A paracoccidioidomicose (PCM) é uma infecção sistêmica causada pelo fungo Paracoccidioides sp. A doença é endêmica na maior parte dos países da América Latina, sendo os pulmões os órgãos mais afetados. O diagnóstico de PCM é feito com base na história clínica e epidemiológica, e confirmado pela visualização microscópica das estruturas fúngicas do Paracoccidioides a partir de secreções ou tecidos. Por vezes, a apresentação clínica e os achados histopatológicos da PCM simulam os de outras doenças, como a sarcoidose, e ocasionalmente são encontradas nas lesões formas excepcionalmente pequenas do Paracoccidioides, que podem ser confundidas com outros fungos, como o Histoplasma capsulatum. O presente trabalho objetivou identificar e caracterizar os casos que simularam sarcoidose e os que apresentaram formas pequenas de Paracoccidioides dentre os 856 casos de paracoccidioidomicose diagnosticados no Laboratório de Micologia do Complexo Hospitalar Santa Casa de Porto Alegre, no período de 1981 a dezembro de 2013. Foram identificados 8 casos mimetizando a sarcoidose e 12 apresentando formas pequenas de Paracoccidoides. Dos casos que simularam a sarcoidose, todos eram fumantes do sexo masculino, com idades variando entre 27 e 59 anos (média = 42,5 anos) e apresentaram tosse produtiva, infiltrado fibronodular bilateral aos exames de imagem e granulomas sarcoides na biópsia pulmonar, recebendo prednisona como tratamento inicial. O diagnóstico de PCM, nestes casos, foi realizado por meio de cortes histológicos corados ao GMS (n=8), exame direto do escarro (n=2) e imunodifusão (n=4). Dentre os casos que apresentaram formas pequenas do Paracoccidioides, todos eram fumantes, tinham idades variando entre 33 e 68 anos (média = 55,58 anos), 10 eram do sexo masculino, e 10 apresentavam sintomas consistentes com os da PCM (dois pacientes eram assintomáticos). O diagnóstico etiológico, em todos os casos, foi realizado por meio de cortes histológicos seriados corados ao GMS (que revelaram as formas multibrotantes patognomônicas do Paracoccidioides) e corroborado por exame direto do escarro (n=3), imunodifusão (n=6) e cultura (n=1). Com isto, ressalta-se a importância da consideração dos diagnósticos diferenciais da paracoccidioidomicose. Visto que a sarcoidose é um diagnóstico de exclusão, o achado de granulomas epitelioides sem necrose deve incitar a busca ativa por agentes etiológicos com a utilização das colorações de ZN e GMS. E nos casos em que se encontram formas pequenas ou pouco usuais do Paracoccidioides, devem-se realizar cortes histológicos seriados e escalonados corados ao GMS e utilizar técnicas laboratoriais complementares, como imunodifusão e cultura, para assegurar o seu diagnóstico. / Paracoccidioidomycosis (PCM) is a systemic infection caused by the fungus Paracoccidioides sp. The disease is endemic in most Latin American countries, and the lungs are the most affected organs. The diagnosis of PCM is based on clinical and epidemiological features, and confirmed by the microscopic visualization of yeasts of Paracoccidioides from clinical specimens. Sometimes the clinical presentation and histopathological findings of PCM mimic those of other diseases, such as sarcoidosis, and occasionally exceedingly small forms of Paracoccidioides are found in lesions, which can be confused with other fungi, such as Histoplasma capsulatum. This study aimed to identify and characterize the cases that simulated sarcoidosis and those with small forms of Paracoccidioides among the 856 cases of paracoccidioidomycosis diagnosed in the Mycology Laboratory of the Hospital Complex Santa Casa de Porto Alegre, from 1981 to December 2013. 8 cases were identified mimicking sarcoidosis and 12 with small forms of Paracoccidoides. All the cases that mimicked sarcoidosis were male smokers, aged between 27 and 59 years (mean = 42.5 years) and had productive cough, bilateral fibronodular infiltrates on X-ray and sarcoid granulomas in the lung biopsy, receiving prednisone as initial treatment. The diagnosis of PCM in these cases was carried out by histological sections stained with GMS (n = 8), direct examination of sputum (n = 2) and immunodiffusion (n = 4). Among the cases with small forms of Paracoccidioides, all were smokers, aged between 33 and 68 years (mean = 55.58 years), 10 were male, and 10 had symptoms consistent with PCM (two patients were asymptomatic). The etiologic diagnosis in all the 12 cases was made by serial tissue sections stained with GMS (which revealed the multibudding pathognomonic forms of Paracoccidioides) and confirmed by direct examination of sputum (n = 3), immunodiffusion (n = 6) and culture (n = 1). In conclusion, it is emphasized the importance of considering the differential diagnosis of paracoccidioidomycosis. Since sarcoidosis is a diagnosis of exclusion, the finding of epithelioid granulomas without necrosis should encourage the active search for etiologic agents with the use of ZN and GMS stains. And in cases in which small or unusual forms of Paracoccidioides are found, serial histological sections stained with GMS and complementary laboratory techniques such as immunodiffusion and culture should be performed to ensure their diagnosis.

A paracoccidioidomicose (PCM) é uma infecção sistêmica causada pelo fungo Paracoccidioides sp. A doença é endêmica na maior parte dos países da América Latina, sendo os pulmões os órgãos mais afetados. O diagnóstico de PCM é feito com base na história clínica e epidemiológica, e confirmado pela visualização microscópica das estruturas fúngicas do Paracoccidioides a partir de secreções ou tecidos. Por vezes, a apresentação clínica e os achados histopatológicos da PCM simulam os de outras doenças, como a sarcoidose, e ocasionalmente são encontradas nas lesões formas excepcionalmente pequenas do Paracoccidioides, que podem ser confundidas com outros fungos, como o Histoplasma capsulatum. O presente trabalho objetivou identificar e caracterizar os casos que simularam sarcoidose e os que apresentaram formas pequenas de Paracoccidioides dentre os 856 casos de paracoccidioidomicose diagnosticados no Laboratório de Micologia do Complexo Hospitalar Santa Casa de Porto Alegre, no período de 1981 a dezembro de 2013. Foram identificados 8 casos mimetizando a sarcoidose e 12 apresentando formas pequenas de Paracoccidoides. Dos casos que simularam a sarcoidose, todos eram fumantes do sexo masculino, com idades variando entre 27 e 59 anos (média = 42,5 anos) e apresentaram tosse produtiva, infiltrado fibronodular bilateral aos exames de imagem e granulomas sarcoides na biópsia pulmonar, recebendo prednisona como tratamento inicial. O diagnóstico de PCM, nestes casos, foi realizado por meio de cortes histológicos corados ao GMS (n=8), exame direto do escarro (n=2) e imunodifusão (n=4). Dentre os casos que apresentaram formas pequenas do Paracoccidioides, todos eram fumantes, tinham idades variando entre 33 e 68 anos (média = 55,58 anos), 10 eram do sexo masculino, e 10 apresentavam sintomas consistentes com os da PCM (dois pacientes eram assintomáticos). O diagnóstico etiológico, em todos os casos, foi realizado por meio de cortes histológicos seriados corados ao GMS (que revelaram as formas multibrotantes patognomônicas do Paracoccidioides) e corroborado por exame direto do escarro (n=3), imunodifusão (n=6) e cultura (n=1). Com isto, ressalta-se a importância da consideração dos diagnósticos diferenciais da paracoccidioidomicose. Visto que a sarcoidose é um diagnóstico de exclusão, o achado de granulomas epitelioides sem necrose deve incitar a busca ativa por agentes etiológicos com a utilização das colorações de ZN e GMS. E nos casos em que se encontram formas pequenas ou pouco usuais do Paracoccidioides, devem-se realizar cortes histológicos seriados e escalonados corados ao GMS e utilizar técnicas laboratoriais complementares, como imunodifusão e cultura, para assegurar o seu diagnóstico. / Paracoccidioidomycosis (PCM) is a systemic infection caused by the fungus Paracoccidioides sp. The disease is endemic in most Latin American countries, and the lungs are the most affected organs. The diagnosis of PCM is based on clinical and epidemiological features, and confirmed by the microscopic visualization of yeasts of Paracoccidioides from clinical specimens. Sometimes the clinical presentation and histopathological findings of PCM mimic those of other diseases, such as sarcoidosis, and occasionally exceedingly small forms of Paracoccidioides are found in lesions, which can be confused with other fungi, such as Histoplasma capsulatum. This study aimed to identify and characterize the cases that simulated sarcoidosis and those with small forms of Paracoccidioides among the 856 cases of paracoccidioidomycosis diagnosed in the Mycology Laboratory of the Hospital Complex Santa Casa de Porto Alegre, from 1981 to December 2013. 8 cases were identified mimicking sarcoidosis and 12 with small forms of Paracoccidoides. All the cases that mimicked sarcoidosis were male smokers, aged between 27 and 59 years (mean = 42.5 years) and had productive cough, bilateral fibronodular infiltrates on X-ray and sarcoid granulomas in the lung biopsy, receiving prednisone as initial treatment. The diagnosis of PCM in these cases was carried out by histological sections stained with GMS (n = 8), direct examination of sputum (n = 2) and immunodiffusion (n = 4). Among the cases with small forms of Paracoccidioides, all were smokers, aged between 33 and 68 years (mean = 55.58 years), 10 were male, and 10 had symptoms consistent with PCM (two patients were asymptomatic). The etiologic diagnosis in all the 12 cases was made by serial tissue sections stained with GMS (which revealed the multibudding pathognomonic forms of Paracoccidioides) and confirmed by direct examination of sputum (n = 3), immunodiffusion (n = 6) and culture (n = 1). In conclusion, it is emphasized the importance of considering the differential diagnosis of paracoccidioidomycosis. Since sarcoidosis is a diagnosis of exclusion, the finding of epithelioid granulomas without necrosis should encourage the active search for etiologic agents with the use of ZN and GMS stains. And in cases in which small or unusual forms of Paracoccidioides are found, serial histological sections stained with GMS and complementary laboratory techniques such as immunodiffusion and culture should be performed to ensure their diagnosis.

Abstract Reactive oxygen species (ROS) can cause severe damage to cells and organs but they are also important mediators of inflammatory responses and cellular signalling. Due to the significant role of ROS, the cells have evolved a broad antioxidative system to regulate the concentration of these species. Peroxiredoxins (Prxs) are enzymes that participate in the regulation of the cellular redox-homeostasis by detoxifying hydrogen peroxide. Prxs are not classified as conventional antioxidant enzymes and their physiological role, whether protective or regulatory, is still unclear. The aim of this project was to study the localization and regulation of Prxs in normal human lung and also their role in selected lung disorders (pulmonary sarcoidosis, pleural mesothelioma, lung carcinomas and chronic obstructive disorder, COPD). Additionally the expression of thioredoxin (Trx) and thioredoxin reductase (TrxR) was analysed in the lung of smokers and COPD patients. These enzymes are important reductants in cell and Prxs are one of their targets. Lung is an important organ in the field of ROS and antioxidant research since it is especially vulnerable to exogenous oxidative stress caused by pollutants, cigarette smoke and also by high oxygen pressure. The results showed that all six human Prxs were expressed in healthy human lung but in a cell-specific manner. The most prominent expression was detected in the epithelium and in macrophages, the cells most prone to oxidative stress. There were also differences in subcellular locations of Prxs. The expression of Prxs in non-malignant lung diseases (pulmonary sarcoidosis and COPD) and in smoker's lung was very similar with that in normal lung. Higher expression of Prx V and VI was detected in a subpopulation of macrophages sampled from COPD patients' lung. In contrast, Trx expression was induced in the bronchial epithelium of smoker's lung. Differences in the expression compared to normal lung were seen in lung malignancies (pleural mesothelioma and lung carcinomas). Interestingly, different Prxs were highly expressed in different types of carcinomas. In pleural mesothelioma, all Prxs except Prx IV were highly expressed when compared to normal pleura, in adenocarcinoma Prxs I, II, VI and especially IV, and in squamous cell carcinoma Prxs I, II and IV were upregulated. Tests performed on cultured cells in vitro revealed only a minor increase in the Prx expression after severe oxidant stress in malignant lung cell line originating from alveolar type II pneumocytes (A549) or non-malignant cell line derived from bronchial epithelium. None of the tested growth factors or cytokines affected Prx expression or oxidation state, but severe oxidant stress influenced remarkably the oxidation state of the Prxs.

Sarcoidosis is a multisystem granulomatous disease commonly involving the lungs and mediastinal lymph nodes with the exact etiology being unclear. The simultaneous presence of malignant disease such as breast cancer and sarcoidosis has been reported. Sarcoidosis preceding a diagnosis of malignancy and that occurring years after treatment of malignant disease has been noted in the past. The presence of sarcoidosis in the setting of malignant disease carries a high risk of misdiagnosis. In this article, we report the case of a 45-year-old female with stage IA invasive ductal carcinoma of left breast that was in remission for 2 years; however, radiological imaging including magnetic resonance imaging of thoracic spine and positron emission tomography–computed tomography scanning were highly suspicious for malignant disease metastasis versus lymphoma with the widespread lymphadenopathy. Multiple tissue biopsies with histopathological evaluation allowed us to definitively exclude malignant disease metastasis and to correctly diagnose her atypical presentation of sarcoidosis.

Sarcoidosis is a disease characterized as noncaseation granulomas. Granulomas are clusters of cells that form a discrete nodule. This research was important because Black American women develop saroidosis at a higher rate than any other race. The purpose of this phenomenological qualitative study was to examine the impact of sarcoidosis in the lives of Black American women diagnosed with the disease and to consider how occupational experiences may have contributed to participants' development of sarcoidosis. Research states that domestic work such as cleaning, when performed on a daily basis or as an occupation, can contribute to adverse health effects. The framework of this study utilized the transtheoretical model of behavior change while the overall research questions centered on the effects of sarcoidosis on the quality of life of Black American women. This qualitative research included interviews with thirteen Black American women diagnosed and living with sarcoidosis at various stages. Data were collected using the software tool HyperRESEARCH. Both purposive sampling and snowball sampling technique was used for this research. Data were gathered using a general profile of the lived experiences of women with sarcoidosis. The findings revealed that the common lived experience that has potentially put Black American women at risk for developing sarcoidosis is bleach. My recommendations for further research would be to expand the locations of participants to across the United States. The implications for positive social change may result from broader knowledge of the disease through education, even for those who are not at risk for developing it. Chronic sarcoidosis can be fatal if untreated.

No description available.

Mucosal associated invariant T (MAIT) cells are a population of innate-like lymphocytes, with an emerging role in tuberculosis (TB). They are characterised by the expression of high levels of CD161 and IL-18Rα, possession of a Vα7.2⁺ T cell receptor (TCR), and restriction by the MHC class I-related protein (MR1). MAIT cells can be activated by MR1 presenting microbe-derived riboflavin metabolites; or, by the cytokines IL-12 and IL-18 in a TCR-independent fashion. How human MAIT cells integrate these signals for their activation in response to Mtb is unclear. Lymphatic TB (LNTB) is a common extra-pulmonary manifestation of TB; however, little is known about the status of MAIT cells in LNTB - or in other granulomatous diseases, such as sarcoidosis. In this study, an in vitro approach was used to probe MAIT cell activation by Mtb, and the roles of IL-12/-18, the TCR, cell-cell contact and the immunological synapse (IS). Following TCR ligation, TNFα expression was rapid and transient, and was enhanced following sustained IL-12/-18 exposure. IFNγ expression occurred following sustained exposure to ng/ml concentrations of IL-12/-18; however, alongside TCR stimulation, pg/ml concentrations were sufficient. Using an artificial bilayer system, CD161 was excluded from the central regions of the MAIT cell IS, whilst the distribution of IL-18Rα remained unaffected. In response to Mtb and BCG, MR1 was necessary for rapid activation and TNFα expression, IL-12/-18 were necessary for robust and sustained IFNy expression, whilst an anti-Mtb effect was indicated in an intracellular infection model. Assessment of patients with TB or sarcoid lymphadenopathy revealed a depletion of MAIT cells in the blood in sarcoidosis, but not LNTB. In both groups, MAIT cells could be detected within a proportion of sampled lymph nodes. Overall, these findings indicate the importance of inflammatory cytokine signals in the induction of high-intensity and sustained MAIT cell effector function, including in response to Mtb. The observation of a numerical deficiency of MAIT cells in sarcoidosis requires further investigation.

INTRODUÇÃO: A hipertensão pulmonar (HP) tem impacto prognóstico negativo na sarcoidose. Não foram publicados estudos de rastreamento de HP seguido de confirmação por estudo hemodinâmico da circulação pulmonar entre pacientes ambulatoriais com sarcoidose. OBJETIVOS: 1) verificar a prevalência de HP em pacientes ambulatoriais com sarcoidose; 2) testar a associação do peptídeo natriurético tipo B (BNP) e de dados tomográficos e funcionais pulmonares com HP na sarcoidose e 3) comparar, em pacientes com refluxo tricúspide (VRT) 2,5 m/s, o achado de pressão sistólica da artéria pulmonar (PSAP 40 mmHg) estimada no ecocardiograma com o diagnóstico de hipertensão pulmonar pelo padrão ouro (pressão média da artéria pulmonar PAPm 25 mmHg na avaliação hemodinâmica invasiva). CASUÍSTICA E MÉTODO: 72 dos 163 pacientes do Serviço de Pneumologia do InCor, com diagnóstico de sarcoidose (critérios da American Thoracic Society) realizaram ecocardiograma para mensuração da velocidade do refluxo tricúspide (VRT) e cálculo da PSAP. Pacientes com VRT 2,5 m/s (possível HP) foram submetidos à avaliação hemodinâmica invasiva. Foram realizados também dosagem do BNP sérico, tomografia de tórax de alta resolução e prova de função pulmonar completa. RESULTADOS: 19 pacientes apresentaram VRT 2,5 m/s: 18 realizaram cateterismo e um faleceu antes do procedimento. HP foi diagnosticada em 4 pacientes, com prevalência de 5,6% (IC95% 0,2-10,8%). O valor do BNP sérico foi semelhante nos pacientes com e sem HP (mediana 15,5 vs 11,0 pg/ml, p>0.05). Pacientes com HP tiveram mais alterações tomográficas sugestivas de fibrose (50% vs 4,7%; p=0,04), menor VEF1 (63,7±4,9 vs 85,6±14,8; p=0,02) e tendência a menor CVF (69,1±16,2 vs. 86,7±16,2; p=0,07). A capacidade pulmonar total e a difusão de monóxido de carbono não distinguiram pacientes com e sem HP. Pela estimativa do ecocardiograma, 5 pacientes tiveram PSAP 40 mmHg (6,9%), mas só dois destes pacientes tinham HP (PAPm25 mmHg) no estudo invasivo. O critério PSAP 40 mmHg pela estimativa do ecocardiograma levou a erro diagnóstico em 5 pacientes: 3 falso-positivos e 2 falso-negativos. CONCLUSÃO: A prevalência de HP entre os pacientes ambulatoriais com sarcoidose foi de 5,6%. O BNP não distinguiu pacientes com HP. Pacientes com HP tiveram função pulmonar mais comprometida e mais achados sugestivos de fibrose. A PSAP foi pouco acurado no diagnóstico de HP / BACKGROUND: Pulmonary hypertension (PH) has negative impact in sarcoid patients prognosis. Prevalence of pulmonary hypertension (PH) among sarcoid outpatients has not been investigated by screening studies confirmed by hemodynamic evaluation. OBJECTIVES: (1) to determine the prevalence of PH among sarcoid outpatients in a tertiary center; (2) test whether brain natriuretic peptide (BNP) levels, tomographic findings or pulmonary function tests distinguish patients with and without PH and (3) compare the presence of systolic pulmonary artery pressure estimated by echocardiogram (SPAP 40 mmHg) to the diagnostic gold standard for PH (mean pulmonary artery pressure mPAP 25 mmHg) measured by pulmonary artery catheterization, in patients with tricuspid reflux velocity (TRV) 2.5 m/s. METHODS: Seventy-two of 163 outpatients from InCor-Hospital das Clinicas Pulmonary Division with sarcoidosis (ATS criteria) underwent echocardiographic evaluation to asses TRV and to estimate SPAP. Patients with TRV 2.5 m/s (possible PH) underwent pulmonary artery catheterization. BNP dosage, lung function testing (spirometry, lung volumes by plethismography and single-breath carbon monoxide diffusing capacity DLCOsb) and high-resolution CT (HRCT) also were performed. RESULTS: Nineteen patients had TRV2.5 m/s; 18 underwent hemodynamic evaluation (one patient died before the procedure). PH (mPAP 25 mmHg) was diagnosed in 4 patients and its prevalence was 5.6% (IC95% 0.2-10.8%). Patients with and without PH had similar BNP values (median 15.5 vs 11 pg/ml, p>0.05). Patients with PH had more tomographic findings suggesting pulmonary fibrosis (50% x 4.7%; p=0.04), lower forced expiratory volume in 1st second (63.7±4.9 vs. 85.6±14.8; p=0.02) and a trend to lower forced vital capacity (69.1±16.2 vs. 86.7±16.2;p=0.07). Total lung capacity and DLCOsb values were similar between both groups. Echocardiogram estimated SPAP 40 mmHg was present in 5 patients (6.9%), but only 2 of those had PH (mPAP 25 mmHg). Echocardiogram PASP 40 mmHg misdiagnosed 5 patients: 3 false positive and 2 false negative. CONCLUSION: PH prevalence in sarcoid outpatients was 5.6%. BNP levels did not mash PH patients. PH patients had worse lung function. SPAP estimated by echocardiogram was not accurate to diagnose PH

INTRODUÇÃO: A hipertensão pulmonar (HP) tem impacto prognóstico negativo na sarcoidose. Não foram publicados estudos de rastreamento de HP seguido de confirmação por estudo hemodinâmico da circulação pulmonar entre pacientes ambulatoriais com sarcoidose. OBJETIVOS: 1) verificar a prevalência de HP em pacientes ambulatoriais com sarcoidose; 2) testar a associação do peptídeo natriurético tipo B (BNP) e de dados tomográficos e funcionais pulmonares com HP na sarcoidose e 3) comparar, em pacientes com refluxo tricúspide (VRT) 2,5 m/s, o achado de pressão sistólica da artéria pulmonar (PSAP 40 mmHg) estimada no ecocardiograma com o diagnóstico de hipertensão pulmonar pelo padrão ouro (pressão média da artéria pulmonar PAPm 25 mmHg na avaliação hemodinâmica invasiva). CASUÍSTICA E MÉTODO: 72 dos 163 pacientes do Serviço de Pneumologia do InCor, com diagnóstico de sarcoidose (critérios da American Thoracic Society) realizaram ecocardiograma para mensuração da velocidade do refluxo tricúspide (VRT) e cálculo da PSAP. Pacientes com VRT 2,5 m/s (possível HP) foram submetidos à avaliação hemodinâmica invasiva. Foram realizados também dosagem do BNP sérico, tomografia de tórax de alta resolução e prova de função pulmonar completa. RESULTADOS: 19 pacientes apresentaram VRT 2,5 m/s: 18 realizaram cateterismo e um faleceu antes do procedimento. HP foi diagnosticada em 4 pacientes, com prevalência de 5,6% (IC95% 0,2-10,8%). O valor do BNP sérico foi semelhante nos pacientes com e sem HP (mediana 15,5 vs 11,0 pg/ml, p>0.05). Pacientes com HP tiveram mais alterações tomográficas sugestivas de fibrose (50% vs 4,7%; p=0,04), menor VEF1 (63,7±4,9 vs 85,6±14,8; p=0,02) e tendência a menor CVF (69,1±16,2 vs. 86,7±16,2; p=0,07). A capacidade pulmonar total e a difusão de monóxido de carbono não distinguiram pacientes com e sem HP. Pela estimativa do ecocardiograma, 5 pacientes tiveram PSAP 40 mmHg (6,9%), mas só dois destes pacientes tinham HP (PAPm25 mmHg) no estudo invasivo. O critério PSAP 40 mmHg pela estimativa do ecocardiograma levou a erro diagnóstico em 5 pacientes: 3 falso-positivos e 2 falso-negativos. CONCLUSÃO: A prevalência de HP entre os pacientes ambulatoriais com sarcoidose foi de 5,6%. O BNP não distinguiu pacientes com HP. Pacientes com HP tiveram função pulmonar mais comprometida e mais achados sugestivos de fibrose. A PSAP foi pouco acurado no diagnóstico de HP / BACKGROUND: Pulmonary hypertension (PH) has negative impact in sarcoid patients prognosis. Prevalence of pulmonary hypertension (PH) among sarcoid outpatients has not been investigated by screening studies confirmed by hemodynamic evaluation. OBJECTIVES: (1) to determine the prevalence of PH among sarcoid outpatients in a tertiary center; (2) test whether brain natriuretic peptide (BNP) levels, tomographic findings or pulmonary function tests distinguish patients with and without PH and (3) compare the presence of systolic pulmonary artery pressure estimated by echocardiogram (SPAP 40 mmHg) to the diagnostic gold standard for PH (mean pulmonary artery pressure mPAP 25 mmHg) measured by pulmonary artery catheterization, in patients with tricuspid reflux velocity (TRV) 2.5 m/s. METHODS: Seventy-two of 163 outpatients from InCor-Hospital das Clinicas Pulmonary Division with sarcoidosis (ATS criteria) underwent echocardiographic evaluation to asses TRV and to estimate SPAP. Patients with TRV 2.5 m/s (possible PH) underwent pulmonary artery catheterization. BNP dosage, lung function testing (spirometry, lung volumes by plethismography and single-breath carbon monoxide diffusing capacity DLCOsb) and high-resolution CT (HRCT) also were performed. RESULTS: Nineteen patients had TRV2.5 m/s; 18 underwent hemodynamic evaluation (one patient died before the procedure). PH (mPAP 25 mmHg) was diagnosed in 4 patients and its prevalence was 5.6% (IC95% 0.2-10.8%). Patients with and without PH had similar BNP values (median 15.5 vs 11 pg/ml, p>0.05). Patients with PH had more tomographic findings suggesting pulmonary fibrosis (50% x 4.7%; p=0.04), lower forced expiratory volume in 1st second (63.7±4.9 vs. 85.6±14.8; p=0.02) and a trend to lower forced vital capacity (69.1±16.2 vs. 86.7±16.2;p=0.07). Total lung capacity and DLCOsb values were similar between both groups. Echocardiogram estimated SPAP 40 mmHg was present in 5 patients (6.9%), but only 2 of those had PH (mPAP 25 mmHg). Echocardiogram PASP 40 mmHg misdiagnosed 5 patients: 3 false positive and 2 false negative. CONCLUSION: PH prevalence in sarcoid outpatients was 5.6%. BNP levels did not mash PH patients. PH patients had worse lung function. SPAP estimated by echocardiogram was not accurate to diagnose PH

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, which primarily affects middle-aged women. The liver histology is characterized by inflammation and destruction of the intrahepatic bile ducts as well as a high frequency of granuloma. Although the etiology is unknown, the occurrence of associated multiorganic abnormalities such as Sjogren's syndrome, scleroderma, rheumatic disorders and thyroid gland diseases have been cited as evidence favouring an autoimmune background. Addison and Gull in 1851 described the first patient with jaundice and xanthomatosis. PBC was first mentioned in 1876 as an entity by Hanot. PBC was considered to be a rare disease until in 1973 Sherlock and Scheuer described 100 patients. Since then a greater awareness of the disease combined with a wider use of laboratory screening methods has led to the discovery of an increasing number of patients with PBC. In an epidemiological investigation of PBC in the northern part of Sweden a point prevalence of 151 per 106 was found, which is the highest so far reported, and the mean annual incidence amounted to 13.3 per 106. Asymptomatic PBC was present in more than one third of the patients which is consistent with the finding in other epidemiological investigations and is supposed to explain the higher prevalence of PBC and the better prognosis. Nevertheless 25 patients died during the study period, 14 as a direct consequence of the liver disease. Chronic intrahepatic cholestasis has been reported in sarcoidosis and, moreover, a high frequency of liver granuloma is found. The implication of the present study is that a negative Kveim test in combination with positive mitochondrial antibodies is accurate in differentiating PBC from sarcoidosis. Multisystem involvement is frequently observed in PBC and the present study confirms this. In the prospective investigation of 26 PBC patients 50 % had arthropathy considered to be associated with PBC. Rheumatoid arthritis was found in 5 patients, who all had symptoms of liver disease in addition. Lung function impairment was present in 56% (1 asymptomatic PBC). Most commonly a reduced diffusion capacity was found (36%). Bronchial asthma was present in three patients, and severe lung emphysema in one. Features of Sjogren's syndrome was found in 73% (3 asymptomatic PBC). In 6 patients keratoconjunctivitis sicca (KCS) was evident with the rose bengal test demonstrating corneal staining and a Schirmer test of less than 5 mm. Radiological findings of sialectasia were demonstrated in 6 patients, of whom 5 had KCS as well. The ultimate treatment in PBC is liver transplantation and to calculate the need for that, good epidemiological surveys are needed, and also indicators of hepatocellular function. The present investigation indicates that determination of the von Willebrand factor could be used for this purpose. / <p>Härtill 6 uppsatser</p> / digitalisering@umu