Desenvolupament d’un nou model murí humanitzat de pemfigoide ampul•lar obtingut a partir de cèl•lules mare humanes fol·liculars

Resum: El pemfigoid ampul•lar és una malaltia cutània autoimmune. La majoria dels pacients presenten autoanticossos contra proteïnes de la membrana basal de la pell, concretament en contra de la col·làgena XVII, específicament envers el epítop immunodominant, l’NC16A. La patogenicitat dels anticossos ha estat demostrada mitjançant experiments in vitro i in vivo. L’escassa homologia existent entre l’NC16A i el seu homòleg murí (NC14A), ha dificultat l’el·laboració de models animals d’aquesta malaltia. En aquest treball demostrem que el sèrum de pacients amb pemfigoid ampul•lar produeix separació dermo-epidèrmica en pell de ratolí humanitzada obtinguda a partir de cèl•lules mare humanes del provinents fol·licle pil·lós

Epidemiología del vitíligo y patologías asociadas

El nostre estudi consisteix a revisar els patrons i característiques del vitíligo en la població que acudeix a la consulta de Dermatologia del Centre de Salut de Burjassot,estudiar la freqüència d'associació de patologia autoinmune i discriminar la utilitat de sol·licitar una bateria de autoanticossos com screening de malalties autoinmunes en pacients amb vitíligo. És un estudi descriptiu, transversal i no experimental. Es va realitzar entre Gener- Juny 2010 amb 54 pacients. Es va confirmar l'associació amb malalties autoinmunes com a patologia tiroïdal, anèmia perniciosa, diabetis mellitus, malaltia de Addison, etc. sobretot en sexe femení i localització no segmentaria.

Antineutrophil cytoplasmic antibodies in sera from colectomised ulcerative colitis patients and its relation to the presence of pouchitis.

BACKGROUND: Few studies have evaluated the influence of colectomy on antineutrophil cytoplasmic antibody (ANCA) positivity in ulcerative colitis (UC). In small series of patients it has been suggested that ANCA positivity in UC might be predictive for development of pouchitis after colectomy. AIMS: To assess the prevalence of ANCA in UC patients treated by colectomy and a Brooke's ileostomy (UC-BI) or ileal pouch anal anastomosis (UC-IPAA), and the relation between the presence of ANCA, the type of surgery, and the presence of pouchitis. SUBJECTS: 63 UC patients treated by colectomy (32 with UC-BI and 31 with UC-IPAA), 54 UC, and 24 controls. METHODS: Samples were obtained at least two years after colectomy. ANCA were detected by indirect immunofluorescent assay. RESULTS: There were no differences between patients with (36.3%) or without pouchitis (35.0%) and between patients with UC (55%), UC-BI (40.6%), and UC-IPAA (35.4%). However, ANCA prevalence significantly decreases in the whole group of operated patients (38.0%) compared with non-operated UC (p = 0.044). CONCLUSIONS: The prevalence of ANCA in operated patients was significantly lower than in non-operated UC, suggesting that it might be related either to the presence of inflamed or diseased tissue. ANCA persistence is not related to the surgical procedure and it should not be used as a marker for predicting the development of pouchitis.

Outcomes from studies of Antineutrophil Cytoplasm Antibody Associated Vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force.

Objectives: We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in anti-neutrophil cytoplasm antibody associated vasculitis (AAV), and to assess the quality of evidence for outcome measures in AAV. Methods: Using a systematic Medline search, we categorised the identified studies according to diagnoses. Factors affecting remission, relapse, renal function and overall survival were identified. Results: A total of 44 papers were reviewed from 502 identified by our search criteria. There was considerable inconsistency in definitions of end points. Remission rates varied from 30% to 93% in Wegener granulomatosis (WG), 75% to 89% in microscopic polyangiitis (MPA) and 81% to 91% in Churg¿Strauss syndrome (CSS). The 5-year survival for WG, MPA and CSS was 74¿91%, 45¿76% and 60¿97%. Relapse (variably defined) was common in the first 2 years but the frequency varied: 18% to 60% in WG, 8% in MPA, and 35% in CSS. The rate of renal survival in WG varied from 23% at 15 months to 23% at 120 months. Methods used to assess morbidity varied between studies. Ignoring the variations in definitions of the stage of disease, factors influencing remission, relapse, renal and overall survival included immunosuppressive therapy used, type of organ involvement, presence of ANCA, older age and male ender. Conclusions: Factors influencing remission, relapse, renal and overall survival include the type of immunosuppressive therapy used, pattern of organ involvement, presence of ANCA, older age and male gender. Methodological variations between studies highlight the need for a consensus on terminology and definitions for future conduct of clinical studies in AAV.

Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis.

with specific ANA, in particular of the IgG3 isotype, had significantly more severe biochemical and histological disease compared with those who were seronegative. None of the controls was positive.Conclusions: Disease specific ANA are present in the majority of patients with PBC when investigated at the level of immunoglobulin isotype. PBC specific ANA, in particular of the IgG3 isotype, are associated with a more severe disease course, possibly reflecting the peculiar ability of this isotype to engage mediators of damage.