RESUMEN
OBJECTIVES: To evaluate the prevalence and meaning of antineutrophil cytoplasmic antibodies (ANCA) positivity in a cohort of IgG4-related disease (IgG4-RD). METHODS: We identified patients with ANCA determination from a retrospective cohort of 69 patients with IgG4-RD. ANCA were measured by indirect immunofluorescence microscopy (IIF) and/or proteinase 3 (PR3)-ANCA and MPO-ANCA by ELISA. IIF patterns were classified as perinuclear (P-ANCA), cytoplasmic (C-ANCA) and atypical (X-ANCA). We compared the ANCA-positive vs the ANCA-negative IgG4-RD group. RESULTS: Out of 69 patients, 31 IgG4-RD patients had an ANCA determination. Four patients with concomitant systemic autoimmune diseases were excluded. We found positive ANCA by IIF in 14 (56%) of 25 patients tested. The most common IIF pattern was C-ANCA in eight (57.1%), followed by dual C-ANCA/X-ANCA in four (28.6%) and P-ANCA and dual C-ANCA/P-ANCA in one each (7.1%). Of the 20 patients with ANCA determination by both IIF and ELISA, four have positive ANCA by ELISA (three for MPO-ANCA and one for PR3-ANCA). Of the two patients with only ELISA determination, one was positive for MPO-ANCA. The prevalence of ANCA positivity by ELISA was 22.7% (5 out of 22 patients). ANCA was more frequent in the Mikulizc/systemic phenotype (42.9%) compared with other phenotypes (P = 0.04). ANCA-positive IgG4-RD patients had more frequently lymph node and kidney involvement, high IgG1 levels and erythrocyte sedimentation rate, and positive antinuclear antibodies. CONCLUSION: ANCA are found in a significant number of patients with IgG4-RD and differed from the ANCA-negative group in terms of clinical and serological features.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Enfermedad Relacionada con Inmunoglobulina G4/inmunología , Enfermedades Renales/inmunología , Ganglios Linfáticos/inmunología , Mieloblastina/inmunología , Peroxidasa/inmunología , Adulto , Anciano , Enfermedades de la Aorta/inmunología , Enfermedades de las Vías Biliares/inmunología , Estudios de Casos y Controles , Femenino , Humanos , Enfermedades del Aparato Lagrimal/inmunología , Hepatopatías/inmunología , Masculino , Microscopía Fluorescente , Persona de Mediana Edad , Mieloblastina/metabolismo , Enfermedades Pancreáticas/inmunología , Peroxidasa/metabolismo , Espacio Retroperitoneal , Estudios Retrospectivos , Enfermedades de las Glándulas Salivales/inmunologíaRESUMEN
Nesta revisao sao explicados cada um dos quatro mecanismos de defesa da bile contra a infecçao por microorganismos patógenos, bem como suas açoes integradas. Nesse contexto, incluem-se as barreiras anatômicas (complexos unitivos e esfíncter de Oddid), os mecanismos físicos (fluxo biliar e muco), os fatores químicos (sais biliares) e os mecanismos imunológicos (células de Kupffer e imunoglobulina A secretada). A quebra do funcionamento harmônico desses mecanismos pode levar a sérias infecçoes. Nesse sentido, o aumento da pressao intra-biliar (causada por obstruçao parcial ou completa do fluxo biliar) e doenças do parênquima hepático desempenham papel fundamental. Dessa forma, confirma-se a importância da preservaçao desses mecanismos de defesa no indivíduo saudável.
Asunto(s)
Sistema Biliar/inmunología , Enfermedades de las Vías Biliares/inmunología , Infecciones Bacterianas/inmunología , Ácidos y Sales Biliares/fisiología , Bilis/metabolismo , Sistema Biliar/microbiología , Enfermedades de las Vías Biliares/microbiología , Esfínter de la Ampolla Hepatopancreática/inmunología , Inmunoglobulinas/metabolismo , Infecciones Bacterianas/microbiología , Moco/metabolismoRESUMEN
A test of lymphocyte cytotoxicity for isolated adult rabbit hepatocytes has been performed using lymphocytes from 40 children with acute or chronic liver disease. Positive cytotoxicity was not observed in 26 children without liver disease and rarely in 13 children with disease affecting primarily the biliary tract. Temporarily positive tests were found in those with acute hepatocellular disease, but tests remained positive in patients with chronic active hepatitis, while liver function tests remained abnormal. Persistently positive test occurred in those with liver disease associated with alpha-antitrypsin deficiency. Such altered immunoresponsiveness could be an important pathogenic mechanism leading to chronic liver disease in childhood.