CLAUDIA GIULI SANTI
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
7 resultados
Resultados de Busca
Agora exibindo 1 - 7 de 7
- Increased serum levels of vascular endothelial growth factor in pemphigus foliaceus patients with erythroderma(2017) MIYAMOTO, D.; SOTTO, M. N.; OTANI, C. S. V.; FUKUMORI, L. M. I.; PEREIRA, N. V.; SANTI, C. G.; MARUTA, C. W.; BURNIER, M. N. N.; REBEIS, M. M.; AOKI, V.Background Erythroderma is a clinical skin syndrome shared by patients with cutaneous disorders of distinct aetiologies as a result of the combined actions of chemokines, adhesion molecules, and cytokines, such as vascular endothelial growth factor (VEGF). Objective To evaluate the profile of serum levels of VEGF and soluble vascular endothelial growth factor receptor 1 (sVEGFR-1) in pemphigus foliaceus (PF) patients with erythroderma. Methods We conducted a retrospective study, which included (i) a chart review of all PF patients from the Autoimmune Blistering Clinic, University of Sao Paulo, Brazil, from January 1991 to December 2014, together with an evaluation of demographic variables, hospitalization duration and complications and (ii) analysis of the circulating VEGF and sVEGFR-1 levels in PF patients with erythroderma by ELISA. The controls included patients with pemphigus vulgaris or psoriasis. Results We observed higher serum VEGF levels in PF patients during erythroderma than during the non-erythrodermic phase. PF patients showed increased serum levels of sVEGFR-1 during the erythrodermic phase in comparison to controls. Interestingly, the sVEGFR-1 and antidesmoglein-1 levels were positively correlated during the non-erythrodermic period. Conclusion Erythroderma, which represents one clinical form of PF, implies more severe outcomes. The circulating levels of VEGF, a potent endothelial activator, are increased in PF patients with erythroderma; this result suggests the contribution of the blood vessel endothelium to the pathogenesis of this clinical syndrome. Interestingly, our findings showed a positive correlation between the sVEGFR-1 and antidesmoglein-1 antibody levels, indicating a suppressive response to VEGF augmentation during the erythrodermic phase of PF.
- Characterization of the humoral and insitu autoantibody profile of scalp involvement in pemphigus(2016) MARAGNO, L.; BUSSATO, W. M. M.; MARUTA, C. W.; FUKUMORI, L. M. I.; SOTTO, M. N.; SANTI, C. G.; AOKI, V.
conferenceObject Comparative analysis of the in situ interleukin 23/T-helper 17 lymphocyte axis cytokine profile in IgA pemphigus, subcorneal pustular dermatosis, and pustular psoriasis(2014) SIGNORELLI, Thais Helena Dias; MARUTA, Celina Wakisaka; SANTI, Claudia Giuli; SOTTO, Mirian Nacagami; DIAS, Natasha Favoretto; ROMITI, Ricardo; AOKI, Valeria- Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus(2012) BARBOSA, Natasha D. Fairbanks; AGUIAR, Lana M. de; MARUTA, Celina W.; AOKI, Valeria; SOTTO, Miriam N.; LABINAS, Glaucia H. O.; PERIGO, Alexandre M.; SANTI, Claudia GiuliBackground: Vulvo-cervico-vaginal involvement has rarely been reported in pemphigus vulgaris (PV) and has not been reported in pemphigus foliaceus (PF). Objectives: We sought to evaluate genital lesions and Papanicolaou (Pap) smears in female patients with PV and PF. Methods: This prospective study includes all consecutive cases of female patients with PV and PF seen from May 2009 to February 2010. Gynecologic examination was performed and Pap smears were collected for cytologic analysis from each patient. Results: A total of 56 patients were given a diagnosis of pemphigus (41 PV and 15 PF). Genital involvement was observed in 9 patients with PV (22%) and the vulva was the most common genital site of involvement. Of these 9 patients, 8 presented with active skin/mucous lesions. Four of 15 patients with PF had genital lesions and vulva was the exclusive site of involvement. Three of 4 patients with PF and genital involvement also showed active cutaneous lesions. Six of 56 patients (5 PV and 1 PF) presented with atypical squamous cells of undetermined significance in Pap smear analysis. Upon further pathologic review, acantholytic cells were seen, confirming the diagnosis of pemphigus. Limitations: A small number of PF cases were studied. Conclusions: Vulvar lesions were the second most frequent site of mucous membrane PV. Herein we report the first case to our knowledge of symptomatic genital lesions in a patient with PF. Moreover, acantholytic cells in Pap smears were found in a patient with PF who was in complete remission off therapy with no clinical genital lesions and no circulating anti-desmoglein-1 and anti-desmoglein-3 autoantibodies. Gynecologic evaluation in patients with pemphigus, including a careful evaluation of Pap smears, should be recommended. (J Am Acad Dermatol 2012;67:409-16.)
- Increased expression of in situ IL-31RA and circulating CXCL8 and CCL2 in pemphigus herpetiformis suggests participation of the IL-31 family in the pathogenesis of the disease(2020) MORAIS, K. L.; MIYAMOTO, D.; ORFALI, R. L.; MARUTA, C. W.; SANTI, C. G.; SOTTO, M. N.; SILVA, L. F. F. da; BRANCO, A. C. C. C.; SATO, M. N.; AOKI, VBackground Pemphigus herpetiformis (PH) is a rare clinical subtype of pemphigus with the presence of urticarial plaques, severe pruritus, rare acantholysis and eosinophilic spongiosis. Objectives The aim of this study was to investigate the influence of IL-31 and pro-inflammatory cytokines/chemokines in the pathogenesis of PH. Methods Twenty-five patients with PH and three groups: pemphigus foliaceus (PF = 14), pemphigus vulgaris (PV = 15) and healthy controls (HC = 20) were selected for this study. The groups were analysed by immunohistochemistry utilizing IL-31, IL-31RA, IL-4, IL-17 and TNF-alpha antibodies. Serum levels of IL-4, IL-13, TNF, CXCL8, CCL5 and CCL2 were evaluated by cytometric bead array. Results Analysis of IL-31 family of PH patients revealed the following findings: (i) Enhanced in situ expression of IL-31 in PH samples, compared to PF and to PV (epidermis); (ii) Cutaneous IL-31RA expression in PH samples was higher than in PF, PV and HC groups (epidermis and dermis); (iii) PF patients that evolved to PH showed significant increased IL-31RA epidermal expression during the PH phase. Profile of pro-inflammatory cytokines (IL-4, IL-17 and TNF-alpha) in PH patients' skin exhibited: (i) Enhanced IL-4 expression, when compared to patients with PF (epidermis and dermis) and with PV (epidermis); (ii) Augmented IL-17 expression than PF and PV patients (epidermis); (iii) Augmented expression of TNF-alpha when compared to PF at the epidermal level. Evaluation of circulating cytokines and chemokines showed higher levels of CXCL8 and CCL2 in PH sera compared to HC group. Conclusions IL-31 and IL-31RA, cytokines related to pruritus, and pro-inflammatory chemokines (CXCL8 and CCL2) seem to exert a role in the pathogenesis of PH. These findings support future studies to clarify the role of IL-31 pathway as a potential therapeutic target for patients with PH.
conferenceObject Increased vascular endothelial growth factor expression in erythrodermic pemphigus foliaceus patients(2016) MIYAMOTO, Denise; OTANI, Camila Sayuri Vicentini; FUKUMORI, Ligia Maria Ichimura; PEREIRA, Naiura Vieira; SOTTO, Mirian Nacagami; MARUTA, Celina Wakisaka; SANTI, Claudia Giuli; REBEIS, Marina Mattos; AOKI, Valeria- Clinical and immunopathological evaluation of epidermolysis bullosa acquisita(2011) DELGADO, L.; AOKI, V.; SANTI, C.; GABBI, T.; SOTTO, M.; MARUTA, C.P>Background. Epidermolysis bullosa acquisita (EBA) is a subepidermal blistering disease with IgG antibodies against collagen VII. The disease is heterogeneous and can lead to significant morbidity. Aim. To characterize the clinical and laboratory profile of patients with EBA from Sao Paulo, Brazil. Methods. In total, 12 patients (mean age 24 years) were analysed for cutaneous and mucosal involvement, laboratory data and response to treatment. Results. Mucosal involvement occurred in 11 of the 12 patients (eyes in 4/12, nose in 4/9, pharynx-larynx in 5/9 and oesophagus in 4/10; 3 patients did not undergo nasopharyngeal examination and 2 paediatric patients did not undergo endoscopy). Using direct immunofluorescence, different patterns of deposits were found at the basement membrane zone: IgG (12/12), IgA (6/12), IgM (4/12), C3 (11/12). Indirect immunofluorescence (IIF) was positive in 6 of 12 patients, and IIF on salt-split skin detected dermal deposition in 10 of 12 patients. Antinuclear antibodies were found in 3 of 12 patients, but none of them fulfilled the criteria for systemic lupus erythematosus. After treatment, total remission was achieved in three patients and partial remission in five (three were maintained on minimal treatment, one on the full treatment and one was able to come off treatment). Two patients were lost to follow-up and the remaining two had disease flares. Complications were mainly mucosal (oesophageal stenosis, laryngeal synechia, symblephara and trichiasis). Conclusions. Mucosal involvement in EBA is a determining factor for disease morbidity. Complete evaluation of the patient, focusing on both cutaneous and extracutaneous sites is essential, as EBA may evolve to refractory disease, severely compromising its outcome.