Allergic bronchopulmonary aspergillosis in a lung transplant recipient treated with mepolizumab.

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction to Aspergillus spp. ABPA diagnosis may be challenging due to its non-specific presentation. Standard ABPA treatment consists of systemic corticosteroids and antifungal agents. Mepolizumab, a monoclonal antibody against interleukin-5 seems to be a promising treatment for ABPA. Data about ABPA following lung transplantation (LuTx) are scarce. LuTx recipients are at higher risk for adverse effects of ABPA treatment compared to the general population. Here we present a case of a LuTx recipient who was successfully treated with mepolizumab for ABPA following LuTx. Prolonged administration of high dose prednisone was thus avoided. To our knowledge, this is the first case describing mepolizumab administration following LuTx. Mepolizumab seems particularly attractive as a corticosteroid-sparing agent or as an alternative option to antifungal treatments, because of its excellent safety profile and low risk of drug interactions.

Outcomes of SARS-CoV-2 infection among lung transplant recipients: A single center retrospective study.

BACKGROUND: Lung transplant recipients (LTRs) are at increased risk for coronavirus disease 2019 (COVID-19)-associated complications. METHODS: We aimed to describe the outcomes of polymerase chain reaction-documented severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in LTRs followed at our institution from March 2020 to July 2022. The primary outcome investigated was hospitalization or death from COVID-19-related symptoms within 28 days from diagnosis. RESULTS: Overall, 60 cases were included, of which 18 (30%) reached the primary outcome. Only one patient (2%) died. Anti-spike monoclonal antibodies (mAbs) were administered as early treatment in 36 patients (casirivimab/imdevimab = 2, sotrovimab = 31, and tixagevimab/cilgavimab = 3). Multivariate analysis revealed that age >60 years (p = .003; odds ratio [OR] 9.41; confidence interval [CI] 2.52-41.05) was associated with a higher risk for the primary outcome, while administration of mAbs as early treatment (p = .030; OR 0.23; CI 0.06-0.87) was associated with a lower risk. No effect of vaccination and SARS-CoV-2 variant was observed. Forced expiratory volume in 1 s and forced vital capacity values did not decrease among 37 patients who had spirometry performed 1 month after COVID-19. CONCLUSIONS: We observed a relatively low morbidity and mortality of COVID-19 in LTR. mAb administration was associated with a better outcome.

Impact of COVID-19 on long-term lung function in lung transplant recipients: A single-center retrospective cohort study.

Available data are limited concerning long-term lung function (LF) evolution after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in lung transplant (LT) recipients. The aim of this study is to determine the effect of first SARS-CoV-2 infection on long-term LF in LT recipients. We analyzed spirometry results of LT recipients followed at our institution (March 2020 to July 2022) at 3, 6, and 12 months after first SARS-CoV-2 infection. Overall, 42 LT patients of our cohort (70%) with COVID-19 were included for long-term LF analysis. Forced expiratory volume in 1 s (FEV1 ) declined significantly at 3 months (-4.5%, -97 mL, 95% CI [-163; -31], p < .01), but not at 6 and 12 months (-3.9%, -65 mL, 95% CI [-168; +39], p = .21). Results were quite similar for the forced vital capacity. Spirometry values declined significantly at 3 months after COVID-19 in LT recipients, presented a mixed decline at 6 months, and no significant decline at 12 months.

[When to consider lung transplantation?] / Quand penser à la transplantation pulmonaire ?

Pulmonary transplantation remains the ultimate therapeutic option for selected patients with an advanced pulmonary disease and terminal respiratory insufficiency when all other therapeutic options have been exhausted. The optimal time-frame to proceed to a first discussion and evaluation about lung transplantation may be difficult to determine. This article describes the pathway of a patient towards lung transplantation and summarizes the criteria, which may help to timely identify eligibility for this therapeutic modality. We will focus mainly on the 2021 update of the International Society for Heart and Lung Transplantation (ISHLT) recommendations for the selection of lung transplant candidates.

La transplantation pulmonaire reste l'ultime option thérapeutique pour des patients sélectionnés présentant une maladie pulmonaire avancée au stade d'insuffisance respiratoire terminale, une fois les autres traitements reconnus épuisés. Le moment idéal pour une première discussion et l'évaluation d'une transplantation pulmonaire peut être difficile à identifier. Cet article décrit le parcours d'un patient vers la transplantation pulmonaire et résume les différents facteurs qui permettent d'identifier son éligibilité pour ce traitement. Nous nous focalisons notamment sur les recommandations pour la sélection des receveurs de transplantation pulmonaire mises à jour en 2021 par l'International Society for Heart and Lung Transplantation (ISHLT).

Analysis of NLRP3, MVK and TNFRSF1A variants in adult Greek patients with autoinflammatory symptoms.

OBJECTIVES: Autoinflammatory diseases are characterised by abnormal hyperactivity of the innate immune system, causing systemic inflammation. The cryopyrin associated periodic syndrome (CAPS), the hyper IgD syndrome (HIDS) and the TNF receptor-associated periodic syndrome (TRAPS), are autoinflammatory conditions associated with mutations in the NLRP3, MVK and TNFRSF1A genes, respectively. We present the experience of our Department with these rare syndromes analysing genetic and clinical data of adult patients encountered between January 2011 and September 2017. METHODS: Eighty-eight adult patients with clinical suspicion of CAPS, HIDS and TRAPS were sequentially recruited and genetically tested for specific mutations in NLRP3, MVK and TNFRSF1A using Sanger sequencing. Clinical picture of mutation carriers was reviewed. Allele frequencies were compared to those described for the normal population by the 1000 Genomes project. RESULTS: Seventy-two of the 88 adult patients were found to be positive for mutations or polymorphisms. One patient carried two pathogenic MVK mutations (pV377I/c.1129G>A and c.850delG) and another one carried a pathogenic heterozygous pΑ439V/c.1316C>T NLRP3 mutation. Seventeen patients carried variants of uncertain significance. The pS434S/c.1302C>T NLRP3 mutation is slightly increased in our patients compared to the reference population and seems to correlate with severe symptom presentation. CONCLUSIONS: In rare cases, periodic fever and inflammatory symptoms in adults can be attributed to mutations in NLRP3, MVK and TNFRSF1A. Clinical assessment and genetic analysis are critical for proper diagnosis and treatment of autoinflammatory diseases.