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

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.

Unveiling the Causal Link of herpes virus infection and Cutaneous Leukocytoclastic Angiitis: Insights from Mendelian Randomization

“…Various studies have explored the connection between the herpesvirus family and different types of angiitis. For example, EBV may be associated with anti-neutrophil cytoplasmic antibody-associated angiitis (AAV), Henoch-Schönlein purpura (HSP) and systemic vasculitis, varicella zoster virus causes giant cell arteritis, Acute Retinal Necrosis (ARN) and lgA angiitis, HSV infection is linked to cerebrovascular disease, and CMV infection can cause skin necrotizing angiitis (4,6,(27)(28)(29)(30)(31) . However, our MR analysis did not find a causal relationship between these viruses and CLA.…”

Systemic lupus erythematosus and antineutrocytic cytoplasmic antibody-associated vasculitis overlap syndrome presenting mainly with alveolar hemorrhage: A case report and literature review

“…[2] Anti-neutrophil cytoplasmic antibody (ANCA)associated vasculitis (AAV) includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (Wegener), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and its typical serological sign is that ANCAs can be detected. [3] Serious organ-threatening disease involvement with rapidly progressive glomerulonephritis (GN), diffuse alveolar…”

“…[ 2 ] Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (Wegener), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and its typical serological sign is that ANCAs can be detected. [ 3 ] Serious organ-threatening disease involvement with rapidly progressive glomerulonephritis (GN), diffuse alveolar hemorrhage (DAH) but also gastrointestinal. [ 4 ] Generally, SLE and AAV are 2 different diseases, but a case was reported that a patient can have SLE and AAV at the same time, which called SLE/AAV overlap syndrome.…”