Abstract Purpose of review (Levamisole adulterated) cocaine can cause a number of symptoms. One of the most severe is cocaine-induced vasculitis, which is hard to both diagnose and treat. We conducted a review to summarize the most recent findings on symptomatology, treatment options, anti-neutrophil cytoplasmic antibodies (ANCA) positivity and pathophysiology. Recent findings In the past years multiple large cohort studies have been published extensively describing the symptomology and rates of ANCA positivity in patients with (levamisole-adulterated) cocaine-induced vasculitis. These studies also give more insight into the effects of different treatment strategies. Summary The mainstay of treatment is abstinence of cocaine supported by antibiotics in case of concomitant infections and/or immunosuppressive medication depending on symptoms. ANCA positivity is a hallmark of more extensive disease and is a characteristic of immune system activation. In cocaine-induced vasculitis, dual positivity for both proteinase 3(PR3)- and myeloperoxidase (MPO)-ANCA is described and some patients are found to have human elastase type (HNE-)ANCA. HNE-ANCA positivity varies in patients with cocaine-induced midline destruction (CIMDL) from 28-84%, but has not been researched thoroughly in patients with cocaine-induced vasculitis. We present our hypothesis of a “sliding-scale” by which CIMDL turns into cocaine-induced systemic vasculitis based upon recent literature and we describe the mechanisms by which cocaine-induced vasculitis develops.