RESUMO
A 74-year-old non-smoker female presented to our attention with a history of dyspnea and cough. CT scan revealed multiple areas of patchy ground glass attenuation associated to a diffuse mosaic oligoemia. Scattered bilateral subcentimetric pulmonary nodules were also present. Patient underwent a surgical lung biopsy. Specimens showed features of diffuse neuroendocrine hyperplasia, microhoneycombing, fibroblast foci. A final diagnosis of diffuse neuroendocrine hyperplasia with obliterative bronchiolitis and UIP was rendered.
Assuntos
Bronquiolite Obliterante/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/diagnóstico por imagem , Nódulos Pulmonares Múltiplos/diagnóstico , Células Neuroendócrinas/patologia , Idoso , Bronquiolite Obliterante/complicações , Tosse/etiologia , Dispneia/etiologia , Feminino , Humanos , Hiperplasia/diagnóstico , Fibrose Pulmonar Idiopática/complicações , Pulmão/patologia , Nódulos Pulmonares Múltiplos/complicações , Tomografia Computadorizada por Raios XRESUMO
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
At present, no studies have evaluated the role of bronchoscopic transbronchial lung biopsy (TBLB) in the diagnosis of diffuse drug-induced lung disease (DILD), and there is no consensus for a definite diagnostic workup approach for this rare clinical entity. The aim of the present study was to evaluate the clinical usefulness of TBLB in diffuse DILD. This study was a retrospective analysis of patients with diffuse DILD, who underwent bronchoscopy. The role of TBLB was assessed to determine whether the histological results are useful for the final diagnosis. Over a 5-yr period, 44 patients underwent bronchoscopy, and all had a bronchoalveolar lavage (BAL). Thirty-three of the 44 patients underwent TBLB (75%), and the results of TBLB were diagnostically helpful in 25 (75.7%) of the procedures. No histopathologic abnormality was identified in 6 (18%) of the 33 patients. In 2 patients (6%) the obtained samples were not adequate, since no lung parenchyma was obtained. No severe complications related to bronchoscopic procedures occurred. In conclusion, TBLB is a safe procedure, and is diagnostically helpful in the majority of cases of patients with diffuse DILD. Histological data obtained by TBLB further corroborate clinical, laboratory, radiologic and BAL results for a definitive diagnosis of diffuse DILD.