Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography.

BACKGROUND: Usual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable usual interstitial pneumonia pattern on HRCT who were subjected to surgical lung biopsy. METHODS: HRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded. RESULTS: A total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%) followed by idiopathic pulmonary fibrosis (24.0%), interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%) and familial interstitial lung disease (10.0%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and fibroblastic foci on surgical lung biopsy had a high risk of death. CONCLUSIONS: The most common disease associated with a probable usual interstitial pneumonia pattern on HRCT is fibrotic hypersensitivity pneumonitis followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequencies of usual interstitial pneumonia and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indications for surgical lung biopsy.

Neurofibromatosis type 1: State-of-the-art review with emphasis on pulmonary involvement.

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant dysplasia of the ectoderm and mesoderm with a variable clinical expression, but near-complete penetrance before the age of 5 years. The estimated incidence is 1 in 3000 births. NF-1 is characterized by collections of neurofibromas, café-au-lait spots, axillary and inguinal freckling, and pigmented hamartomas in the iris (Lisch nodules). Pulmonary manifestations of NF-1, which usually include bilateral basal reticulations and apical bullae and cysts, are reported in 10-20% of adult patients. Clinically, neurofibromatosis-associated diffuse lung disease (NF-DLD) usually presents with nonspecific respiratory symptoms, including dyspnea on exertion, shortness of breath, and chronic cough or chest pain, at the time of diagnosis. Computed tomography (CT) is highly accurate for the identification and characterization of NF-DLD; it is the most reliable method for the diagnosis of this lung involvement. Various CT findings of NF-DLD, including cysts, bullae, ground-glass opacities, bibasilar reticular opacities, and emphysema, have been described in patients with NF-1. The typical CT pattern, however, is characterized by upper-lobe cystic and bullous disease, and basilar interstitial lung disease. Currently, the goal of NF-DLD treatment is the earliest possible diagnosis, focusing on symptom relief and interventions that positively alter the course of the disease, such as smoking cessation. The aim of this review is to describe the main clinical, pathological, and imaging aspects of NF-1, with a focus on pulmonary involvement.

Histoplasmosis pulmonar aguda / Acute pulmonary histoplasmosis

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Reversed halo sign: high-resolution CT scan findings in 79 patients.

BACKGROUND: The purpose of this study was to evaluate the high-resolution CT (HRCT) scan findings of patients with the reversed halo sign (RHS) and to identify distinguishing features among the various causes. METHODS: Two chest radiologists reviewed the HRCT scans of 79 patients with RHS and determined the CT scan findings by consensus. We studied the morphologic characteristics, number of lesions, and presence of features associated with RHS. RESULTS: Forty-one patients presented with infectious diseases (paracoccidioidomycosis, TB, zygomycosis, invasive pulmonary aspergillosis, Pneumocystis jiroveci pneumonia, histoplasmosis, cryptococcosis), and 38 presented with noninfectious diseases (cryptogenic organizing pneumonia, pulmonary embolism, sarcoidosis, edema, lepidic predominant adenocarcinoma [formerly bronchiolo-alveolar carcinoma], granulomatosis with polyangiitis [Wegener]). The RHS walls were smooth in 58 patients (73.4%) and nodular in 21 patients (26.6%). Lesions were multiple in 40 patients (50.6%) and single in 39 patients (49.4%). CONCLUSION: The presence of nodular walls or nodules inside the halo of the RHS is highly suggestive of granulomatous diseases.