Granulomatosis with polyangiitis according to geographic origin and ethnicity: clinical-biological presentation and outcome in a French population.

Objectives: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans. Methods: Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed. Results: Among the 760 patients, 689 (91%) were white Europeans, 33 (4.3%) were North Africans and 22 (2.9%) were sub-Saharans (n = 8) or Afro-Caribbeans (French West Indies, n = 14). Black sub-Saharans and Afro-Caribbeans, compared with white Europeans, were significantly younger at GPA diagnosis (P = 0.003), had more frequent central nervous system involvement (P = 0.02), subglottic stenosis (P = 0.002) and pachymeningitis (P = 0.009), and tended to have more frequent chondritis and retroorbital tumour. Median serum creatinine levels and Birmingham Vasculitis Activity Score were significantly lower in sub-Saharans and Afro-Caribbeans (P = 0.002 and P = 0.003, respectively). In contrast, in comparison with white Europeans, North Africans had only less frequent arthralgias (P = 0.004). Time to relapse was shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans [adjusted HR = 1.96 (95% CI: 1.09, 3.51) (P = 0.02)], and did not differ for North Africans. In contrast, overall survival was not significantly different according to ethnicity. Conclusion: Our findings indicated different GPA clinical presentations in white Europeans and sub-Saharans and Afro-Caribbeans, with black patients having more frequent severe granulomatous manifestations. In addition, time to relapse was significantly shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans.

Prevención, diagnóstico y manejo de lesiones laringotraqueales agudas y subagudas posintubación en pacientes pediátricos / Prevention, diagnosis and management of acute and subacute laryngotracheal post-intubation lesions in pediatric patients

Las lesiones laringotraqueales asociadas a intubación se deben principalmente a una técnica defectuosa y a daño por presión del tubo endotraqueal sobre la mucosa; además influyen características propias del paciente y de los cuidados de enfermería. Hasta el 40% de los pacientes pediátricos intubados pueden presentar alteraciones laríngeas inmediatas y hasta 30% tienen estridor o disnea posextubación. Si estos síntomas persisten por más de 3 días tendrían indicación de laringotraqueoscopía. Las lesiones más habituales son edema, úlceras y tejido de granulación. El edema puede producir obstrucción respiratoria aguda que puede manejarse con reintubación con tubos más pequeños y aplicación tópica de crema de corticoides con antibióticos. Las úlceras y granulaciones pueden evolucionar hacia secuelas cicatriciales que comprometen la fisiología laringotraqueal; las granulaciones exofíticas deben retirarse endoscópicamente. Aunque la incidencia de estenosis subglótica posintubación ha disminuido en las últimas décadas, situándose entre 2,7%y 4,2%, algunos estudios sugieren un subdiagnóstico debido a lesiones poco sintomáticas al alta. En el período cicatricial activo, estas estenosis pueden dilatarse para evitar llegar a una cirugía abierta. El manejo otorrinolaringológico de estos pacientes en etapas tempranas es fundamental para evitar secuelas cicatriciales irreversibles que requieren de cirugías complejas, con riesgo vital por obstrucción de la vía aérea.

Intubation-associated laryngotracheal injuries are mainly caused by a defective technique and endotracheal tube pressure-induced mucosal damage; patient factors and nursing care are also important. Up to 40% of intubated pediatric patients may show immediate laryngeal alterations and up to 30% have post-extubation stridor or dyspnea. If these symptoms last for over 3 days, laryngotracheoscopy is indicated. Edema, ulcers and granulation tissue are the most usual lesions. Edema can lead to acute airway obstruction, and is managed by reintubation with a smaller tube and topical application of a corticosteroid and antibiotic cream. Ulcers and granulations can lead to scarring that compromise laryngotracheal physiology; exophytic granulations must be removed endoscopically. Although the incidence of post-intubation subglottic stenosis has diminished over the last decades to about2,7% to 4,2%, some studies suggest that there is a subdiagnosis because of oligosymptomatic lesions at the time of discharge. On the active scarring period, dilatation of the stenosis can be used to avoid open surgery. Early otorhinolaryngologic management of these patients is fundamental for avoiding irreversible cicatricial sequels that require complex surgeries, with life risk due to airway obstruction.