Patrícia Caetano Mota

Pulmonary rehabilitation in patients with bronchiectasis: pulmonary function, arterial blood gases, and the 6-minute walk test.

BACKGROUND: Information regarding the effects of pulmonary rehabilitation (PR) on pulmonary function (PF), arterial blood gases (ABG), and 6-minute walk distance (6MWD) in patients with bronchiectasis is scant in the literature. METHODS: To evaluate the effects of PR on these indices in this population, a retrospective evaluation of those who attended PR from 2007 to 2010, was made. Pulmonary rehabilitation lasted a mean of 12 weeks and included cycle ergometer exercise for 30 minutes, 3 times per week, with additional upper limbs and quadriceps training. PF, ABG, and 6MWD were evaluated before and after PR to determine the potential influence of gender, exacerbations, underlying cause of bronchiectasis, severity of obstruction, and colonization with bacteria. RESULTS: Forty-one patients (48.8% males; median age, 54 years) were included; 25 had severe obstruction and 19 were colonized with bacteria. Following PR, no significant changes were detected in PF or ABG. Median 6MWD before PR was 425 m and post-PR was 450 m (P = .431). Outcomes did not show any interaction with gender, colonization, or exacerbations. However, patients with idiopathic bronchiectasis did show a significant improvement in forced vital capacity in percent of predicted and residual volume after PR (P = .016 and .048, respectively). Patients with severe obstruction showed a statistically significant decrease in percent of predicted residual volume (P = .025). CONCLUSION: There appears to be a beneficial impact of PR on PF in certain groups of patients with bronchiectasis. In addition, PR indications and protocols for patients with bronchiectasis may need to be adapted to accommodate specific patients, so that expressive exercise capacity improvement can be achieved.

Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare entity, characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. The etiology and pathophysiology are unknown. The prognosis is poor, with no effective therapies other than lung transplantation. IPPFE should be properly identified so that it can be approached correctly. This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE.

Lung and renal transplantation

UNLABELLED Renal transplantation is the most common type of solid organ transplantation and kidney transplant recipients are susceptible to pulmonary complications of immunosuppressive therapy, which are a diagnostic and therapeutic challenge. AIM To evaluate patients admitted to the Renal Transplant Unit (RTU) of Hospital de S. João with respiratory disease. SUBJECT AND METHODS We performed a retrospective study of all patients admitted to RTU with respiratory disease during a period of 12 months. RESULTS Thirty-six patients were included. Mean age 55.2 (+/-13.4) years; 61.1% male. Immunosuppressive agents most frequently used were prednisolone and mycophenolate mofetil associated with ciclosporin (38.9%) or tacrolimus (22.2%) or rapamycin (13.9%). Thirty-one patients (86.1%) presented infectious respiratory disease. In this group the main diagnoses were 23 (74.2%) pneumonias, 5 (16.1%) opportunistic infections, 2 (6.5%) tracheobronchitis, and 1 case (3.2%) of lung abscesses. Microbiological agent was identified in 7 cases (22.6%). Five patients (13.9%) presented rapamycin-induced lung disease. Fibreoptic bronchoscopy was performed in 15 patients (41.7%), diagnostic in 10 cases (66.7%). Mean hospital stay was 17.1 (+/-18.5) days and no related death was observed. CONCLUSION Respiratory infections were the main complications in these patients. Drug-induced lung disease implies recognition of its features and a rigorous monitoring of drug serum levels. A more invasive diagnostic approach was determinant in the choice of an early and more specific therapy.

Acute fibrinous and organizing pneumonia: A report of 13 cases in a tertiary university hospital.

IntroductionAcute fibrinous and organizing pneumonia (AFOP) is a rare diffuse pulmonary disease, but it is not yet known whether it is a distinct form of interstitial pneumonia or simply a reflection of a tissue sampling issue. MethodsCross-sectional evaluation of clinical and radiological findings, treatments, and outcomes for patients with histologically confirmed AFOP at a tertiary university hospital between 2002 and 2015. ResultsThirteen patients (7 women, 53.8%) with a mean ± SD age of 53.5 ± 16.1 years were included. The main symptoms were fever (69.2%), cough (46.2%), and chest pain (30.8%). All patients presented a radiological pattern of consolidation and 5 (38.5%) had simultaneous ground-glass areas. Histology was obtained by computed tomography-guided transthoracic biopsy in 61.5% of cases and by surgical lung biopsy in the remaining cases. Several potential etiologic factors were identified. Eight patients (61.5%) had hematologic disorders and 3 had undergone an autologous hematopoietic cell transplant. Two (15.4%) had microbiologic isolates, 5 (38.4%) had drug-induced lung toxicity, and 2 (15.4%) were classified as having idiopathic AFOP. In addition to antibiotics and diuretics used to treat the underlying disease, the main treatment was corticosteroids, combined in some cases with immunosuppressants. Median survival was 78 months and 6 patients (46.2%) were still alive at the time of analysis. ConclusionOur findings for this series of patients confirm that AFOP is a nonspecific reaction to various agents with a heterogeneous clinical presentation and clinical course that seems to be influenced mainly by the severity of the underlying disorder.

Analysis of sarcoidosis in the Oporto region (Portugal)

BACKGROUND Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal. METHODS A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014. RESULTS We identified 409 patients with sarcoidosis (females, 58.9%; mean age at diagnosis, 38.9±13.4 years; smokers, 14.4%]. All the patients were diagnosed according to the ERS/ATS/WASOG consensus statement and 64.1% had evidence of noncaseating epithelioid cell granulomas in biopsy specimens. Bronchoalveolar lavage was performed as part of the diagnostic work-up in 289 patients and 90.2% had lymphocytosis (CD4/CD8 ratio ≥3.5 in 60.9% of cases). Exertion dyspnea, cough, and constitutional symptoms were the most common presenting symptoms; 10.1% of patients were asymptomatic, 22.8% had Löfgren syndrome, and 50.5% had extrathoracic involvement. Radiographic stages of disease according to the Scadding criteria were as follows: stage 0 (5.2%), stage I (33.7%), stage II (47.0%), stage III (8.4%), and stage IV (5.7%). Impaired respiratory function was observed in 45.6% patients and was mostly mild. Systemic treatment was administered in 58.6% of cases. Overall, 45.3% of patients experienced disease resolution. CONCLUSION The epidemiological and clinical characteristics of this cohort of patients with sarcoidosis from the Oporto region in northern Portugal revealed epidemiological and clinical characteristics that were generally similar to those described in other Western Europe populations and in the US ACCESS study. However, we found a higher proportion of patients who progressed to chronic forms.

Transbronchial cryobiopsy in the diagnosis of desquamative interstitial pneumonia.

Desquamative interstitial pneumonia (DIP) is a rare interstitial pneumonia usually associated with cigarette smoke.1,2 It is characterized by the accumulation of intra-alveolar macrophages, sometimes associated with giant cells.1,3 The diagnosis may be suggested by patchy ground-glass opacification with a predilection for the mid and lower lung lobes on high-resolution computed tomography (HRCT); subpleural involvement is also typical. Irregular lines, traction bronchiectasis, cysts, emphysema, and nodules are other possible findings of DIP.4 Bronchoalveolar lavage fluid nearly always contains an increased number of alveolar macrophages.2,4 Histologically, DIP is characterized by the accumulation of macrophages in the alveolar spaces associated with interstitial inflammation and/or fibrosis. The macrophages usually contain light brown pigment. Lymphoid nodules and a sparse but distinct eosinophilic infiltrate are common.3 Surgical lung biopsy is still required to make a definitive diagnosis.1--3 Transbronchial lung cryobiopsy (TBLC) is a new endoscopic technique that has recently shown superior diagnostic yield to conventional transbronchial biopsy (TBB).5--7 The advantage of the cryoprobe, compared with conventional TBB or TBB using jumbo forceps is that larger pieces of tissue, without crush artifacts, can be extracted during the freeze-thaw cycle, allowing the identification of complex pathologic patterns. The technique permits visualization of peripheral structures of the secondary pulmonary lobule and facilitates immunohistochemical staining. In addition, TBLC can be performed on an outpatient basis and is both an easier and safer procedure for patients with comorbidities, as it reduces the complications and mortality associated with surgical lung biopsy.6 Most of the data available to date is on TBLC overall diagnostic yield and complication rates.5 However, it is also important for clinicians to know the diagnostic accuracy of TBLC in particular diffuse lung diseases, especially in cases in which histologic evaluation is an essential component of

Associations between sarcoidosis clinical course and ANXA11 rs1049550 C/T, BTNL2 rs2076530 G/A, and HLA class I and II alleles.

A genetic background may be responsible for the different clinical courses in sarcoidosis. We analyzed associations between sarcoidosis clinical course and HLA class I/II alleles and susceptibility gene SNPs ANXA11 rs1049550 C/T and BTNL2 rs2076530 G/A in a Portuguese population, investigating possible gene–gene interactions.

Learning curve for transbronchial lung cryobiopsy in diffuse lung disease

INTRODUCTION Transbronchial lung cryobiopsy (TBLC) is increasingly used in the diagnosis of diffuse lung disease (DLD), but no data have yet been published on the learning curve associated with this technique. AIM To evaluate diagnostic yield, lung tissue sample length and area, and procedure-related complications in a cohort of TBLC procedures to define the learning curve and threshold for proficiency. METHODS Retrospective analysis of the first 100 TBLCs performed in different segments of the same lobe in patients with suspected DLD. We compared diagnostic yield, sample length and area, and complications between consecutive groups of patients. RESULTS The overall diagnostic yield for TBLC was 82%. Median sample length was 5.4mm (IQR, 5-6) and median area was 19.5mm2 (IQR, 13.3-25). Pneumothorax was the most common complication (18%). On comparing the two groups of 50 consecutive patients, a significant difference was found for diagnostic yield (74% vs 90%; p=0.04), sample length (5.0mm [2.5-16] vs 6.0mm [4-12;] p<0.01) and area (17.5mm2 [6-42] vs 21.5mm2 [10-49]; p<0.01). Logarithm regression was applied to median diagnostic yield and sample length and area for groups of 10 consecutive patients to define the learning curve, which plateaued after approximately 70 procedures. CONCLUSIONS Our findings suggest that proficiency in TBLC is achieved at approximately the 70th procedure; however they need to be validated in more series and cohorts.

Evaluation of clinical presentation and outcome of patients with respiratory bronchiolitis-associated interstitial lung disease.

. Nickel N, Golpon H, Greer M, Knudsen L, Olsson K, Westerkamp V, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2012;9:589--96. . Foris V, Kovacs G, Tscherner M, Olschewski A, Olschewski H. Biomarkers in pulmonary hypertension: what do we know? Chest. 2013;144:274--83. . Fijalkowska A, Kurzyna M, Torbicki A, Szewczyk G, Florczyk M, Pruszczyk P, et al. Serum N-terminal brain natriuretic peptide as in scleroderma-associated pulmonary hypertension. Eur Heart J. 2006;27:1485--94. 6. Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30: 2493--537.

WITHDRAWN: Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity

This article has been withdrawn for editorial reasons because the journal will be published only in English. In order to avoid duplicated records, this article can be found at http://dx.doi.org/10.1016/j.rppnen.2014.04.008. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.