| Key messages |
| • Cocaine toxicology should be performed especially in young patients diagnosed with limited granulomatosis with polyangiitis. • ANCA is common, but the subtype is not specific for cocaine-induced nasal disease. • Treatment should focus on cocaine withdrawal rather than immunosuppression. |
| Summary |
> Objectives Cocaine and cocaine mixed with levamisole are increasingly used in the UK and cause significant direct nasal damage as well as promoting vasculitis. Our objectives were the following: (1) identify the main symptoms and presentation of cocaine-induced vasculitis; (2) provide evidence on best practices for the investigation and diagnosis of cocaine-induced vasculitis; and (3) analyze patients’ clinical outcomes to understand optimal management of the condition. > Methods We performed a retrospective case series analysis of patients presenting with cocaine-induced midline destructive lesions or vasculitis consistent 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 the 23 samples provided tested positive when routine urine toxicology was performed; 9 patients who denied ever using cocaine were identified as cocaine users based on a urine toxicology analysis, and 11 who said they were former 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 presented with acute renal failure. 56% of our patients tested positive for PR3-ANCA and none tested positive for MPO-ANCA. Remission of symptoms required discontinuation of cocaine even when immunosuppression was administered. |
| Editor’s note : Antineutrophil cytoplasmic antibodies (ANCA) are immunoglobulins (Ig), usually IgG, directed against proteins in the primary granules of polymorphonuclear leukocytes and the lysosomes of monocytes. They are detected in patients with small vessel vasculitis, but their true role in the pathogenesis of these diseases is unknown and they are not always related to clinical activity. Various studies demonstrate the existence of ANCA in other diseases, such as infections or neoplasms, and in up to 2% of healthy individuals. In these cases they could be considered epiphenomena of inflammation. |
| Conclusion |
Our data show that cocaine-induced vasculitis is more common than first reported and that toxicology should be considered in all patients who appear to have isolated nasal involvement with vasculitis.
We argue that MPO-ANCA positivity might not be as common as previously reported and that dual positive ANCAs are rare and therefore lack of PR3 and dual positive MPO-ANCA should not be used to exclude induced disease. for cocaine.
Patients with destructive nasal lesions , especially young patients, should undergo urinary toxicology testing for cocaine before GPA is diagnosed and immunosuppressive therapy is considered.
The ANCA pattern is not specific for cocaine-induced destructive midline lesions.
We are reassured to report that renal and pulmonary involvement appears to be rare. Furthermore, we advocate cessation of cocaine use as a first strategy, without the use of immunosuppressants, for the management of these patients.
| Comments |
A new article in Rheumatology Advances in Practice , published by Oxford University Press, indicates that granulomatosis with polyangiitis, a nasal disease that causes inflammation of blood vessels and commonly presents with symptoms in the sinuses, throat, lungs, and kidneys, can be commonly misdiagnosed. Researchers believe that many patients identified with the sinus-nasal limited form of the disease may actually be suffering from nasal damage due to cocaine use.
Cocaine is the second most commonly abused drug in the UK, with 2.6% of the population aged 16-59 using it. About 4.8 million people in the United States (or 1.7% of those ages 12 and older) report using cocaine in the past year. Cocaine can cause significant health problems, including cocaine-induced destructive midline lesions and several other vascular problems. However, evidence shows that cocaine use can trigger the production of certain antibodies that can lead to a clinical presentation that closely resembles idiopathic granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis ).
Along with occasional general symptoms such as arthralgia, fatigue, and rash , the similarity between idiopathic granulomatosis with polyangiitis (GPA) and damage due to cocaine makes diagnosis difficult for doctors. While idiopathic granulomatosis with polyangiitis (GPA) is rare, affecting approximately 3 in every 100,000 people, researchers here believe the potential for misdiagnosis is serious, as common treatments for GPA can be ineffective and even dangerous. for cocaine users.
The researchers here conducted a retrospective review of patients who visited vasculitis clinics for treatment at the Queen Elizabeth Hospital, Birmingham, and the Royal Free Hospital, London. They identified 42 patients and found that current cocaine use was common, about 86% of the samples provided were positive when routine urine toxicology was performed; 9 patients who denied ever using cocaine were identified as cocaine users based on urine toxicology analysis, while 11 who claimed to be former users still tested positive.
The researchers note that ten patients referred to vasculitis treatment centers had previously been diagnosed with GPA and given immunosuppressive medications and, despite this treatment, still had ongoing nasal problems.
The researchers here believe that doctors should screen patients with destructive nasal lesions or isolated sinonasal disease for cocaine before making a diagnosis of GPA. Immunosuppressive medications, they note, are often ineffective if cocaine use persists and would also increase the risk of significant adverse effects, including infection.
“This is an important paper that has changed our practice,” said Aine Burns, one of the paper’s authors. “We now include urine samples for drugs of abuse in our initial investigations of patients with idiopathic granulomatosis with polyangiitis (GPA) and in those who appear not to be responding to treatment. Sadly, we have seen young people with life-changing disfigurement due to cocaine-induced granulomatosis with polyangiitis . A better understanding of this condition prevents us from further harming patients by administering inappropriate, potentially toxic and useless treatments. “There needs to be greater awareness of this complication of cocaine use among users, the public and health professionals.”
| What does this mean for patients ? |
Cocaine causes a form of inflammation of the blood vessels that primarily leads to a skin rash and destruction of nasal structures. This can mimic a disease called granulomatosis with polyangiitis (GPA), in which inflammation of the blood vessels occurs for an unknown reason. There is little evidence on how best to detect and treat cocaine-induced illness and whether it should be treated with strong immunosuppression such as GPA.
In our study, we reviewed data from 42 patients with cocaine-induced disease over a 5-year period. Our results show that 32% denied cocaine use but tested positive in the urine test. ANCA antibody is commonly found in patients with cocaine-induced disease and GPA; the ANCA pattern does not differentiate between the two conditions.
Patients found resolution of their symptoms only once they stopped using cocaine, and treatment with strong immunosuppression alone did not cause resolution of symptoms. This study suggests that all patients presenting with skin rashes and nasal symptoms similar to GPA should undergo urinalysis. Furthermore, the most important intervention for a good clinical outcome is the cessation of cocaine use. This is more important than the use of immunosuppression, which could potentially harm patients who use cocaine.
Reference : “ Cocaine-induced granulomatosis with polyangiitis – an under-recognized condition,” is available ". Rheumatology Advances in Practice , Volume 7, Issue 1, 2023, rkad027, https://doi.org/10.1093/rap/rkad027
