The cancer risk according to three subtypes of ANCA-associated vasculitis: A propensity score-matched analysis of a nationwide study.

OBJECTIVE: It remains unknown whether cancer risk differs among the three subtypes of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and what the cancer risk factors are. We conducted a nationwide study in Korea to evaluate the risk of cancer in patients with AAV and to identify the risk factors for cancer. METHODS: We analyzed the Health Insurance Review and Assessment database of Korea and identified 1982 patients diagnosed with AAV between January 1, 2007 and December 31, 2017. The patients and controls with no history of AAV or cancer were matched 1:4 by propensity scores. The study outcome measure was incidence of cancer during 11 years of follow-up. RESULTS: Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) numbered 684, 606, and 692, respectively. The overall incidence of cancer was higher among patients with AAV than in controls (HR 1.32, 95% CI 1.08-1.61). The risk of hematological malignancy, lung cancer, and bladder cancer in the GPA group, lung cancer in the MPA group, and hematological malignancy in the EGPA group were significantly higher than in controls (HR 7.39, 3.20, 4.20, 2.86, and 4.65, respectively). Age, male sex, GPA subtype, and cyclophosphamide use were significantly associated with cancer risk in patients with AAV. CONCLUSION: Overall cancer incidence was increased in patients with AAV. Cancer risk was higher in patients with GPA than in those with MPA or EGPA. The use of cyclophosphamide was associated with an increased risk of cancer, while rituximab was not.

Eosinophilic Granulomatosis With Polyangiitis Presenting as an Acute Coronary Syndrome.

Eosinophilic granulomatosis with polyangiitis, formerly Churg-Strauss Syndrome, is an uncommon disorder that carries a high mortality when coronary artery disease develops. Early recognition and treatment is crucial. We highlight an unusual presentation of acute coronary syndrome not associated with atherosclerotic coronary disease. (Level of Difficulty: Intermediate.).

Antineutrophilic cytoplasmic antibody-associated vasculitis with and without renal involvement: C3 contributes to prognosis, but renal involvement does not.

OBJECTIVES: We investigated the impact of renal involvement at diagnosis on the prognosis of patients with antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: The relationship between renal involvement at diagnosis, clinical variables at diagnosis, and prognosis (including relapse episodes, initiation of dialysis, and death) was examined in 101 Japanese patients with AAV. RESULTS: Sixty-eight patients had renal involvement at diagnosis. The renal-involvement patients had significantly higher ages at diagnosis, significantly lower hemoglobin levels, and significantly lower platelet levels. They had significantly lower C3 levels, but showed no significant difference in C4 levels. Overall survival rate was significantly worse in patients with than in patients without renal involvement (P = 0.003, log-rank test). Multivariable analysis using a logistic regression model demonstrated that C3 contributed to dialysis initiation: odds ratio (per 10 mg/dL of C3): 0.68; range: 0.49-0.90; P = 0.007. A Cox proportional hazard model revealed that the C3 level and age at diagnosis contributed significantly to overall survival: hazard ratio (per 10 mg/dL of C3) 0.81, range 0.69-0.95, P = 0.011; 1.08, 1.02-1.15, P = 0.013, respectively. Renal involvement did not contribute significantly to overall survival. Patients with C3 levels ≥100 mg/dL had a better survival rate than patients with C3 levels <100 mg/dL. CONCLUSIONS: Although patients with renal involvement had higher ages, lower C3 levels at diagnosis, and poorer prognoses, multivariable analysis demonstrated that the C3 level and age at diagnosis, but not renal involvement, contributed significantly to overall survival. Our results demonstrate the relationship between C3 hidden behind renal involvement and AAV prognosis.

Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener / Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC), que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW) nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC) dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.