Objective: To present the high-resolution computed tomography (HRCT) findings of pulmonary alveolar microlithiasis. Methods: The HRCT scans of 10 adult patients (seven females and three males; mean age, 38.7 years) were retrospectively analyzed. The films were studied independently by two radiologists. Results: The most common tomographic findings were ground-glass attenuation and linear subpleural calcifications, which were seen in 90% of the patients. Other relevant findings were small parenchymal nodules, calcification along the interlobular septa, nodular cissures, subpleural nodules, subpleural cysts, dense consolidations, and a mosaic pattern of attenuation. Conclusions: The HRCT findings presented by individuals with pulmonary alveolar microlithiasis are distinct. In most cases, such findings can form the basis of the diagnosis, eliminating the need to perform a lung biopsy.

Keywords: Tomography, X-Ray Computed; Lithiasis/lung; Lung diseases.

Alveolar adenoma is a rare benign neoplasm of the lungs, and very few cases have been described in the literature. Patients with alveolar adenoma are frequently asymptomatic and are diagnosed through the accidental discovery of a singular, well-delineated nodule on a routine chest X-ray. The definitive diagnosis is made histologically, and the treatment consists of surgical resection of the nodule..

Keywords: Adenoma; Lung neoplasms; Pulmonary alveoli/pathology; Coin lesion, pulmonary;

Microscopic Polyangiitis is a form of Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated small-vessel vasculitis that preferentially involves venules, capillaries and arterioles and may also involve arteries and veins. It is one of the most common primary systemic small-vessel vasculitis. Its clinical presentation is not distinguishable from the Wegener's granulomatosis (WG) and the Churg-Strauss syndrome (CSS). These types of small-vessel vasculitis are histologically similar and can be differentiated by the presence of granulomatous inflammation in WG or asthma in CSS. The case of a 66-year-old man with microscopic polyangiitis presenting with alveolar hemorrhage is reported with a discussion of the differential diagnosis of other types of pulmonary small-vessel vasculitis.

Keywords: Microscopic polyangiitis. Alveolar hemorrhage. Pulmonary vasculitis

Objective: The aim of this study was to present the evolution of four patients presenting pulmonary alveolar proteinosis and treated at the State University of Londrina School of Medicine. We focus on the importance of whole-lung lavage as the treatment of choice. Methods: A retrospective study of four patients, three females and one male, 22 to 34 years old, presenting similar histories of progressive dyspnea and dry cough. The final diagnosis was established through open-lung biopsy. Three of the patients underwent whole-lung lavage in the Department of Surgery. The procedures were performed under general anesthesia and using a double-lumen endotracheal tube. Results: One patient presented spontaneous regression of the pulmonary alveolar proteinosis without the need for whole-lung lavage. In the other three cases, the number of lavages varied: in one patient, a single unilateral lavage resulted in complete remission of the bilateral process; in another patient, three lavages yielded no significant improvement; in the remaining patient, four lavages provided intervening periods of transient improvement. Conclusion: In the cases evaluated, whole-lung lavage proved an efficient treatment for pulmonary alveolar proteinosis. Although some patients presented a certain resistance to the procedure, it might lead to complete remission of the disease in others.

Keywords: Pulmonary alveolar proteinosis /diagnosis; Bronchoalveolar lavage; Macrophages, alveolar;

Alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including systemic lupus erythematosus, which generally presents as pulmonary-renal syndrome. It is believed that the pathogenesis of microangiopathy is related to deposits of immune complexes that lead to activation of cellular apoptosis. The authors report two cases of alveolar hemorrhage and respiratory failure, both requiring mechanical ventilation. The two cases had opposite outcomes after pharmacological therapy. The presence of anti-glomerular basement membrane antibodies in one of the cases demonstrates the multiplicity of physiopathological mechanisms that may be involved. This multiplicity of mechanisms provides a possible explanation for the heterogeneous responses to the available treatments.

Keywords: Lupus erythematosus systemic/etiology. Lupus nephritis/etiology. Respiratory insufficiency/complications.

A pneumonia por Pneumocystis carinii (PPC), que é a infecção oportunista que mais comumente põe em risco a vida de pacientes com síndrome da imunodeficiência adquirida (SIDA), tem sido há bastante tempo associada a outros estados de imunodeficiência. Os relatos iniciais sugeriram que a apresentação clínica da PPC em SIDA difere de sua apresentação em outras situações, embora ambas tenham sofrido modificações nos últimos anos. O objetivo do presente estudo é comparar os achados clínicos e laboratoriais da PPC em SIDA com os de pacientes portadores de PPC sem SIDA. Foram revisados os prontuários médicos de todos os pacientes em nossa instituição que tiveram o diagnóstico confirmado de PPC e SIDA durante o ano de 1994 (17 pacientes) e sem SIDA no período de 1992-1994 (dez pacientes). Quando da apresentação, os pacientes sem SIDA tinham uma duração de sintomas significativamente menor (8,4 ± 7,7 vs 19,5 ± 10,2 dias, p < 0,05), menor PaO2 média a ar ambiente (47 ± 8,2 vs 63 ± 10mmHg, p < 0,05), e mais freqüente infiltrado unilateral no radiograma do tórax (60 vs 6%, p < 0,05). O índice de mortalidade da PPC foi marcadamente maior nos pacientes sem SIDA (40 vs 6%, p < 0,05), e este fato estava associado com retarde no início do tratamento específico nestes pacientes. Devido a seu curso mais agudo, apresentação radiológica atípica e alto índice de mortalidade, concluímos que o diagnóstico de PPC deve ser afastado em pacientes que recebem terapia imunossupressora e que profilaxia anti-PPC deve ser fortemente considerada em pacientes que serão imunossuprimidos.

Keywords: PPC-AIDS. PPC não-AIDS.

Objetivo: Realizar a tradução para a língua portuguesa e a adaptação cultural para uso no Brasil do Quality of Communication Questionnaire (QOC) para pacientes com DPOC em cuidados paliativos. Métodos: Após a aprovação do autor do questionário e do comitê de ética da instituição, a versão original do QOC com 13 itens foi traduzida, de forma independente, por dois tradutores brasileiros com fluência na língua inglesa. Uma síntese das duas traduções foi realizada por uma médica bilíngue e os dois tradutores, que chegaram a um consenso, gerando uma versão na língua portuguesa. Essa versão foi retraduzida por dois tradutores nativos de países de língua inglesa com fluência na língua portuguesa. Para resolver quaisquer discrepâncias, um comitê de especialistas comparou a versão original com todas as versões produzidas e, assim, obteve-se a versão pré-final do QOC. O pré-teste foi aplicado em 32 pacientes internados em UTIs de três hospitais públicos da grande Florianópolis (SC) para avaliar a clareza e a aceitabilidade cultural da versão pré-final do QOC. Resultados: A média de idade dos pacientes foi de 48,5 ± 18,8 anos. A maioria dos itens foi bem compreendida e aceita, recebendo pontuações ≥ 8. Um item, sobre morte, suscitou dificuldades na compreensão dos participantes do pré-teste. Ao submeter a retradução ao autor do QOC, ele solicitou alterações em dois itens, que foram acatadas. Após essas alterações, a versão final foi aprovada. Conclusões: A tradução e a adaptação cultural do QOC para uso no Brasil foi concluída com sucesso.

Keywords: Doença pulmonar obstrutiva crônica; Inquéritos e questionários; Comunicação; Unidades de terapia intensiva.

Pulmonary alveolar microlithiasis (PAM) is a rare disease that affects both lungs. It is characterized by the presence of small calculi (calcium phosphate) within the alveolar spaces. We report the case of a 26-year-old female whose diagnosis was based on characteristic findings on chest X-rays and high-resolution computed tomography scans. The patient, 28 weeks pregnant, was rehospitalized 10 months after the diagnosis, presenting hypoxemic acute respiratory failure and severe restrictive ventilatory defect on spirometry. After 32 completed weeks of gestation (228 days), she was submitted to cesarean section, and the outcome was successful for mother and newborn. PAM has a variable clinical course. It is suggestive of an autosomal recessive inheritance pattern and has been associated with positive family history. The etiology of PAM is unclear, and many authors speculate that there is a local enzymatic defect responsible for the intra-alveolar accumulation of calcium. Reports of patients with PAM who become pregnant are exceptional, and this is the first case described in Brazil. The course of this disease is usually slow and progressive, and patients typically die of cardiorespiratory failure. The present case illustrates the need to offer female patients, especially those with advanced disease, genetic counseling and orientation regarding the risks of pregnancy. Currently, the only effective therapy is lung transplantation.

Keywords: Pulmonary alveoli; Pregnancy; Respiratory insufficiency.

A 27 year-old female patient, cocaine user, presenting hemoptysis and progressive dyspnea with onset 48 hours prior to hospital admission, without any other signs or symptoms. Serum tests for infectious diseases, collagen disorders and vasculitis were negative. Urinalysis was type I. Computed tomography of the chest showed diffuse alveolar infiltrate, affecting mainly the lower left lobe. A thoracoscopic lung biopsy was performed to clarify the diagnosis. The histopathological findings showed capillaritis and diffuse intra-alveolar hemorrhage. Treated with steroid and cyclophosphamide pulse therapy, a good clinical and radiographical response was obtained. The recently described pauci-immune pulmonary capillaritis is characterized by the presence of isolated pulmonary capillaritis and negative serum testing for auto-immune diseases.

Keywords: Hemorrhage. Pulmonary alveoli/physiopathology.

Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.