Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition

Charn Gill; Joseph Sturman; Leyla Ozbek; Scott R Henderson; Aine Burns; Sally Hamour; Ruth J Pepper; Lisha McClelland; Dimitrios Chanouzas; Simon Gane; Alan D Salama; Lorraine Harper

doi:10.1093/rap/rkad027

Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition

Charn Gill, Joseph Sturman, Leyla Ozbek, Scott R Henderson, Aine Burns, Sally Hamour, Ruth J Pepper, Lisha McClelland, Dimitrios Chanouzas, Simon Gane, Alan D Salama, Lorraine Harper^*

^*Corresponding author for this work

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Abstract

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.

Original language	English
Article number	rkad027
Journal	Rheumatology Advances in Practice
Volume	7
Issue number	1
DOIs	https://doi.org/10.1093/rap/rkad027
Publication status	Published - 4 Apr 2023

Bibliographical note

Keywords

cocaine
drug-induced vasculitis
matosis with polyangiitis
ANCA
treatment

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10.1093/rap/rkad027Licence: Creative Commons: Attribution (CC BY)

GillC2023Cocaine-inducedFinal published version, 2.02 MBLicence: Creative Commons: Attribution (CC BY)

Cite this

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title = "Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition",

abstract = "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.",

keywords = "cocaine, drug-induced vasculitis, matosis with polyangiitis, ANCA, treatment",

author = "Charn Gill and Joseph Sturman and Leyla Ozbek and Henderson, {Scott R} and Aine Burns and Sally Hamour and Pepper, {Ruth J} and Lisha McClelland and Dimitrios Chanouzas and Simon Gane and Salama, {Alan D} and Lorraine Harper",

note = "{\textcopyright} The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.",

year = "2023",

month = apr,

day = "4",

doi = "10.1093/rap/rkad027",

language = "English",

volume = "7",

journal = "Rheumatology Advances in Practice",

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T1 - Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition

AU - Gill, Charn

AU - Sturman, Joseph

AU - Ozbek, Leyla

AU - Henderson, Scott R

AU - Burns, Aine

AU - Hamour, Sally

AU - Pepper, Ruth J

AU - McClelland, Lisha

AU - Chanouzas, Dimitrios

AU - Gane, Simon

AU - Salama, Alan D

AU - Harper, Lorraine

PY - 2023/4/4

Y1 - 2023/4/4

N2 - 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.

AB - 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.

KW - cocaine

KW - drug-induced vasculitis

KW - matosis with polyangiitis

KW - ANCA

KW - treatment

U2 - 10.1093/rap/rkad027

DO - 10.1093/rap/rkad027

M3 - Article

C2 - 37026037

SN - 2514-1775

VL - 7

JO - Rheumatology Advances in Practice

JF - Rheumatology Advances in Practice

IS - 1

M1 - rkad027

ER -

Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition

Abstract

Bibliographical note

Keywords

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