative, HHV-8 negative	Glucocorticoids decreased, cyclophosphamide discontinued, radiation therapy, systemic chemotherapy (treatment not specified)	Not provided
Endo and Nagata[28]	73/M	GPA	Not provided	Not provided	Glucocorticoids (IV followed by oral prednisolone), IV cyclophosphamide 4 cycles) then azathioprine	11 Months	Gastrointestinal ulcerations (upper and lower tract)	HIV negative, HHV-8 positive	Corticosteroids tapered from 11 mg per day to 6 mg per day, azathioprine continued	Ulcerations and lesions improved, vasculitis in remission
Berti et al. [24]	67/M	EGPA	Glomerulonephritis, sinusitis, asthma, nasal polyposis	Not provided	Glucocorticoids (oral), mycophenolate mofetil	Not provided	Cutaneous	HIV negative, HHV-8 positive	Mycophenolate mofetil was discontinued, prednisone continued (5 mg per day)	Regression KS, vasculitis in remission

AAV anti-neutrophil cytoplasmic antibody-associated vasculitis, ANCA anti-neutrophil cytoplasmic antibody, EGPA eosinophilic granulomatosis with polyangiitis, 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