RESUMO
Rhinoscleroma is a progressive chronic granulomatous disease of the upper respiratory tract that may extend to the tracheobronchial tract. It is common belief that the pathology is determined by Klebsiella Rhinoscleromatis. In the authors' opinion, the infection with Klebsiella Rhinoscleromatis may not represent the only etiopathogenic factor of the disease. Rhinoscleroma is reported in many countries, but has a peculiar social and geographic distribution, in that it assumes an endemic character only in some regions of the Middle East, West Russia, North Africa, Indonesia, Central and South America. In Europe, most of the cases are reported in Poland, Hungary and Romania. In Italy, Rhinoscleroma is almost exclusively located in the southern and island regions. Rhinoscleroma is predominantly reported in rural areas, in the presence of poor socio-economic conditions, which according to many authors would be a co-factor triggering the disease. In this article, the authors review some inconsistencies in etiology, histology and epidemiology of Rhinoscleroma. Based on the overall picture, they propose that intrinsic factors, possibly of genetic origin, may give rise to the disease, and suggest possible lines of research to distinguish between extrinsic and intrinsic factors as determinants for Rhinoscleroma.
Assuntos
Predisposição Genética para Doença , Imunidade Celular/genética , Rinoscleroma/epidemiologia , Rinoscleroma/imunologia , Humanos , Klebsiella pneumoniae , Rinoscleroma/genética , Rinoscleroma/microbiologiaRESUMO
CONTEXT: The sinonasal tract is frequently affected by a variety of nonneoplastic inflammatory disease processes that are often multifactorial in their etiology but commonly have a molecular genetic component. OBJECTIVE: To review the molecular genetics of a variety of nonneoplastic inflammatory diseases of the sinonasal tract. DATA SOURCES: Inflammatory lesions of the sinonasal tract can be divided into 3 main categories: (1) chronic rhinosinusitis, (2) infectious diseases, and (3) autoimmune diseases/vasculitides. The molecular diagnosis and pathways of a variety of these inflammatory lesions are currently being elucidated and will shed light on disease pathogenesis and treatment. CONCLUSIONS: The sinonasal tract is frequently affected by inflammatory lesions that arise through complex interactions of environmental, infectious, and genetic factors. Because these lesions are all inflammatory in nature, the molecular pathology surrounding them is most commonly due to upregulation and down-regulation of genes that affect inflammatory responses and immune regulation.
Assuntos
Doenças Nasais/genética , Aspirina/efeitos adversos , Doenças Autoimunes/genética , Doenças Autoimunes/imunologia , Síndrome de Churg-Strauss/genética , Síndrome de Churg-Strauss/imunologia , Granulomatose com Poliangiite/genética , Granulomatose com Poliangiite/imunologia , Humanos , Infecções/genética , Infecções/imunologia , Leishmaniose/genética , Leishmaniose/imunologia , Hanseníase/genética , Hanseníase/imunologia , Micoses/genética , Micoses/imunologia , Doenças Nasais/imunologia , Rinite/genética , Rinite/imunologia , Rinoscleroma/genética , Rinoscleroma/imunologia , Sarcoidose/genética , Sarcoidose/imunologia , Sinusite/genética , Sinusite/imunologia , Vasculite/genética , Vasculite/imunologiaRESUMO
OBJECTIVE: Respiratory scleroma (RS) is a progressive, chronic, granulomatous disease caused by Klebsiella rhinoscleromatis. There is only one report of RS association with HLA-DQ3. In this study, molecular association of HLA class II and RS was determined. STUDY DESIGN AND SETTING: Nine RS patients and 163 healthy controls were compared. DQA1, DQB1, and DRB1 loci were typed. RESULTS: Statistical analysis demonstrated association between DQB1*0301 and susceptibility to RS (P(c) = 0.004). Haplotype analysis showed an association of DQA1*03011-DQB1*0301 (P = 1.21E-19) and DRB1*0407-DQA1*03011-DQB1*0301 (P = 0.0002). CONCLUSIONS: Results established that DQA1*03011-DQB1*0301 haplotype is a strong risk factor for development of RS.
Assuntos
Antígenos HLA-DQ/genética , Rinoscleroma/genética , Adulto , Mapeamento Cromossômico , Feminino , Frequência do Gene/genética , Predisposição Genética para Doença/genética , Genótipo , Cadeias alfa de HLA-DQ , Cadeias beta de HLA-DQ , Antígenos HLA-DR/genética , Cadeias HLA-DRB1 , Haplótipos , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de RiscoRESUMO
Se presenta una familia con 6 miembros afectados de escleroma. El estudio inmunogenético revela la existencia de un antígeno del complejo principal de histocompatibilidad (HLA) en todos ellos (DQ3), lo que implica inmunidad celular alterada. los niveles de inmunoglobulinas muestran un aumento de la IgG que puede corresponder a alguna alteración de la inmunidad humoral agregada relacionada, ya sea con factores extrínsecos o con factores intrínsecos no determinados por el complejo principal de histocompatibilidad (HLA).
Assuntos
Humanos , Klebsiella pneumoniae/imunologia , Rinoscleroma/genética , Imunoglobulinas/imunologia , Imunoglobulinas/metabolismo , Distúrbios Nutricionais/imunologia , Rinoscleroma/imunologiaRESUMO
Three patients with rhinoscleroma who presented with nasal and pharyngeal symptoms are described. Treatment with ceforanide, a new second-generation cephalosporin with high intrinsic activity against Klebsiella, and possessing a long half-life, was used as outpatient treatment. A total of 120 g of ceforanide was administered over a period of two months. All three patients showed signs of clinical improvement, but only two of three became bacteriologically sterile at the end of two months; one patient relapsed bacteriologically but not clinically, once the drug was discontinued. Second- and third-generation cephalosporins appear to have excellent activity against the causative pathogens of rhinoscleroma. Because of the impracticality of administering these agents parenterally over prolonged periods of time, there is a need for the development of an oral cephalosporin with similar intrinsic activity and beta-lactamase stability. Perhaps the novel beta-lactam antibiotics such as the penems and monobactams, some of which can be administered orally, will answer that need.
