RESUMEN

INTRODUCTION: The complement system has an important role in the pathogenesis of vasculitis associated with antineutrophilic cytoplasmic antibody (AAV) mainly at the level of the kidneys because patients with complement deposits on the glomerular basal membrane present more aggressive disease compared with those with pauci-immune vasculitis. AIM: To analyze the association of hypocomplementemia with the clinical manifestations, laboratory data, renal histology, progress to renal insufficiency, and mortality of patients with AAV. METHODS: Retrospective cohort study (2000-2007) included 93 patients with AAV. Hypocomplementemia is defined as having C3 values lower than 80 mg/dL or C4 values below 15 mg/dL. Demographic, statistical, clinical, hematological, serological, and histopathological characteristics of all the patients with and without diagnosis of hypocomplementemia were compared. In order to evaluate variable independence, a logistic regression analysis was used. RESULTS: Ninety-three patients were studied of whom 63 (67.7%) had complement dosage at the moment of AAV diagnosis. Seven patients (11.1%) presented hypocomplementemia and a greater kidney involvement compared with normocomplementemic patients. Thirty renal biopsies were analyzed and 4 (13.3%) showed immunocomplex (IC) or complement deposits by an immunofluorescence test (IFT). Patients with "non-pauci-immune" AAV also presented terminal chronic renal disease (TCRD). CONCLUSION: There is an association between low complement and the degree of renal damage in patients with AAV. Patients with renal biopsies confirming IC and/or complement deposits showed more aggressive renal disease. Key Points • The complement system has an important role in the pathogenesis of vasculitis associated to antineutrophilic cytoplasmic antibody. • The studies in murine models confirming the complement activation by alternative pathway and particularly the receptor C5a (C5aR) is necessary for the development of glomerulonefritis. • Complement deposit observed in the renal biopsies of patients diagnosed with AAV was correlated to greater kidney damage, greater proteinuria and major disease activity compared to patients diagnosed with typical pauci-immune vasculitis. • The presence of hypocomplementemia at the onset of the disease was also associated with a greater organ involvement, poor prognosis and greater mortality.

Asunto(s)

Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Activación de Complemento , Complemento C3/análisis , Complemento C4/análisis , Enfermedades Renales/inmunología , Adulto , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Biopsia , Complemento C3/inmunología , Complemento C4/inmunología , Femenino , Estudios de Seguimiento , Granulomatosis con Poliangitis/sangre , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/inmunología , Humanos , Riñón/inmunología , Riñón/patología , Enfermedades Renales/complicaciones , Enfermedades Renales/diagnóstico , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/diagnóstico , Fallo Renal Crónico/inmunología , Masculino , Microscopía Fluorescente , Persona de Mediana Edad , Análisis de Regresión , Estudios Retrospectivos

RESUMEN

BACKGROUND: Leprosy is a treatable infectious disease caused by Mycobacterium leprae. However, there is additional morbidity from leprosy-associated pathologic immune reactions, reversal reaction (RR) and erythema nodosum leprosum (ENL), which occur in 1 in 3 people with leprosy, even with effective treatment of M. leprae. There is currently no predictive marker in use to indicate which people with leprosy will develop these debilitating immune reactions. Our peripheral blood mononuclear cell (PBMC) transcriptome analysis revealed that activation of the classical complement pathway is common to both RR and ENL. Additionally, differential expression of immunoglobulin receptors and B cell receptors during RR and ENL support a role for the antibody-mediated immune response during both RR and ENL. In this study, we investigated B-cell immunophenotypes, total and M. leprae-specific antibodies, and complement levels in leprosy patients with and without RR or ENL. The objective was to determine the role of these immune mediators in pathogenesis and assess their potential as biomarkers of risk for immune reactions in people with leprosy. METHODOLOGY/FINDINGS: We followed newly diagnosed leprosy cases (n = 96) for two years for development of RR or ENL. They were compared with active RR (n = 35), active ENL (n = 29), and healthy household contacts (n = 14). People with leprosy who subsequently developed ENL had increased IgM, IgG1, and C3d-associated immune complexes with decreased complement 4 (C4) at leprosy diagnosis. People who developed RR also had decreased C4 at leprosy diagnosis. Additionally, elevated anti-M. leprae antibody levels were associated with subsequent RR or ENL. CONCLUSIONS: Differential co-receptor expression and immunoglobulin levels before and during immune reactions intimate a central role for humoral immunity in RR and ENL. Decreased C4 and elevated anti-M. leprae antibodies in people with new diagnosis of leprosy may be risk factors for subsequent development of leprosy immune reactions.

Asunto(s)

Anticuerpos Antibacterianos/sangre , Complemento C3d/análisis , Complemento C4/análisis , Eritema Nudoso/epidemiología , Inmunoglobulina G/sangre , Inmunoglobulina M/sangre , Lepra Lepromatosa/epidemiología , Mycobacterium leprae/inmunología , Adulto , Anciano , Anticuerpos Antibacterianos/inmunología , Linfocitos B/inmunología , Complemento C3d/inmunología , Complemento C4/inmunología , Eritema Nudoso/sangre , Eritema Nudoso/inmunología , Femenino , Perfilación de la Expresión Génica , Humanos , Inmunidad Activa/inmunología , Inmunoglobulina G/inmunología , Inmunoglobulina M/inmunología , Lepra Lepromatosa/sangre , Lepra Lepromatosa/inmunología , Masculino , Persona de Mediana Edad , Factores de Riesgo

RESUMEN

Benzene is a recognized human carcinogen; however, there are still some gaps in the knowledge regarding the mechanism of toxicity of this organic solvent and potential early biomarkers for the damage caused by it. In a previous study, our research group demonstrated that the adhesion molecules of the immune system (B7.1 and B7.2) could be potential biomarkers in the early detection of immunotoxicity caused by benzene exposure. Therefore, this study was developed to deepen the understanding regarding this important topic, aiming to contribute to the comprehension of the benzene toxicity mechanism mediated by B7.1 and B7.2 and its potential association with the risk of carcinogenicity. B7.1 and B7.2 protein expression in blood monocytes and B7.1 and B7.2 gene expression in PBMCs were evaluated. Additionally, complement C3 and C4 levels in serum were measured, as well as p53 gene expression in PBMCs. Seventy-four gas station workers (GSW group) and 71 non-occupationally exposed subjects (NEG) were evaluated. Our results demonstrated decreased levels of B7.1 and B7.2 protein and gene expression in the GSW group compared to the NEG (n = 71) (p < 0.01). Along the same lines, decreased levels of the complement system were observed in the GSW group (p < 0.01), demonstrating the impairment of this immune system pathway as well. Additionally, a reduction was observed in p53 gene expression in the GSA group (p < 0.01). These alterations were associated with both the benzene exposure biomarker evaluated, urinary trans, trans-muconic acid, and with exposure time (p < 0.05). Moreover, strong correlations were observed between the gene expression of p53 vs. B7.1 (r = 0.830; p < 0.001), p53 vs. B7.2 (r = 0.685; p < 0.001), and B7.1 vs. B7.2 (r = 0.702; p < 0.001). Taken together, these results demonstrate that the immune system co-stimulatory molecule pathway is affected by benzene exposure. Also, the decrease in p53 gene expression, even at low exposure levels, reinforces the carcinogenicity effect of benzene in this pathway. Therefore, our results suggest that the promotion of immune evasion together with a decrease in p53 gene expression may play an important role in the benzene toxicity mechanism. However, further and targeted studies are needed to confirm this proposition.

Asunto(s)

Antígeno B7-1/inmunología , Antígeno B7-2/inmunología , Benceno/toxicidad , Neoplasias/inmunología , Exposición Profesional , Biomarcadores , Carcinógenos , Estudios de Casos y Controles , Complemento C3/inmunología , Complemento C4/inmunología , Humanos , Proteína p53 Supresora de Tumor/genética

RESUMEN

Triple positivity (TP) for antiphospholipid antibodies(aPL) may identify aPL carriers with poorer prognosis. The clinical impact of TP in primary antiphospholipid syndrome(PAPS) remains unclear and further clinical evidences are needed to validate TP as a marker of severity. The aim of this study was to evaluate the impact of TP on the clinical course of PAPS with thrombosis(t-PAPS). We performed a retrospective analysis of a cohort of t-PAPS patients, comparing groups of patients with TP and non-TP profiles according to their demographic, clinical and laboratory features. We included 105 patients with t-PAPS, the median follow-up time of 3.7 years. Twenty-two patients(21%) had TP; the demographic distribution, the presence of cardiovascular risk factors and the site of thrombosis were similar between TP and non-TP patients. The frequency of thrombotic events did not differ between TP and non-TP patients during the study period. Pregnancy morbidities were more frequent in women with t-PAPS and TP than in those with non-TP profile (80% vs. 52.8%, P = 0.05). Patients with t-PAPS and TP presented, at diagnosis, higher dRVVT ratio (median R = 2.44 vs. 1.57, P < 0.0001), higher aCL titer (median = 50UI vs. 35 UI, P < 0.0001), lower C3 levels (median = 1.08 vs. 1.30 mg dL-1 , P = 0.001), lower C4 levels (median = 0.22 vs. 0.25 mg dL-1 , P = 0.05) and higher frequency of positive ANA test (50% vs. 20%, P = 0.008) than patients with t-PAPS and non-TP. Lower-than-normal levels of C3 was independently associated with TP (OR = 5.1, P = 0.02). The presence of TP in patients with t-PAPS was associated with immune derangement, with no effect on the clinical course of the disease.

Asunto(s)

Anticuerpos Antifosfolípidos/inmunología , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/inmunología , Autoanticuerpos/inmunología , Trombosis/etiología , Adulto , Síndrome Antifosfolípido/diagnóstico , Biomarcadores , Comorbilidad , Complemento C3/inmunología , Complemento C4/inmunología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Embarazo , Complicaciones del Embarazo , Estudios Retrospectivos , Factores de Riesgo , Trombosis/diagnóstico , Trombosis/mortalidad , Trombosis/terapia , Adulto Joven

RESUMEN

Blood-feeding insects inject potent salivary components including complement inhibitors into their host's skin to acquire a blood meal. Sand fly saliva was shown to inhibit the classical pathway of complement; however, the molecular identity of the inhibitor remains unknown. Here, we identified SALO as the classical pathway complement inhibitor. SALO, an 11 kDa protein, has no homology to proteins of any other organism apart from New World sand flies. rSALO anti-complement activity has the same chromatographic properties as the Lu. longipalpis salivary gland homogenate (SGH)counterparts and anti-rSALO antibodies blocked the classical pathway complement activity of rSALO and SGH. Both rSALO and SGH inhibited C4b deposition and cleavage of C4. rSALO, however, did not inhibit the protease activity of C1s nor the enzymatic activity of factor Xa, uPA, thrombin, kallikrein, trypsin and plasmin. Importantly, rSALO did not inhibit the alternative or the lectin pathway of complement. In conclusion our data shows that SALO is a specific classical pathway complement inhibitor present in the saliva of Lu. longipalpis. Importantly, due to its small size and specificity, SALO may offer a therapeutic alternative for complement classical pathway-mediated pathogenic effects in human diseases.

Asunto(s)

Inactivadores del Complemento/farmacología , Vía Clásica del Complemento/efectos de los fármacos , Proteínas de Insectos/farmacología , Psychodidae/inmunología , Psychodidae/metabolismo , Saliva/metabolismo , Animales , Cromatografía Líquida de Alta Presión , Activación de Complemento/efectos de los fármacos , Complemento C1/antagonistas & inhibidores , Complemento C1/inmunología , Complemento C1/metabolismo , Complemento C4/antagonistas & inhibidores , Complemento C4/inmunología , Complemento C4/metabolismo , Humanos , Proteínas Recombinantes/farmacología

RESUMEN

We investigated in Brazilian women with SLE the prevalence and levels of high avidity (HA) dsDNA antibodies and tested their correlation with lupus activity and biomarkers of renal disease. We also compared these correlations to those observed with total dsDNA antibodies and antibodies against nucleosome (ANuA). Autoantibodies were detected by ELISA, while C3 and C4 levels were determined by nephelometry. Urine protein/creatinine ratio was determined, and lupus activity was measured by SLEDAI-2K. The prevalence of total and HA dsDNA antibodies was similar to but lower than that verified for ANuA. The levels of the three types of antibodies were correlated, but the correlation was more significant between HA dsDNA antibodies and ANuA. High avidity dsDNA antibodies correlated positively with ESR and SLEDAI and inversely with C3 and C4. Similar correlations were observed for ANuA levels, whereas total dsDNA antibodies only correlated with SLEDAI and C3. The levels of HA dsDNA antibodies were higher in patients with proteinuria, but their levels of total dsDNA antibodies and ANuA were unaltered. High avidity dsDNA antibodies can be found in high prevalence in Brazilian women with SLE and are important biomarkers of active disease and kidney dysfunction.

Asunto(s)

Anticuerpos Antinucleares/inmunología , Afinidad de Anticuerpos/inmunología , Lupus Eritematoso Sistémico/inmunología , Adolescente , Adulto , Anciano , Anticuerpos Antinucleares/sangre , Biomarcadores , Brasil , Complemento C3/inmunología , Complemento C4/inmunología , Femenino , Humanos , Inmunoglobulina G/sangre , Inmunoglobulina G/inmunología , Lupus Eritematoso Sistémico/patología , Lupus Eritematoso Sistémico/fisiopatología , Persona de Mediana Edad , Proteinuria/etiología , Proteinuria/metabolismo , Adulto Joven

RESUMEN

BACKGROUND: Obesity in pregnancy is associated with systemic inflammation, immunological changes and adverse maternal-fetal outcomes. Information on the association between maternal obesity and breast milk composition is scarce. This study describes changes and relationships between biochemical and immunological parameters of colostrum and serum of overweight and obese women. METHODS: Colostrum and blood samples were collected from 25 normal weight, 24 overweight and 19 obese women for determination of glucose, total protein, triglycerides, cholesterol, immunoglobulins, complement proteins (C3 and C4), fat and calorie content and C-reactive protein (CRP). RESULTS: Glucose was higher in colostrum of obese women (p = .002). In normal weight and obese women, total protein content was higher in colostrum than in serum (p = .001). Serum triglycerides (p = .008) and cholesterol (p = .010) concentrations were significantly higher in overweight and obese women than in their normal weight counterparts, but in colostrum their concentrations were similar across the three groups. Secretory IgA (sIgA) in colostrum and IgA in serum concentrations were significantly higher (p = .001) in overweight and obese mothers, whereas IgG and IgM concentrations did not vary among the groups (p = .825). Serum C3 (p = .001) and C4 (p = .040) concentrations were higher in obese women. No differences in colostrum complement proteins were detected among the groups. Calorie content (p = .003) and fat (p = .005) concentrations in colostrum and serum CRP (p = .002) were higher in obese women. CONCLUSIONS: The results corroborate the hypothesis that colostrum of overweight and obese women undergoes biochemical and immunological changes that affect its composition, namely increasing glucose concentrations, calorie content, fat and sIgA concentrations.

Asunto(s)

Calostro/inmunología , Obesidad/inmunología , Complicaciones del Embarazo/inmunología , Adolescente , Adulto , Glucemia/metabolismo , Proteína C-Reactiva/inmunología , Estudios de Casos y Controles , Colesterol/metabolismo , Calostro/metabolismo , Complemento C3/inmunología , Complemento C4/inmunología , Estudios Transversales , Grasas/metabolismo , Femenino , Glucosa/metabolismo , Humanos , Inmunoglobulinas/inmunología , Obesidad/metabolismo , Sobrepeso/inmunología , Sobrepeso/metabolismo , Embarazo , Complicaciones del Embarazo/metabolismo , Proteínas/metabolismo , Triglicéridos/metabolismo , Adulto Joven

RESUMEN

INTRODUCTION: Mannose binding lectin (MBL) has been linked to predisposition to systemic lupus erythematosus (SLE) and to disease activity. Some studies found deposits of MBL in glomerular tissue of patients with lupus nephritis. There is no research about the deposition of MBL in skin. MATERIALS AND METHODS: Skin biopsies from lesional and non lesional skin of 4 discoid lupus erythematosus (DLE) and 10 SLE patients were submitted to immunofluorescence staining for IgG, IgA, IgM, C3, C4, C1q, C5b-9 and MBL. Charts were reviewed for demographic, clinical and serological data. Patients with SLE had disease activity measured by SLEDAI. RESULTS: MBL was found only in SLE lesional skin and its presence showed an association trend towards higher disease activity. Deposition of C5b-9 occurred in vessels only in patients with SLE (70%) and in the two patients with kidney involvement. CONCLUSIONS: MBL deposition was found in the lesional skin of SLE patients but not in SLE non lesional skin nor in DLE patients, and it seems to be less frequent and less strong than observed in the kidneys biopsies, suggesting that the complement participation in the pathophysiology of SLE process may not be the same in these two clinical manifestations.

Asunto(s)

Lupus Eritematoso Discoide/patología , Lupus Eritematoso Sistémico/patología , Lectina de Unión a Manosa/genética , Piel/patología , Adulto , Complemento C1q/genética , Complemento C1q/inmunología , Complemento C3/genética , Complemento C3/inmunología , Complemento C4/genética , Complemento C4/inmunología , Complejo de Ataque a Membrana del Sistema Complemento/genética , Complejo de Ataque a Membrana del Sistema Complemento/inmunología , Femenino , Expresión Génica , Humanos , Inmunoglobulinas/genética , Inmunoglobulinas/inmunología , Lupus Eritematoso Discoide/genética , Lupus Eritematoso Discoide/inmunología , Lupus Eritematoso Sistémico/genética , Lupus Eritematoso Sistémico/inmunología , Masculino , Lectina de Unión a Manosa/inmunología , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Piel/inmunología

RESUMEN

The complement system is the first line of defence against pathogen infection and can be activated by the classic, alternative and lectin pathways. Trypanosoma cruzi, the causative agent of Chagas disease, has to evade complement system killing and invade the host cells to progress in infection. T. cruzi infectious stages resist complement-mediated killing by expressing surface receptors, which dissociate or prevent C3 convertase formation. Here, we present the first evidence that T. cruzi activates the complement lectin pathway. We detected rapid binding of mannan-binding lectin, H-ficolin, and L-ficolin to the surface of T. cruzi, and found that serum depleted of these molecules failed to kill parasites. Furthermore, lectin pathway activation by T. cruzi required the MBL-associated serine protease 2 (MASP2) activity resulting in C2 factor cleavage. In addition, we demonstrate that the infectious stage of T. cruzi inhibits the lectin pathway activation and complement killing expressing the complement C2 receptor inhibitor trispanning (CRIT) protein. Transgenic parasites overexpressing CRIT were highly resistant to complement-mediated killing. CRIT-derived peptides inhibited both C2 binding to the surface of T. cruzi and parasite killing. Biochemical studies revealed that the CRIT extracellular domain 1 inhibits MASP2 cleavage of C2 factor and thereby impairs C3 convertase formation. Our findings establish that the complement lectin pathway recognizes T. cruzi and provide molecular insights into how the infectious stage inhibits this activation to resist complement system killing.

Asunto(s)

Lectina de Unión a Manosa de la Vía del Complemento/inmunología , Trypanosoma cruzi/citología , Trypanosoma cruzi/inmunología , Animales , Muerte Celular , Membrana Celular/metabolismo , Complemento C2/inmunología , Complemento C4/inmunología , Glicosilación , Humanos , Lectinas/sangre , Lectinas/metabolismo , Estadios del Ciclo de Vida , Lectina de Unión a Manosa/metabolismo , Serina Proteasas Asociadas a la Proteína de Unión a la Manosa/inmunología , Polisacáridos/metabolismo , Unión Proteica , Estructura Terciaria de Proteína , Receptores de Superficie Celular/química , Receptores de Superficie Celular/metabolismo , Proteínas Recombinantes/metabolismo , Suero , Trypanosoma cruzi/crecimiento & desarrollo , Ficolinas

RESUMEN

Complement activation and C3 deposition on the surface of parasitic helminths may be important for recruitment of leukocytes and for damage to the target organism via cell-mediated mechanisms. Inhibition of complement activation would therefore be advantageous to parasites, minimizing damage and enhancing migration through tissues. The aim of this study was to determine ex vivo if complement activation by, and leukocyte adherence to, the nematode Nippostrongylus brasiliensis change as the parasite matures and migrates through the murine host. Pathways of activation of complement and the mechanism of adherence of leukocytes were also defined using sera from mice genetically deficient in either C1q, factor B, C1q and factor B, C3, or C4. Substantive deposition of C3 and adherence of eosinophil-rich leukocytes were seen with infective-stage (L3) but not with lung-stage (L4) larvae. Adult intestinal worms had low to intermediate levels of both C3 and leukocyte binding. For L3 and adult worms, complement deposition was principally dependent on the alternative pathway. For lung-stage larvae, the small amount of C3 detected was dependent to similar degrees on both the lectin and alternative pathways. The classical pathway was not involved for any of the life stages of the parasite. These results suggest that in primary infections, the infective stage of N. brasiliensis is vulnerable to complement-dependent attack by leukocytes. However, within the first 24 h of infection, N. brasiliensis acquires the ability to largely avoid complement-dependent immune responses.

Asunto(s)

Activación de Complemento/inmunología , Leucocitos/citología , Leucocitos/fisiología , Nippostrongylus/fisiología , Infecciones por Strongylida/inmunología , Infecciones por Strongylida/parasitología , Animales , Adhesión Celular , Complemento C1q/inmunología , Complemento C3/inmunología , Complemento C4/inmunología , Factor B del Complemento/fisiología , Ratones , Mutación , Nippostrongylus/inmunología

RESUMEN

OBJECTIVE: To evaluate whether oral supplementation with arginine affects the humoral and innate immune response after vaccination against Streptococcus pneumoniae in a group of people aged 60 y and older, free-living in the community. DESIGN: A randomized controlled trial with one supplemented group and one control group. SETTING: Older persons living in the community. SUBJECTS: A total of 29 adults aged 60 y and older. INTERVENTIONS: The older people were randomized into two groups, one with arginine supplementation (15 g/day) for 4 weeks after pneumococcal vaccine. The control group received only the vaccine. Anthropometric measurements and immune system function parameters: neutrophil chemotaxis and phagocytosis, natural killer cell activity, determination of serum pneumococcal polysaccharide antibodies and serum C3 and C4. RESULTS: Neutrophil phagocytosis and the serum concentration of complement (C3 and C4) did not differ between groups. IgG antibodies against pneumococcal polysaccharide serotypes 1, 5 and 6B increased in both groups. The following parameters increased in the arginine-supplemented group compared to the nonsupplemented group: neutrophil chemotaxis (34 vs 19 units of migration, P = 0.002), natural killer cell cytotoxicity (23.3 vs 13.4 10 M/Ul 40%, P = 0.011) and IgG against antigen 5 (12.3 vs 6.2 mug/ml, P = 0.044). CONCLUSIONS: This study suggests that, after the pneumococcal vaccine, the intake of arginine increased neutrophil chemotaxis, natural killer cytotoxicity and serum concentration of IgG against antigen 5 in older people. These results suggest that arginine supplementation may enhance the immune response elicited by the pneumococcal vaccine in older people. SPONSORSHIP: Supported in part by CAPES and FAEPA.

Asunto(s)

Anticuerpos Antibacterianos/inmunología , Formación de Anticuerpos/efectos de los fármacos , Arginina/farmacología , Inmunidad Innata/efectos de los fármacos , Vacunas Neumococicas/inmunología , Administración Oral , Anciano , Anciano de 80 o más Años , Anticuerpos Antibacterianos/sangre , Formación de Anticuerpos/fisiología , Arginina/administración & dosificación , Quimiotaxis/efectos de los fármacos , Complemento C3/inmunología , Complemento C4/inmunología , Suplementos Dietéticos , Femenino , Humanos , Inmunidad Innata/fisiología , Inmunoglobulina G/sangre , Células Asesinas Naturales/efectos de los fármacos , Masculino , Persona de Mediana Edad

RESUMEN

This study was undertaken to determine if Brazilian Pemphigus foliaceus (BPF) autoantibodies will fix complement as do P. vulgaris (PV) autoantibodies. When sections of human skin were examined following indirect immunofluorescence (IF) staining, BPF autoantibodies reacted with the upper layers of epidermis, while PV autoantibodies were reactive with the lower layers. When tissue culture epidermal cells were used for indirect IF, BPF autoantibodies were not detected after plating until 24 h, while PV autoantibodies reacted within 18 h of plating. Using complement IF staining methods, BPF autoantibodies were found to fix C1q, C4 and C3 to whole skin sections in an intercellular pattern and to the surface of cultured keratinocytes. Although reactive with different epidermal cell surface antigens, autoantibodies in BPF will fix complement in a fashion similar to autoantibodies in PV.

Asunto(s)

Autoanticuerpos/inmunología , Pénfigo/inmunología , Animales , Células Cultivadas , Enzimas Activadoras de Complemento/inmunología , Complemento C1/inmunología , Complemento C1q , Complemento C3/inmunología , Complemento C4/inmunología , Pruebas de Fijación del Complemento , Células Epidérmicas , Técnica del Anticuerpo Fluorescente , Humanos , Inmunoglobulina G/inmunología , Queratinas , Ratones , Ratones Endogámicos BALB C , Piel/inmunología