Objectives Cocaine and cocaine mixed with levamisole are increasingly used in the UK and result in significant direct nasal damage in addition to promoting vasculitis. Our aims were as follows: (1) to identify the main symptoms and presentation of cocaine-induced vasculitis; (2) to provide evidence regarding the best practice for the investigation and diagnosis of cocaine-induced vasculitis; and (3) to analyse the clinical outcomes of patients in order to understand the optimal management for the condition. Methods We performed a retrospective case series analysis of patients presenting with cocaine-induced midline destructive lesions or vasculitis compatible with granulomatosis with polyangiitis (GPA) from two large tertiary vasculitis clinics between 2016 and 2021. Results Forty-two patients (29 Birmingham, 13 London) with cocaine-induced midline lesions or systemic disease were identified. The median age was 41 years (range 23–66 years). Current cocaine use was common, and 20 of 23 samples provided were positive when routine urine toxicology was performed; 9 patients who denied ever using cocaine were identified as using cocaine based on urine toxicology analysis, and 11 who stated they were ex-users still tested positive. There was a high incidence of septal perforation (75%) and oronasal fistula (15%). Systemic manifestations were less common (27%), and only one patient had acute kidney injury. Fifty-six per cent of our patients were PR3-ANCA positive, with none testing positive for MPO-ANCA. Symptom remission required cocaine discontinuation even when immunosuppression was administered. Conclusion Patients with destructive nasal lesions, especially young patients, should have urine toxicology performed for cocaine before diagnosing GPA and considering immunosuppressive therapy. The ANCA pattern is not specific for cocaine-induced midline destructive lesions. Treatment should be focused on cocaine cessation and conservative management in the first instance in the absence of organ-threatening disease.
Aim Extramedullary plasmacytoma are rare neoplasms, which mainly occur in the head and neck, the most common site being the nasal cavity or septum. Patients may present with epistaxis, nasal obstruction, or rhinorrhoea. Extension into the orbit is rare but can lead to visual disturbances. Method This case report pertains to a 69-year-old female patient who initially presented with a 6-week history of recurrent sinusitis, with left sided cheek pain and diplopia. Results A CT and MRI scan was performed, which showed opacification of the left maxillary, ethmoid and sphenoid sinuses with bony destruction of the medial and anterior maxillary walls and into the anterior soft tissues. The mass was completely obstructing the left nasal cavity extending into the inferomedial orbit. Staging CT scans also showed nodal disease in the neck. Biopsy with histology was positive for CD138, with diffuse proliferation of plasma cells of varying maturity and atypia. Additionally, mono IgG kappa was also positive as was CD45 suggestive of a lymphoid neoplasm. Bone marrow biopsy and PET scan confirmed there was no systemic involvement. Radiotherapy was initiated after the patient recovered from COVID -19. A repeat MRI scan 6 weeks post radiotherapy showed a partial response with reduction in the size of the tumour, resolution of the intra-orbital extension and disappearance of paraprotein. Conclusions This case report illustrates need for earlier consideration of EMP diagnostically to reduce the risk of conversion of the plasmacytoma to multiple myeloma, which is a known possibility and to ensure minimal delay in the commencement of treatment.
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