RESUMO
We present a case of a 15-year-old girl with bilateral parotid and sub-mandibular salivary gland enlargement as the sole presentation of primary Sjögren's syndrome. The clinical, radiological, immunological and pathological features have been discussed. The relevant literature has been reviewed. To our knowledge this is the only reported case of Sjögren's syndrome presenting as multicystic disease with bilateral major salivary gland involvement.
Assuntos
RNA Citoplasmático Pequeno , Síndrome de Sjogren/complicações , Adolescente , Anticorpos Antinucleares/sangue , Autoanticorpos/sangue , Autoantígenos/imunologia , Feminino , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Imageamento por Ressonância Magnética , Doenças Parotídeas/etiologia , Doenças Parotídeas/imunologia , Fator Reumatoide/sangue , Ribonucleoproteínas/imunologia , Doenças das Glândulas Salivares/etiologia , Doenças das Glândulas Salivares/imunologia , Síndrome de Sjogren/imunologia , Glândula Submandibular/imunologia , Antígeno SS-BRESUMO
Lateralisation was studied in 601 cases of carcinoma of the nasal cavity and paranasal sinuses. Squamous and anaplastic carcinomata and adenocarcinoma had a pronounced left-sided predominance in the ethmoid and to a much lesser extent in the nose. The results of a study of 298 cases of septal defection are invoked in support of the hypothesis that squamous and anaplastic carcinomata, as well as adenocarcinoma, are caused by exogenous factors.
Assuntos
Seio Etmoidal , Seio Frontal , Neoplasias Nasais/patologia , Neoplasias dos Seios Paranasais/patologia , Seio Esfenoidal , Adenocarcinoma/etiologia , Adenocarcinoma/patologia , Carcinoma/etiologia , Carcinoma/patologia , Carcinoma de Células Escamosas/patologia , Carcinoma de Células de Transição/patologia , Seio Etmoidal/patologia , Feminino , Seio Frontal/patologia , Humanos , Masculino , Cavidade Nasal/patologia , Septo Nasal/anormalidades , Neoplasias Nasais/etiologia , Neoplasias dos Seios Paranasais/etiologia , Seio Esfenoidal/patologiaRESUMO
Wegener's granulomatosis (WG) is a necrotising granulomatous disease affecting the upper and/or lower respiratory tracts and is associated with focal glomerulonephritis. Formerly believed to be a multisystem disease, a localised form (LWG) is now recognised as a distinct subtype. We describe 6 cases of LWG with no renal or pulmonary involvement detected at the presentation or during follow-up. The total follow-up period ranged from 3 to 7 years in 5 cases. The diagnosis was based on clinical features, antineutrophil cytoplasmic antibody test and histological findings (necrotising granulomatous vasculitis, epithelioid granulomas with varying degrees of chronic inflammatory cells). All patients responded to standard immunosuppressive treatment. Our cases highlight the predilection of LWG for the head and neck region and hence these patients frequently present in the ENT departments. LWG has been discussed as a subtype of WG with a better prognosis and the previous literature has been reviewed on this subject. A high index of suspicion helped by serology and histology enables an early diagnosis, and commencement of proper treatment can prevent the irreversible destructive lesions.