Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.

Following the disclosure of the results of the study "Impressions of patients, doctors and medical students regarding the appearance of doctors 1 ," some colleagues have called our attention to the increasingly common use of tattoos among medical students, a factor not investigated in that article. Being such skin decoration permanent, we decided to complement the information provided by the previous study with a small research among patients treated at Hospital das Clínicas, FMRP-USP. Sixty patients -20 inpatients and 40 outpatients, 34 female, mean age 55.6 ± 15.4 years -were addressed by the same investigator at all times.A standard questionnaire was used to detect the presence or absence of discomfort regarding seven personal appearance items used by doctors. Due to the results of the original study, four items were included in each list, in addition to the tattoo element, which had been linked to high level of discomfort, and two with minimum degree of discomfort.The percentage of patients who would feel uncomfortable with the items studied was:• for male physicians: Bermuda shorts, 65%; shorts, 65%; facial piercing, 56.7%; tattoo, 46.7%; many rings, • 43.3%; closed shoes, 0%; white coat, 0%.• for female physicians: facial piercings, 58.3%; crop tops, 56.7%; shorts, 50%; tattoo, 43.3%; Bermuda shorts, 41.7%; closed shoes, 0%; white coat, 0%.The data suggest that between 40 and 50% of patients would feel uncomfortable if treated by a medical professional with tattoos. Even though these figures are high, other items of appearance, such as facial piercing and use of shorts, had a higher degree of disapproval. Therefore, we can conclude that the degree of disapproval by patients to the use of tattoos by medical doctors is intermediate, suggesting that contemporary society is more accepting of the use of this ornament.Anyway, these results confirm, once again, the preference of most patients for medical professionals who adopt a more traditional and conservative posture and appearance.

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Agradeço a todos que contribuíram, direta ou indiretamente, para a execução deste trabalho. São inúmeras pessoas que participaram de algum capítulo da minha vida e, dessa forma, me ensinaram tudo o que sei hoje. As páginas dessa tese seriam insuficientes para descrever seus nomes e sua importância. Minha gratidão a vocês será eterna. Um agradecimento especial a todos os professores e mestres que me ensinaram e orientaram desde de o início das primeiras palavras até, finalmente, a confecção desta tese. Minha esperança é que a sociedade, um dia, reconheça seu grande valor. Ao Prof. Dr. José Antônio Baddini Martinez, meu orientador, que cumpriu este papel com muito ânimo e empenho, estando sempre presente e disponível mesmo quando maiores problemas exigiam sua atenção. Ficam aqui documentados minha gratidão, admiração e respeito. Aos docentes, médicos contratados e a todos os funcionários da Seção de Pneumologia, que muito me ensinaram e incentivaram. Ao Prof. Dr. Rodrigo Calado e a equipe do Laboratório de Hematologia (Bárbara, Flávia, Rita e André), que tornaram possível a análise genética deste projeto.