Background Cocaine-induced vasculitis is an increasingly recognized syndrome that can present with a number of manifestations. This mimic of idiopathic vasculitis has become more common secondary to an increase in the use of cocaine that has been mixed or cut with the adulterant levamisole. Prior case reports have outlined the difficulty in distinguishing this syndrome from similar rheumatologic disorders.
Objectives In this case series, we describe the organ involvement, clinical manifestations, and laboratory findings of five patients with cocaine-induced vasculitis in order to further the ability to diagnose this disorder.
Methods Information was collected regarding each of the following factors: demographics, past medical history, organ involvement, laboratory data, radiologic and pathologic findings, treatment, and eventual outcome pending discharge.
Results There were several commonalities among the five patients. The subjects ranged in age from 37 to 46 years. All tested positive for cocaine by urine drug screen. All patients demonstrated oral or nasal involvement including ulcers and epistaxis with four of the five additionally showing skin ulcerations and/or nonspecific skin rash. Three out of five patients had kidney involvement. Of the three biopsies performed, a skin biopsy did not reveal vasculitis, but two kidney biopsies showed pauci-immune glomerulonephritis. All patients were ANCA positive, but there was no consistent pattern amongst the group. Two patients were cANCA positive, two were pANCA positive, and one was atypical pANCA positive. Only one patient with cANCA had concurrent PR3-ANCA positivity. One patient with p-ANCA also had PR3-ANCA positivity. Two patients with pANCA positivity and one patient with atypical pANCA positivity were MPO-ANCA positive.
Of note, one patient had a remote diagnosis of polyarteritis nodosa seven years prior to his admission and was in remission. He presented with new onset sinus disease; his biopsy was positive for granulomatosis with polyangiitis. One patient had scleritis. One patient developed ground glass opacities on her CT chest on readmission. One patient had mononeuritis multiplex.
Treatment regimens varied among the patients. One patient improved with prednisone and skin grafts, while a second patient with scleritis and epistaxis improved with prednisone alone. Three of the five patients demonstrated no further progression when treated with prednisone or methylprednisolone doses of 30-60 mg/day. One patient was initially given heparin for an initial diagnosis of antiphospholipid antibody syndrome. One patient was discharged to hospice due to severe renal involvement with skin ulcerations over 80 percent of her body. Two patients were readmitted within one year for further complications of the cocaine-induced vasculitis.
Conclusions Cocaine-induced vasculitis has varied presentations and clinical manifestations, however, the majority of our patients had skin and renal manifestations. All patients had positive ANCA testing, but patterns were wide-ranging. The patients appeared to respond favorably when treated with corticosteroids. Further studies are required to increase recognition of this disorder and to determine the optimal management.
Disclosure of Interest None Declared