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Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: Clínica y tratamiento / Anti-neutrophil cytoplasmic antibody-associated vasculitis: Clinical aspects and treatment
Paolini, María Virginia; Ruffino, Juan Pablo; Fernández Romero, Diego S..
  • Paolini, María Virginia; Hospital Dr. Carlos G. Durand. Unidad de Inmunología e Histocompatibilidad. Buenos Aires.
  • Ruffino, Juan Pablo; Hospital Dr. Carlos G. Durand. Unidad de Inmunología e Histocompatibilidad. Buenos Aires.
  • Fernández Romero, Diego S.; Hospital Dr. Carlos G. Durand. Unidad de Inmunología e Histocompatibilidad. Buenos Aires.
Medicina (B.Aires) ; 73(2): 119-126, abr. 2013. tab
Article in Spanish | LILACS | ID: lil-694750
RESUMEN
Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA) comprenden a un grupo de enfermedades caracterizadas por la inflamación de la pared de pequeños vasos. Analizamos las características epidemiológicas y clínicas en una serie de 47 pacientes 23 (49%) granulomatosis de Wegener (GW), 15 (32%) poliangeítis microscópica (PAM) y nueve (19%) vasculitis limitada al riñón (VLR). La edad media al inicio de los síntomas fue de 50.7 ± 14.9 años. La manifestación clínica más frecuente fue el compromiso renal en 41 (87%) pacientes, seguido por el pulmonar en 26 (55%) y el otorrinolaringológico en 17 (36%). En 26 (55%) se asoció compromiso renal y pulmonar. La forma clínica más frecuente fue la generalizada en 23 (49%), seguida por la grave en 18 (38%). El 89% presentaron determinaciones de ANCA positivas. Cuatro (8%) no recibieron tratamiento inmunosupresor de inicio. De los 43 que recibieron tratamiento de inicio, 29 (67%) tuvieron remisión completa, con un tiempo de remisión promedio de 35.3 meses. Once (26%) presentaron recaídas, diez (91%) recaídas mayores y uno (9%) menor. Doce (28%) fallecieron, siete en forma temprana y cinco durante la evolución de la enfermedad. Quince (31%) evolucionaron a insuficiencia renal crónica. Los 26 pacientes en seguimiento tuvieron respuesta al tratamiento y 20 (77%) de ellos estaban en remisión al finalizar el estudio. Las vasculitis asociadas a ANCA continúan siendo enfermedades de alta morbilidad y mortalidad, a pesar de las mejorías logradas con los tratamientos inmunosupresores.
ABSTRACT
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, comprise a group of diseases characterized by inflammation of the wall of small vessels. We analyzed epidemiological and clinical characteristics in a series of 47 patients, 23 (49%) with Wegener granulomatosis (WG), 15 (32%) with microscopic polyangiitis (MPA) and nine (19%) with renal limited vasculitis (RLV). The mean age at onset of symptoms was 50.7 ± 14.9 years. The most frequent clinical manifestation was renal involvement in 41 (87%), followed by pulmonary manifestations in 26 (55%) and ENT involvement in 17 (36%). In 26 (55%) it presented with simultaneous pulmonary and renal involvement. The most frequent clinical category was the generalized form in 23 (49%), followed by the severe form in 18 (38%). Eighty nine percent of patients had positive ANCA test. Four (8%) received no immunosuppressive treatment. Of the 43 patients who were treated, 29 (67%) achieved complete remission with an average length of remission of 35.3 months. Eleven (26%) had a relapse, ten (91%) had a major relapse and one had a minor relapse. Twelve (28%) patients died, seven died early and five late during the course of the disease. Fifteen (31%) progressed to chronic renal failure. All 26 patients in follow-up had response to treatment and 20 (77%) were in remission at the end of the study. Despite the improvements achieved with immunosuppressive treatments, morbidity and mortality rates in ANCA-associated vasculitis remain high.
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Full text: Available Index: LILACS (Americas) Main subject: Antibodies, Antineutrophil Cytoplasmic / Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Kidney Diseases Type of study: Diagnostic study / Observational study / Prognostic study / Risk factors Limits: Adult / Aged / Aged80 / Female / Humans / Male Country/Region as subject: South America / Argentina Language: Spanish Journal: Medicina (B.Aires) Journal subject: Medicine Year: 2013 Type: Article Affiliation country: Argentina

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Full text: Available Index: LILACS (Americas) Main subject: Antibodies, Antineutrophil Cytoplasmic / Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Kidney Diseases Type of study: Diagnostic study / Observational study / Prognostic study / Risk factors Limits: Adult / Aged / Aged80 / Female / Humans / Male Country/Region as subject: South America / Argentina Language: Spanish Journal: Medicina (B.Aires) Journal subject: Medicine Year: 2013 Type: Article Affiliation country: Argentina