ERS International Congress 2021: highlights from the Pulmonary Vascular Diseases Assembly.

This article aims to summarise the latest research presented at the virtual 2021 European Respiratory Society (ERS) International Congress in the field of pulmonary vascular disease. In light of the current guidelines and proceedings, knowledge gaps are addressed and the newest findings of the various forms of pulmonary hypertension as well as key points on pulmonary embolism are discussed. Despite the comprehensive coverage of the guidelines for pulmonary embolism at previous conferences, discussions about controversies in the diagnosis and treatment of this condition in specific cases were debated and are addressed in the first section of this article. We then report on an interesting pro-con debate about the current classification of pulmonary hypertension. We further report on presentations on Group 3 pulmonary hypertension, with research exploring pathogenesis, phenotyping, diagnosis and treatment; important contributions on the diagnosis of post-capillary pulmonary hypertension are also included. Finally, we summarise the latest evidence presented on pulmonary vascular disease and COVID-19 and a statement on the new imaging guidelines for pulmonary vascular disease from the Fleischner Society.

Beta-HCG secretion by a pulmonary pleomorphic carcinoma: A case report.

Ectopic secretion of beta-subunit of human chorionic gonadotropin (ß-HCG) in pulmonary pleomorphic carcinoma is remarkably rare. Such unusual ectopic hormone production by lung cancer may be initially misinterpreted as extragonadal choriocarcinoma or germ cell tumor. We report a 56-year-old postmenopausal female, smoker, who presented a 5-month history of progressive dyspnea, dry paroxysmal cough, and significant weight loss. She was referred by a local hospital with the preliminary diagnosis of gestational trophoblastic neoplasia due to a rapidly growing thoracic tumor with persistently elevated serum ß-HCG. Computed tomography of the chest showed a lung mass in the right upper lobe associated with homolateral pleural effusion. Positron emission tomography showed pathological 2-[18F]FDG uptake at the mass lesion. Biopsies were performed. Histological examination described pleomorphic carcinoma with positive immunostaining for ß-HCG. The serum levels of ß-HCG were also elevated indicating ectopic secretion. The patient had rapid clinical deterioration and deceased before chemotherapy initiation. Only a few cases of paraneoplastic ß-HCG secretion have been reported in the literature. Previous studies suggested that the ability to secrete ß-hCG in tumors may correlate to some extent to chemoresistance; thus, it might be useful as a prognosis marker.

Solitary fibrous tumor of the pleura: A giant finding, a benign entity?

Solitary fibrous tumor of the pleura (SFTP) is a rare tumor. The prognosis is relatively good, but up to 20% of the cases are malignant. We presented a case of a large mass of the thorax, showing malignant poor prognosis features. A 73-year-old male, former smoker, presented with a history of dry cough. Chest CT revealed a left bronchopulmonary mass (11 cm × 14 cm) extending from the costal pleura to the left hilum. Histopathologic examination of the transbronchial lung biopsy was consistent with SFTP. A left pneumonectomy was performed due to invasive nature of the lesion. According to features associated to poor prognosis (lung tumor ≥ 10 cm, hipercellularity, abundant intratumor blood vessels and areas of necrosis), and in spite of proliferation index (Ki67+) <1%, the case was discussed in a multidisciplinary meeting, and was assumed to be malignant. At the present time, the patient is his fifth month after surgery, in tight follow up. This case highlights a rare SFTP presenting bad prognosis features, that although completely resected should have long-term follow-up due to the high risk of recurrence.

Tracheobronchopathia Osteochondroplastica: Two Different Facets of a Rare Entity.

Tracheobronchopathia osteochondroplastica (TBPO) is an uncommon benign disease, characterized by osseous or metaplastic cartilaginous nodules in the submucosa of the tracheobronchial tree. TBPO is easy to misdiagnose due to its non-specific clinical manifestation. We describe two cases of TBPO. The first patient was a 57-year-old woman with nocturnal dry cough and wheezing, in whom bronchoscopy revealed small diffuse mucosal irregularities involving the airway until the segmental bronchi. The other patient was a 69-year-old man with progressive worsening dyspnoea and productive cough presenting with severe stenosis of the trachea. Histological examination of both cases was consistent with TBPO. These cases highlight distinct forms of presentation of this rare entity. LEARNING POINTS: Tracheobronchopathia osteochondroplastica (TBPO) can present as a diffuse large airway disease with mild obstructive symptoms or as severe tracheal obstruction.Direct observation by bronchial fibroscopy of lumen protrusions sparing the posterior wall is diagnostic.

Transbronchial lung cryobiopsy in smoking-related interstitial lung diseases.

BACKGROUND: Transbronchial lung cryobiopsy (TBLC) is an emerging technique in the diagnostic approach to diffuse parenchymal lung diseases. However, the role of TBLC in smoking-related Interstitial Lung Diseases (ILDs) is still under discussion. OBJECTIVES: The aim of the present study was to describe our experience with TBLC in diagnostic work-up of patients with smoking-related ILDs. METHOD: We retrospectively reviewed data of patients evaluated in a tertiary hospital ILDs outpatient clinic, who underwent TBLC, from September 2014 to December 2019. TBLC was performed in accordance with the 2018 expert statement from the Cryobiopsy Working Group. RESULTS: Forty-five patients (25 men [55.6%]) with a mean age of 53.9 years [SD, 9.1] were included. The most frequent radiological pattern was ground glass opacity (42 patients). TBLC was performed in different segments of the same lobe in 38 patients and in two lobes in 7 patients. The mean maximal diameter of the samples was 5.2 mm (range, 3-16 mm [SD 2.0]). Pneumothorax occurred in seven patients (15%) and moderate bleeding occurred in one patient. A specific pathological diagnosis was achieved in 43 of 45 patients. The most frequent histopathologic pattern found was desquamative interstitial pneumonia (33 patients), followed by smoking-related interstitial fibrosis (7 patients), respiratory bronchiolitis - ILD (1 patient) and pulmonary Langerhans cell histiocytosis (1 patient). Two patients had alternative diagnosis (Pneumoconiosis and Interstitial Pneumonia with unspecific features) and one patient had normal lung parenchyma. A definitive multidisciplinary team (MDT) diagnosis was reached in 95.5% (43 of 45 cases). Two patients were submitted to additional diagnostic techniques. CONCLUSIONS: The results from this series support TBLC as a safe procedure with a meaningful diagnostic value in the context of a MDT approach of smoking-related ILDs. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (4): e2020013).

Potential roles of marine fungi in the decomposition process of standing stems and leaves of Spartina maritima.

Fungal communities inhabiting live, senescent, and decaying leaf sheaths, stems, and leaf blades of standing plants of Spartina maritima in two Portuguese salt marshes were assessed by morphological identification of fruiting structures and sequence-based identification based on polymerase chain reaction (PCR)-cloning analysis of the internal transcribed spacer (ITS) rDNA. The molecular method enabled identification of infrequent ascomycetes and basidiomycetes (filamentous and yeasts) and the asexual morph of Byssothecium obiones and Phaeosphaeria halima. The occurrence and ecological role of the most frequent fungi on different S. maritima substrates seem to depend on the phase of plant life cycle, and specifically on the availability and microenvironmental conditions of each plant substrate. Specifically, By. obiones, Natantispora retorquens, and Lulworthia sp. 1 were involved in the decay of lower-middle culms, Buergenerula spartinae of middle culms and leaves, P. halima, Phaeosphaeria spartinicola, and Stagonospora sp. 1 of middle-upper leaves, and Mycosphaerella sp. I of upper leaves of early-decaying S. maritima plants. The presence of these fungi on live vegetative structures suggests that they might begin the colonization process as endophytes, gaining a competitive advantage over the other saprobic fungi on the plants.

Lulworthia atlantica: a new species supported by molecular phylogeny and morphological analysis.

This study presents morphological and molecular evidence for the establishment of a new species of Lulworthia based on the evaluation of 11 isolates of Lulworthiales collected in two marinas on the west coast of Portugal initially classified as Lulworthia sp. Maximum likelihood and Bayesian methods were applied to obtain phylogenetic trees for 18S-28S and internal transcribed spacer (ITS1-5.8S-ITS2 = internal transcribed spacer [ITS]) nuc rDNA. The isolates consistently grouped together in a distinct and highly supported clade; all other Lulworthiales analyzed, including the available sequences of Lulworthia spp., segregated separately. These results were supported by common and distinctive morphological characters and enabled recognition and description of the new species Lulworthia atlantica. Two Portuguese isolates were confirmed as Lulworthia cf. purpurea, and two were confirmed as asexual morphs of Zalerion maritima.

Diversity and Ecological Characterization of Sporulating Higher Filamentous Marine Fungi Associated with Spartina maritima (Curtis) Fernald in Two Portuguese Salt Marshes.

Fungal communities associated with early stages of decomposition of Spartina maritima (Curtis) Fernald were assessed in two geographically distinct salt marshes in Portugal by direct observation of fungal sporulating structures. Twenty-three fungal taxa were identified from 390 plant samples, 11 of which were common to both study sites. Natantispora retorquens, Byssothecium obiones, Phaeosphaeria spartinicola, Phoma sp. 1 and Stagonospora sp. were the most frequent fungal taxa in the studied communities. The fungal species Anthostomella spissitecta, Camarosporium roumeguerii, Coniothyrium obiones, Decorospora gaudefroyi, Halosarpheia trullifera, Leptosphaeria marina and Stagonospora haliclysta were recorded for the first time on S. maritima plants; with the exception of C. roumeguerii and L. marina, all of these species were also new records for Portugal. The differences between species composition of the communities associated with S. maritima were attributed to differences in abiotic conditions of the salt marshes. Although the fungal taxa were distributed differently along the host plants, common species to both fungal communities were found on the same relative position, e.g. B. obiones, Lulworthia sp. and N. retorquens occurred on the basal plant portions, Buergenerula spartinae, Dictyosporium pelagicum and Phoma sp. 1 on the middle plant portions and P. spartinicola and Stagonospora sp. on the top plant portions. The distinct vertical distribution patterns reflected species-specific salinity requirements and flooding tolerance, but specially substrate preferences. The most frequent fungi in both communities also exhibited wider distribution ranges and produced a higher number of fruiting structures, suggesting a more active key role in the decay process of S. maritima.

Use of drift substrates to characterize marine fungal communities from the west coast of Portugal.

This survey reports the occurrence, diversity and similarity of marine fungi associated with five categories of drift substrates (Arundo donax, Phragmites australis, Spartina maritima, "other stems" and driftwood) collected on four sandy beaches of the western coast of Portugal. "Other stems" and driftwood are composite samples with a variety of identified and unidentified pieces of non-woody and woody substrates respectively. Fifty-six taxa were identified, including 38 Ascomycota and 18 anamorphic fungi. Twenty-six taxa were generalists; however several cases of "substrate recurrence" were identified. The very frequent fungi differed among the categories of studied substrates, with the exception of Corollospora maritima, very frequent on four categories. Except for S. maritima, P. australis and driftwood, cases of multiple fungal colonization were rare. S. maritima was the single substrate with five different marine fungi on one sample, as well as with the highest number of very frequent fungi, highest percentage of colonization and average number of fungi per sample. Driftwood presented the highest value of fungal richness (37 taxa) and A. donax the lowest (22 taxa). ANOSIM analysis of similarity showed that all substrates supported different fungal communities with the exception of the pair P. australis/"other stems". The effect of sample size on estimated fungal richness was tested, and the results let us conclude that, although most of the sporadic fungi (<1% occurrence) will be detected only in a very large number of samples, 60 samples of A. donax and "other stems" and 70 samples of all the other substrates may suffice to assess their respective representative marine mycota.

Marine fungi from Mira river salt marsh in Portugal.

The occurrence of fungi in the Mira salt marsh, Portugal was investigated for 12 months. Baits of Spartina maritima stems were exposed to permanent or temporary submersion at the upper and lower limits of the intertidal zone. The baits were observed for fruit bodies and spores directly and after incubation in moist chambers. Twenty six marine species were identified (17 Ascomycota, two Basidiomycota and seven mitosporic fungi). Twenty four are new records for Portugal. Nia globospora Barata and Basílio was published as a new species. Species were characterized with respect to frequency of occurrence, colonization capability and substrate succession. The diversity and similarity indexes of the fungi under different conditions were determined.