Abstract We report the case of a patient with symptomatic pulmonary hypertension (PH) associated with diffuse systemic sclerosis (SSc) whose initial assessment suggested a group 3 (clinical classification) PH. The patient had a history of drugs/toxins consumption, which contributed to the development of intrinsic pulmonary vascular disease. This changed the panorama towards the diagnosis of pulmonary arterial hypertension (PAH), with important therapeutic and prognostic implications. In fact, the excellent clinical, laboratory and hemodynamic response to therapy confirmed the hypothesis of a case of drug-associated PAH (DPAH) in a patient with diffuse SSc and lung disease. Considering the presence of DPAH, it was deemed necessary to assess acute vasoreactivity during right heart catheterization (RHC). If criteria were met, the clinical scenario may change towards a favorable and sustained clinical and hemodynamic response with oral calcium channel blockers. However, the response to inhaled nitric oxide was negative in our patient and the therapeutic strategy with dual oral combination therapy with tadalafil and ambrisentan was continued. After six-months of therapy the patient significantly improved, from a high to a low risk of one-year mortality. PH (PH (SSc classification drugstoxins drugs toxins consumption disease PAH, , (PAH) implications fact drugassociated drug DPAH (DPAH RHC. RHC . (RHC) met blockers However continued sixmonths six months improved oneyear one year mortality (PAH (RHC

Resumo A doença veno-oclusiva pulmonar (DVOP) e a hemangiomatose capilar pulmonar são tipos raros de substratos histopatológicos dentro do espectro da hipertensão arterial pulmonar (HAP) com prognóstico muito ruim. Caracterizam-se por um processo fibroproliferativo generalizado das veias e/ou capilares de pequeno calibre com preservação das veias maiores, resultando em um fenótipo de hipertensão pulmonar pré-capilar. A apresentação clínica é inespecífica e semelhante a outras etiologias de HAP. O diagnóstico definitivo é obtido por meio de análise histológica, embora a biópsia pulmonar não seja aconselhada devido ao maior risco de complicações. No entanto, alguns achados adicionais podem permitir um diagnóstico clínico presuntivo de DVOP, especialmente história de tabagismo, uso de drogas quimioterápicas, exposição a solventes orgânicos (particularmente tricloroetileno), baixa capacidade de difusão do monóxido de carbono (DLCO), dessaturação ao esforço e evidências de doença venosa sem doença cardíaca esquerda no exame de imagem, manifestada por uma tríade clássica de opacidades em vidro fosco, linhas septais, e linfadenopatias. O transplante pulmonar é o único tratamento eficaz e os pacientes devem ser encaminhados no momento do diagnóstico, devido à rápida progressão da doença e ao prognóstico ruim. Apresentamos o caso de um homem de 58 anos com HAP com características de envolvimento venoso/capilar em que a suspeita clínica, o pronto diagnóstico e o encaminhamento precoce para transplante pulmonar foram determinantes para um bom desfecho. venooclusiva veno oclusiva DVOP (DVOP (HAP ruim Caracterizamse Caracterizam se eou ou maiores précapilar. précapilar pré capilar. pré-capilar histológica complicações entanto tabagismo quimioterápicas particularmente tricloroetileno, tricloroetileno , tricloroetileno) DLCO, DLCO (DLCO) imagem fosco septais linfadenopatias 5 venosocapilar venoso desfecho (DLCO

Abstract Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome. venoocclusive veno occlusive PVOD (PVOD (PAH prognosis andor or precapillary pre phenotype etiologies analysis complications However smoking use trichloroethylene, trichloroethylene , trichloroethylene) DLCO, DLCO (DLCO) desaturation imaging opacities lines lymphadenopathies treatment 58yearold yearold 58 year old venouscapillary suspicion outcome (DLCO 5

Introdução: O controlo da tuberculose (TB) em profissionais de saúde passa pelo reconhecimento precoce dos contactos, adopção de medidas de protecção eficazes e despiste dos casos de tuberculose latente (TL) e latente recente. Objectivos: Rastreio sistemático de TB e TL nos profissionais de saúde com contacto com doentes ou funcionários com TB e risco de contágio. Material e métodos: Num hospital com aproximadamente 5400 funcionários, entre Setembro de 2007 e Dezembro de 2008, realizaram-se 13 rastreios de risco de TB continuada. Estes foram constituídos por observação clínica, realização de prova de tuberculina (PT), teste de Quantiferon® -TB Gold (QTF) se PT ≥10mm e <15mm e RX tórax. O diagnóstico foi efectuado de acordo com as recomendações da SPP Resultados: Nos 13 rastreios efectuados foram englobados 792 profissionais (563<img border=0 width=19 height=27 src="http://img/revistas/pne/v16n1/16n1a01sf.jpg">; 229<img border=0 width=22 height=27 src="http://img/revistas/pne/v16n1/16n1a01sm.jpg">). Destes, 75 apresentavam sintomas e 31 alterações radiológicas. A PT foi efectuada em 490 profissionais, sendo <10 mm em 250, ≥10 e <15 mm em 175 (QTF positivo em 36) e ≥15 mm em 65. Dos profissionais rastreados, 280 tinham PT anterior =15 mm, pelo que não foi repetida. Durante o rastreio foi diagnosticado um caso de TB activa num profissional, 408 casos de TL (51,5%) e 42 de TL recente (5,3%). Conclusões: A TB é considerada como doença profissional nos profissionais da saúde. O rastreio dos contactos e dos casos de TL é uma importante ferramenta para o diagnóstico precoce de doença activa e para a identificação dos casos de maior risco de doença futura.

Introduction: Control of tuberculosis (TB) in healthcare workers requires an early recognition of contacts, the adoption of efficient protective measures and screening for cases of latent tuberculosis (TL) and recent TL. Aims: Systematic screening for TB and TL in healthcare workers with contact with patients or other workers with TB and risk of contagion. Material and methods: In a hospital with approximately 5400 workers 13 screenings were performed September 2007- December 2008 due to risk of continued TB. These consisted of clinical observation, tuberculin skin test (PT), Quantiferon®-TB Gold (QTF) test if PT ≥10mm and <15mm and chest X-ray. The diagnoses were performed according to the recommendations of the Portuguese Pulmonolgy Society (SPP). Results: 792 workers (563<img border=0 width=19 height=27 src="http://img/revistas/pne/v16n1/16n1a01sf.jpg">; 229<img border=0 width=22 height=27 src="http://img/revistas/pne/v16n1/16n1a01sm.jpg">) were included in the 13 screenings. Of these, 75 presented symptoms and 31 had radiology abnormalities. PT was performed in 490 workers, with results <10 mm in 250, ≥10 and <15 mm in 175 (QTF positive in 36) and =15 mm in 65. Of the screened workers, 280 had a previous PT =15 mm, and therefore it was not repeated. 1 case of active TB was diagnosed in a worker, as well as 408 cases of TL (51.5%) and 42 cases of recent TL (5.3%). Conclusions: TB is considered an occupational disease in healthcare workers. The screening of contacts and TL cases is an important tool for an early diagnosis of active disease and for identifying the cases with higher risk of future disease.