RESUMO
Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly named Churg Strauss Syndrome, is a multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia; it is classified as a vasculitis of the small and medium sized arteries, although the vasculitis is often not clinically apparent in the initial phases of the disease. We present the case of a woman with EGPA who was frequently treated with high dose steroid therapy during hospital admission for refractory asthma. After December 2008, the date when we started Omalizumab, we observed a significative reduction of circulating eosinophils and IgE serum level, and the patient was no more hospitalized for respiratory failure or asthma attacks.
Assuntos
Anticorpos Anti-Idiotípicos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Asma/tratamento farmacológico , Síndrome de Churg-Strauss/tratamento farmacológico , Feminino , Humanos , Imunoglobulina E/sangue , Pessoa de Meia-Idade , OmalizumabRESUMO
Recent evidence suggests that idiopathic nonspecific interstitial pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and some data seem to suggest a possible pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease. After initial review of cases found in the medical records database by searching for the term "NSIP" (n = 63), 37 iNSIP cases were identified, and were re-evaluated using a dynamic integrated multidisciplinary approach. 27 cases with iNSIP were selected for the study. Mean ± sd age at first respiratory symptom was 54.2 ± 8 yrs, 70% were females, and 59% were never-smokers. At follow-up (mean ± sd 59.7 ± 29 months, range 12-138 months), autoimmune diseases occurred in 14 (52%) patients, with seven (26%) cases of autoimmune thyroiditis, six (22%) of undifferentiated connective tissue disease and three (11%) of connective tissue disease. Patients developing autoimmune diseases were older and more frequently never-smoking females. In >50% of patients diagnosed with iNSIP, evidence of autoimmune diseases develops within 2 yrs, suggesting a probable link between the clinical entity of iNSIP and autoimmune disorders.
Assuntos
Doenças Autoimunes/epidemiologia , Pneumonias Intersticiais Idiopáticas/epidemiologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/diagnóstico por imagem , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Incidência , Masculino , Pessoa de Meia-Idade , Prevalência , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/epidemiologia , Radiografia , Estudos Retrospectivos , Fumar/epidemiologia , Tireoidite Autoimune/diagnóstico , Tireoidite Autoimune/diagnóstico por imagem , Tireoidite Autoimune/epidemiologiaRESUMO
The case of a 48-year-old woman in whom focal nodular hyperplasia of the liver developed after busulfan therapy was administered for essential thrombocytosis is described. Focal nodular hyperplasia is a reactive disorder related to a circulation disorder. The close temporal relation between the haematological disease, busulfan treatment and the appearance of hyperplastic diseases of the liver in our patient supports the possibility that the association of the events might not be casual.