We compared the clinical, serologic, radiographic, and histopathologic features of 18 consecutive patients who presented with cocaine-induced midline destructive lesions (CIMDL) with those of all 21 patients with Wegener granulomatosis (WG) with nasal involvement evaluated during the same time period. Routine ANCA tests were positive in 13 of 18 CIMDL patients compared with 19 of 21 WG patients. Clinical and radiographic evaluation revealed that destruction of facial midline structures was significantly more severe in CIMDL than WG. In contrast to WG, there was no other organ involvement and no significant laboratory abnormalities indicating systemic inflammation in CIMDL. Histopathologic evaluation revealed the frequent occurrence of nonspecific inflammation, necrosis, and vascular abnormalities such as leukocytoclastic vasculitis and perivenulitis in CIMDL as well as in WG. Only extravascular microabscesses, necrotizing granulomas, and multinucleated giant cells found in WG were discriminatory features. More detailed analysis of the ANCA found in CIMDL and WG patients showed the following differences. Of 8 P-ANCA-positive CIMDL sera, none reacted with MPO, 4 reacted with PR3, 3 reacted with HLE, 2 of which showed double-reactivity with PR3 and HLE. All of 5 C-ANCA-positive CIMDL patients showed reactivity with PR3. Two of these also reacted with HLE. In contrast, all but 1 of the 19 ANCA-positive WG patients displayed concurrent P-/MPO-ANCA or C-/PR3-ANCA reactivity, respectively. In 1 WG patient the target antigen reactivity was reversed. None of the WG patients displayed double-reactivity. Consequently, routine ANCA testing does not allow an unequivocal distinction between CIMDL and nasal involvement of WG, but more detailed investigations suggest instructive differences between the ANCA immune responses of the 2 patient populations.
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