ABSTRACT
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.
Subject(s)
Bronchiolitis Obliterans/diagnosis , Idiopathic Pulmonary Fibrosis/diagnosis , Lung/diagnostic imaging , Multiple Pulmonary Nodules/diagnosis , Neuroendocrine Cells/pathology , Aged , Bronchiolitis Obliterans/complications , Cough/etiology , Dyspnea/etiology , Female , Humans , Hyperplasia/diagnosis , Idiopathic Pulmonary Fibrosis/complications , Lung/pathology , Multiple Pulmonary Nodules/complications , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Autoimmune Diseases/epidemiology , Idiopathic Interstitial Pneumonias/epidemiology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/diagnostic imaging , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/diagnostic imaging , Connective Tissue Diseases/epidemiology , Female , Follow-Up Studies , Humans , Idiopathic Interstitial Pneumonias/diagnosis , Idiopathic Interstitial Pneumonias/diagnostic imaging , Incidence , Male , Middle Aged , Prevalence , Pulmonary Fibrosis/diagnosis , Pulmonary Fibrosis/diagnostic imaging , Pulmonary Fibrosis/epidemiology , Radiography , Retrospective Studies , Smoking/epidemiology , Thyroiditis, Autoimmune/diagnosis , Thyroiditis, Autoimmune/diagnostic imaging , Thyroiditis, Autoimmune/epidemiologyABSTRACT
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.
