Table 2.
Differentials in clinical, laboratory, histological and treatment characteristics of CIMDL, LAC vasculopathy/vasculitis and cocaine-induced vasculitis.
| CIMDLs | LAC Vasculopathy/Vasculitis | CIV | |
|---|---|---|---|
|
Disease
localizations |
ENT (midline structures) | Local (cutaneous) and systemic | Multi-organ and systemic |
| Disease manifestations | Diffuse necrotizing ulcerative lesions, nasal crusting, nasal-septum perforation, palatal perforation, nasal deformity | Retiform purpura, constitutional symptoms, midline destructive lesions, glomerulonephritis, pulmonary involvement, leukoencephalopathy, arthritis |
Skin rashes, joint involvement, pauci-immune crescentic glomerulonephritis, alveolar hemorrhage |
| ANCA IIF pattern | Perinuclear or cytoplasmatic | Atypical perinuclear | Perinuclear |
| ANCA specificity | Anti-HNE, PR3-ANCA, ANCA negative | MPO-ANCA, PR3-ANCA, anti-HNE | PR3-ANCA, MPO-ANCA, ANCA negative |
|
ANCA
double positivity |
HNE and PR3 | c-ANCA and p-ANCA | PR3 and p-ANCA |
| Other biomarkers | - | APL, low complement levels, ANAs, anti-dsDNA, anti-C1q, cryoglobulin | - |
|
Systemic
inflammation |
Rarely | Common | Common |
|
Hematological
abnormalities |
None | Common (leukopenia, neutropenia, agranulocytosis, hemolytic anemia, thrombocytopenia) | - |
| Histology | Not specific except for cell apoptosis and extensive necrosis |
Thrombotic vasculopathy, leukocytoclastic vasculitis |
Leukocytoclastic vasculitis, commonly pauci-immune necrotizing glomerulonephritis |
| First approach | Cessation of cocaine abuse |
Cessation of cocaine abuse |
Cessation of cocaine abuse |
| Immunosuppressive treatment | Rarely required, only in case of systemic inflammation |
Required in case of severe systemic inflammation or life-threatening symptoms |
Controversial |
| Local and surgery treatment | Conservative local treatment, prosthetic and reconstructive surgery | Supportive local therapy and plastic surgery | - |
ANAs: anti-nuclear antibodies; ANCA: anti-neutrophil cytoplasmatic antibody; anti-dsDNA: anti-double-stranded DNA antibody; APL: antiphospholipid antibody; c-ANCA: cytoplasmatic-ANCA; CIMDLs: cocaine-induced midline destructive lesions; CIV: cocaine-induced vasculitis; ENT: ear, nose and throat; HNE: human neutrophil elastase; IIF: indirect immunofluorescence; LAC: levamisole-adulterated cocaine; MPO: myeloperoxidase; p-ANCA: perinuclear-ANCA; PR3: proteinase 3.