Original Article

Immunohistochemical and morphometric evaluation of COX‑1 and COX-2 in the remodeled lung in idiopathic pulmonary fibrosis and systemic sclerosis

Avaliação imuno-histoquímica e morfométrica de COX-1 e COX-2 no remodelamento pulmonar na fibrose pulmonar idiopática e na esclerose sistêmica

J Bras Pneumol.2013;39(6):692-700

PET/CT and inflammatory mediators in systemic sclerosis-associated interstitial lung disease

PET/TC e mediadores inflamatórios na doença pulmonar intersticial associada à esclerose sistêmica

Andréa L Bastos1, Gilda A Ferreira2, Marcelo Mamede1, Eliane V Mancuzo3, Mauro M Teixeira4, Flávia P S T Santos5, Cid S Ferreira6, Ricardo A Correa3

J Bras Pneumol.2022;48(4):e20210329

Objective: To investigate the correlation of HRCT findings with pulmonary metabolic activity in the corresponding regions using 18F-FDG PET/CT and inflammatory markers in patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). Methods: This was a cross-sectional study involving 23 adult patients with SSc-associated ILD without other connective tissue diseases. The study also involved 18F-FDG PET/CT, HRCT, determination of serum chemokine levels, clinical data, and pulmonary function testing. Results: In this cohort of patients with long-term disease (disease duration, 11.8 ± 8.7 years), a nonspecific interstitial pneumonia pattern was found in 19 (82.6%). Honeycombing areas had higher median standardized uptake values (1.95; p = 0.85). Serum levels of soluble tumor necrosis factor receptor 1, soluble tumor necrosis factor receptor 2, C-C motif chemokine ligand 2 (CCL2), and C-X-C motif chemokine ligand 10 were higher in SSc patients than in controls. Serum levels of CCL2-a marker of fibroblast activity-were correlated with pure ground-glass opacity (GGO) areas on HRCT scans (p = 0.007). 18F-FDG PET/CT showed significant metabolic activity for all HRCT patterns. The correlation between serum CCL2 levels and GGO on HRCT scans suggests a central role of fibroblasts in these areas, adding new information towards the understanding of the mechanisms surrounding cellular and molecular elements and their expression on HRCT scans in patients with SSc-associated ILD. Conclusions: 18F-FDG PET/CT appears to be unable to differentiate the intensity of metabolic activity across HRCT patterns in chronic SSc patients. The association between CCL2 and GGO might be related to fibroblast activity in these areas; however, upregulated CCL2 expression in the lung tissue of SSc patients should be investigated in order to gain a better understanding of this association.

Keywords: Tomography; Lung diseases, interstitial; Scleroderma, systemic; Cytokines.

Idiopathic pulmonary fibrosis and emphysema in smokers

Fibrose pulmonar idiopática simultânea a enfisema em pacientes tabagistas

Denise Rossato Silva, Marcelo Basso Gazzana, Sérgio Saldanha Menna Barreto, Marli Maria Knorst

J Bras Pneumol.2008;34(10):779-786

Histological features and survival in idiopathic pulmonary fibrosis

Achados histológicos e sobrevida na fibrose pulmonar idiopática

Ester Nei Aparecida Martins Coletta, Carlos Alberto de Castro Pereira, Rimarcs Gomes Ferreira, Adalberto Sperb Rubin, Lucimara Sonja Villela, Tatiana Malheiros, João Norberto Stávale

J Bras Pneumol.2003;29(6):371-378

Safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis in Brazil

Segurança e tolerabilidade de Nintedanibe em pacientes com fibrose pulmonar idiopática no Brasil

Carlos Alberto de Castro Pereira1,a, José Antonio Baddini-Martinez2,b, Bruno Guedes Baldi3,c, Sérgio Fernandes de Oliveira Jezler4,d, Adalberto Sperb Rubin5,e, Rogerio Lopes Rufino Alves6,f, Gilmar Alves Zonzin7,g, Manuel Quaresma8,h, Matthias Trampisch9,i, Marcelo Fouad Rabahi10,j

J Bras Pneumol.2019;45(5):e20180414

Evaluation of the histological parameters in usual interstitial pneumonia (idiopathic pulmonary fibrosis)

Avaliação de parâmetros histológicos na pneumonia intersticial usual (fibrose pulmonar idiopática)

Rimarcs Gomes Ferreira, Ester Nei Aparecida Martins Coletta, Osvaldo Giannotti Filho

J Bras Pneumol.2000;26(6):279-285

Interstitial lung disease in patients with progressive systemic sclerosis. A study of 58 cases

Comprometimento do interstício pulmonar em portadores de esclerose sistêmica progressiva. Estudo de uma série de 58 casos

Sergio Fernandes de Oliveira Jezler, Mittermayer Barreto Santiago, Thamine Lessa Andrade, César Araujo Neto, Helio Braga, Álvaro Augusto Cruz

J Bras Pneumol.2005;31(4):300-306

Evaluation of bone disease in patients with cystic fibrosis and end-stage lung disease

Avaliação de doença óssea em pacientes com fibrose cística e doença pulmonar terminal

Cécile A. Robinson1,a, Markus Hofer2,b, Christian Benden1,c, Christoph Schmid3,d

J Bras Pneumol.2019;45(1):e20170280

Objective: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman's rho and multivariate logistic regression analysis were used. Results: A total of 102 adult CF patients were evaluated. The mean age was 28.1 years (95% CI: 26.7-29.5), and the mean body mass index was 17.5 kg/m2 (95% CI: 17.2-18.2). Mean T-scores were −2.3 and −1.9 at the lumbar spine and femoral neck, respectively, being lower in males than in females (−2.7 vs. −2.0 at the lumbar spine and −2.2 vs. −1.7 at the femoral neck). Overall, 52% had a T-score of < −2.5 at either skeletal site. The homozygous Phe508del genotype was found in 57% of patients without osteoporosis and in 60% of those with low BMD. Mean T-scores were not particularly low in patients with severe CFTR mutations. Although the BMI correlated with T-scores at the femoral neck and lumbar spine, serum 25-hydroxyvitamin D and parathyroid hormone levels did not. Conclusions: CFBD is common in CF patients with end-stage lung disease, particularly in males and patients with a low BMI. It appears that CF mutation status does not correlate with CFBD. In addition, it appears that low BMD does not correlate with other risk factors or biochemical parameters. The prevalence of CFBD appears to have recently decreased, most likely reflecting increased efforts at earlier diagnosis and treatment.

Keywords: Lung transplantation; Cystic fibrosis; Bone density; Osteoporosis.

Applicability of the 12-Item Short-Form Health Survey in patients with progressive systemic sclerosis

Aplicabilidade do questionário de qualidade de vida relacionada à saúde - the 12-Item Short-Form Health Survey - em pacientes portadores de esclerose sistêmica progressiva

Thamine Lessa Andrade, Aquiles Assunção Camelier, Fernanda Warken Rosa, Marcia Pina Santos, Sérgio Jezler, Jorge Luiz Pereira e Silva

J Bras Pneumol.2007;33(4):414-422

Review Article

Idiopathic pulmonary fibrosis: current diagnosis and treatment

Fibrose pulmonar idiopática: diagnóstico e tratamento atuais

Alexandre Franco Amaral1, Philippe de Figueiredo Braga Colares1, Ronaldo Adib Kairalla1

J Bras Pneumol.2023;49(4):e20230085

Original Article

Six-minute walk distance and survival time in patients with idiopathic pulmonary fibrosis in Brazil

Distância no teste de caminhada de seis minutos e sobrevida na fibrose pulmonar idiopática no Brasil

Eliane Viana Mancuzo1,2,a, Maria Raquel Soares3,b, Carlos Alberto de Castro Pereira4,c

J Bras Pneumol.2018;44(4):267-272

Pulmonary idiopathic fibrosis: clinical findings and survival in 132 histologically-proven patients

Fibrose pulmonar idiopática: características clínicas e sobrevida em 132 pacientes com comprovação histológica

Adalberto Sperb Rubin, José da Silva Moreira, Nelson da Silva Porto, Klaus Loureiro Irion, Rafael Franco Moreira, Bruno Scheidt

J Bras Pneumol.2000;26(2):61-68

Editorial

Idiopathic pulmonary fibrosis in Brazil: challenges for epidemiological characterization and management

Fibrose pulmonar idiopática no Brasil: desafios para caracterização epidemiológica e abordagem

Bruno Guedes Baldi1

J Bras Pneumol.2017;43(6):401-402

Case Report

Sildenafil for treatment of pulmonary hypertension in association with systemic lupus erythematosus and anti-phospholipid syndrome

Sildenafil no tratamento da hipertensão pulmonar associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio

Eduardo José do Rosário e Souza, Junia Rios Garib, Nasim Michel Garib, Paulo Madureira de Pádua

J Bras Pneumol.2003;29(5):302-304

Original Article

Evaluation of polymerase chain reaction in the diagnosis of pulmonary tuberculosis in indigenous and non-indigenous patients

Avaliação da reação em cadeia da polimerase no diagnóstico da tuberculose pulmonar em pacientes indígenas e não indígenas

Rose Mary Corrêa Santos, Mauricio Morishi Ogusku, José de Moraes Miranda, Maria Cristina Dos-Santos, Julia Ignez Salem

J Bras Pneumol.2006;32(3):234-240

Objective: To evaluate the accuracy of bacteriological methods and of polymerase chain reaction (with primers specific for IS6110 of the Mycobacterium tuberculosis complex) in testing sputum samples from indigenous (Amerindian) and non-indigenous patients. Methods: A total of 214 sputum samples (154 from indigenous patients and 60 from non-indigenous patients) were analyzed in order to determine the accuracy of smear microscopy (direct and concentrated versions) for acid-fast bacilli, culture, and polymerase chain reaction. Results: Both microscopy methods presented low sensitivity in comparison with culture and polymerase chain reaction. Specificity ranged from 91% to 100%, the concentrated acid-fast smear technique being the least specific. Nontuberculous mycobacteria were isolated three times more frequently in samples from indigenous patients than in those from non-indigenous patients. False-positive and false-negative polymerase chain reaction results were more common in the indigenous population. Conclusion: Positivity and isolation of nontuberculous mycobacteria in the acid-fast smear in conjunction with polymerase chain reaction positivity raise the following hypotheses: nontuberculous mycobacteria species with DNA regions homologous to, or even still possessing, the M. tuberculosis IS6110 exist in the Amazon; colonization of the oropharynx or of a tuberculous lesion accelerates the growth of the nontuberculous mycobacteria present in the sputum samples, making it impossible to isolate M. tuberculosis; A history of tuberculosis results in positivity for M. tuberculosis DNA. The absence of bacteriological positivity in the presence of polymerase chain reaction positivity raises questions regarding the inherent technical characteristics of the bacteriological methods or regarding patient history of tuberculosis.

Keywords: Tuberculosis, pulmonary/diagnosis; Mycobacterium tuberculosis, Polymerase chain reaction/methods; Indians, south american