A. Morais

Pneumonia aguda fibrinosa e organizante

The term Acute Fibrinous and Organizing Pneumonia (AFOP) has been proposed by Beasley et al for cases that not fit into the histopathologic criteria of the recognized entities described as acute or subacute clinical presentations. The presence of intra-alveolar fibrin in the form of fibrin ‘balls’ and organizing pneumonia with patchy distribution are the main histological features of this entity. We describe the case of a male patient with the diagnostic of AFOP made by surgical lung biopsy. He had a subacute presentation of symptoms consisting of productive cough, chest pain and fever. Bilateral infiltrates with patchy and diffuse distribution were the predominant features in his chest HRCT scan. The patient had a good clinical course after a treatment with prednisone and cyclophosphamide. Our hope in reporting this case study is to add some more data to the discussion of this new entity. Rev Port Pneumol 2006; XII (5): 615-620

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.

Toxicidade pulmonar induzida pela rapamicina

Resumo As doencas pulmonares induzidas por farmacos constituem uma causa crescente de morbilidade, tendo sido descritas diferentes formas de toxicidade associadas a inumeras substâncias. O sirolimus (rapamicina) e um farmaco imunossupressor usado de forma crescente no contexto do transplante de orgaos solidos, nomeadamente no transplante renal. A toxicidade pulmonar tem sido descrita como um dos potenciais efeitos laterais, nomeadamente causando formas de pneumonite intersticial ou, mais raramente, hemorragia alveolar. Os autores descrevem os casos de quatro doentes (3 do sexo masculino, 1 do sexo feminino) com idades compreendidas entre os 46–71 anos, recipientes de transplante renal (rim cadaver) ha 3 anos (1 doente) e 7 anos (3 doentes). A imunosupressao consistia em micofenolato mofetil, prednisolona e rapamicina. Os quatro doentes foram admitidos por febre, tosse produtiva (2) e dispneia (3). Apresentavam imagem radiologica de infiltrados pulmonares bilaterais de predominio basal. O LBA mostrou alveolite linfocitica em 3 doentes, tendo-se observado no entanto diferentes relacoes CD4/CD8., para alem de neutrofilia em 2 deles. No restante doente, observou- se hemorragia alveolar grave. Nao houve em nenhum dos casos qualquer isolamento de micro organismos patogenicos no LBA. As queixas apresentadas, bem como as alteracoes radiologicas regrediram com a suspensao do farmaco. Estes quatro casos revelaram alguma variedade, quer na apresentacao clinica, quer nos achados dos exames subsidiarios efectuados, nomeadamente no LBA. Este facto pode ter como causa diferentes mecanismos fisiopatologicos a nivel do pulmao induzidos pelo sirolimus.

Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica

Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2-3 years after diagnosis. The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients.

Azitromicina como terapêutica adjuvante na pneumonia organizativa criptogénica

There are literature data about the immunomodulatory properties of some macrolides in cryptogenic organizing pneumonia (COP) as an alternative to corticosteroids in mild disease or as adjuvant to standard therapy. A sixty-year-old female, with a controlled intrinsic asthma, presented with COP and recurrent respiratory exacerbations despite corticosteroid and immunossupressant therapy. Azithromycin (500mg, on alternate days) as an adjuvant to steroids was then started, with clinical and functional improvement and regression of lung infiltrates. Withdrawal of steroids was possible in one year, without evidence of relapse in the next six months. Azithromycin was maintained (three times per week) with no documentation of adverse side effects. This clinical case reinforces the potential role of macrolides anti-inflammatory properties in COP as corticosteroids adjuvant therapy.

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

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.

Chronic granulomatous disease associated with common variable immunodeficiency --- 2 clinical cases

INTRODUCTION Chronic granulomatous disease associated with common variable immunodeficiency (GD-CVID), although well documented, is rare. Granulomatous lesions can affect several organs and are histologically indistinguishable from sarcoidosis. CLINICAL CASES Case 1: A 39-year-old male patient with CVID, asymptomatic although with thrombocytopenia and mediastinal-hilar adenopathies. GD-CVID was diagnosed by bone marrow biopsy. Progressive clinical and radiological improvement was obtained with corticotherapy. Case 2: A 38-year-old male patient with CVID, suffered from asthenia, anorexia, myalgia, lower limbs edemas, and dry cough. He had mediastinal and bilateral hilar adenopathies within which biopsy revealed non-necrotizing granulomatous infiltrate. A spontaneous resolution was detected after 9 months of evolution. CONCLUSION GD-CVID is rare and can mimetize other pathologies, namely, sarcoidosis; it should therefore be publicized and discussed so that it becomes a general clinical knowledge.

Esputo induzido nas doencas pulmonares intersticiais --- Um estudo piloto

INTRODUCTION Induced sputum with hypertonic saline has been suggested as a safer and cheaper alternative to bronchoalveolar lavage for evaluation of patients with interstitial lung diseases (ILD). OBJECTIVE To evaluate the safety and feasibility of sputum induction in ILD and to compare sputum cellular profiles with paired bronchoalveolar lavage fluid results. MATERIAL AND METHODS Twenty patients underwent sputum induction with 4.5% saline within 2 weeks of bronchoalveolar lavage. Total, differential cell counts and cellular viability were assessed. Wilcoxon test and Spearmans rank correlation coefficient were used and a p<0,05 was considered statistically significant. RESULTS From a total of 20 subjects (mean age 49.4±16.4 years, 70% male) a satisfactory sputum sample was obtained in 15 subjects (75%). Induction was stopped in one subject, due to a significant decrease in PEF. The cell profiles for induced sputum and bronchoalveolar lavage fluid (BALF) were different (P <.05), except for eosinophils, and there were no significant correlations between the two methods. Compared to sputum reference values there was an increase of lymphocytes (3.2% vs 0.5%) and eosinophils (1.4% vs 0.0%). Comparing sarcoidosis and hypersensitivity pneumonitis sputum, both diseases had an increase in lymphocytes (4.4 vs 3.9%), with a significant higher neutrophil count in hypersensitivity pneumonitis (65.4% vs 10.6% P <0.05), a finding also seen in BALF. CONCLUSION Induced sputum is feasible and safe in interstitial lung diseases. Although sputum cellular counts are not correlated with bronchoalveolar lavage fluid, sputum cellular profiles may help to distinguish different ILD.