Outcomes of lung transplantation for pleuroparenchymal fibroelastosis: A French multicentric retrospective study.

BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented. METHODS: We conducted a nation-wide multicentric retrospective study in patients who underwent lung or heart-lung transplantation for chronic end-stage lung disease secondary to PPFE between 2012 and 2022 in France. RESULTS: Thirty-one patients were included. At transplantation, median age was 48 years [IQR 35-55]. About 64.5% were women. Twenty-one (67.7%) had idiopathic PFFE. Sixteen (52%) had bilateral LT, 10 (32%) had single LT, 4 (13%) had lobar transplantation and one (3%) had heart-lung transplantation. Operative mortality was 3.2%. Early mortality (<90 days or during the first hospitalization) was 32%. Eleven patients (35.5%) underwent reoperation for hemostasis. Eight (30.8%) experienced bronchial complications. Mechanical ventilation time was 10 days [IQR 2-55]. Length of stay in intensive care unit and hospital were 34 [IQR 18-73] and 64 [IQR 36-103] days, respectively. Median survival was 21 months. Post-transplant survival rates after 1, 2, and 5 years were 57.9%, 42.6% and 38.3% respectively. Low albuminemia (p = 0.046), FVC (p = 0.021), FEV1 (p = 0.009) and high emergency lung transplantation (p = 0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (p = 0.07). CONCLUSION: LT for PPFE is associated with high post-operative morbi-mortality rates. Patients requiring high emergency lung transplantation with advanced disease, malnutrition, or critical clinical status experienced worse outcomes. GOV IDENTIFIER: NCT05044390.

[Follow-up strategies for lung transplant recipients in France]. / Suivi partagé des patients transplantés pulmonaires.

BACKGROUND: Lung transplantation (LT) requires sustained care for a frequently polypathological condition. Follow-up is focused on three main issues: 1/stability of respiratory function; 2/comorbidity management; 3/preventive medicine. About 3000 LT patients in France are treated in 11 LT centers. Given the increased size of the LT recipient cohort, follow-up might be partially shared with peripheral centers. METHODS: This paper presents the suggestions of a working group of the SPLF (French-speaking respiratory medicine society) on possible modalities of shared follow-up. RESULTS: While the main LT center is tasked with centralizing follow-up, particularly the choice of optimal immunosuppression, an identified peripheral center (PC) may serve as an alternative to deal with acute events, comorbidities and routine assessment. Communication between the different centers should be free-flowing. Shared follow-up may be offered from the 3rd postoperative year to stable and consenting patients, whereas unstable and non-observant patients are poor candidates. CONCLUSION: These guidelines may serve as a reference for any pneumologist wishing to effectively contribute to follow-up, even and especially subsequent to lung transplant.

Clinical characteristics of and outcomes for patients with COVID-19 and comorbid lung diseases primarily hospitalized in a conventional pulmonology unit: A retrospective study.

BACKGROUND: Scant data are currently available about a potential link between comorbid chronic lung diseases (CLD) and the risk and severity of the coronavirus disease 2019 (COVID-19) infection. METHODS: To describe the clinical characteristics of and outcomes for patients with COVID-19 infection, including patients with comorbid respiratory diseases, who have been primarily hospitalized in the pulmonology department of Strasbourg University Hospital, France. In this retrospective, single-center study, we included all confirmed cases of COVID-19 from March 3 to April 15, 2020. We then compared the symptoms, biological and radiological findings, and outcomes for patients with and without CLD. RESULTS: Of the 124 patients that were enrolled, the median age was 62 years, and 75 patients (60%) were male. Overall, 40% of patients (n=50) had preexisting CLD, including chronic obstructive pulmonary disease (COPD) (n=15, 12%) and asthma (n=19, 15%). Twenty-eight patients were transferred to the intensive care unit (ICU), and six patients died in our unit. CLD were not predictive of ICU hospitalization, but a significantly higher total mortality was observed (17.6% vs. 5.5%, P<0.05) in these patients. CONCLUSIONS: Our results suggest the lack of an over-representation of CLD in COVID-19, representing 40% of patients in this cohort and even within a pulmonology department. CLD were not a risk factor for ICU management. However, a tendency to higher global mortality was observed in COVID-19 patients with CLD. Further studies are warranted to determine the risk of COVID-19 for patients with comorbid CLD.

Acute Cardiac Failure Due to Takotsubo Cardiomyopathy Secondary to a Phone Call for Lung Transplantation: A Case Report.

Lung transplantation is a therapeutic option for certain end-stage lung diseases. The phone call for lung transplantation is a major event in the life of these patients; as a result, it can generate significant stress. We herein present the case of a 58-year-old female patient with end-stage chronic obstructive pulmonary disease (COPD) who, while on the lung transplantation waiting list, received such a call. Complete transplant work-up, including cardiac tests undertaken shortly before, had revealed no contraindication to lung transplantation. She was admitted with severe acute respiratory failure, and her extensive work-up was compatible with pulmonary edema due to takotsubo cardiomyopathy. The lung transplantation was thus cancelled, owing to the patient's health condition and the poor quality of the graft as well. The patient stayed in the intensive care unit for several days, requiring noninvasive ventilation. The left ventricular function recovered completely within 10 days postdiagnosis, and the patient was discharged 13 days after her admission. The patient was transplanted 1 month thereafter, without any particular problems; she is currently, 8 months post-transplantation, in good condition. In the given case, the call for lung transplantation could have generated emotional stress severe enough to lead to takotsubo cardiomyopathy.

[SaO2 and PaO2 mismatch: Do not forget hemoglobinopathy]. / Discordance entre SaO2 ­ PaO2 : ne pas oublier les hémoglobinopathies.

INTRODUCTION: Different clinico-biological parameters are used to estimate the amount of oxygen available for the organism. Oxygen saturation measured with pulse oxymetry (SpO2), oxygen saturation of arterial blood (SaO2) and oxygen partial pressure of the arterial blood (PaO2) are the most commonly used. CASE REPORT: We report the case of a patient admitted for investigation of respiratory failure in the context of chronic dyspnea of effort. SpO2 and SaO2 were decreased, though the PaO2 was normal. This mismatch between oxygen saturation and PaO2 led to the diagnosis of hemoglobinopathy (Bassett hemoglobin). CONCLUSION: The diagnosis of hemoglobinopathy should be considered in cases of oxygen desaturation with normal respiratory and cardiac investigations. There are no reasons to prescribe long-term oxygen to these patients.

Successful lung retransplantation in a patient with acute fibrinous and organizing pneumonia: a case report.

Acute fibrinous and organizing pneumonia (AFOP) is an unusual histopathologic pattern characterized by the formation of intra-alveolar plugs of fibrin deposition and associated organizing pneumonia. AFOP is considered to be a form of rejection and portends a dismal prognosis. Here, we present the case of a young male patient who initially underwent a double lung transplantation for cystic fibrosis. After 42 months of regular follow-up, he experienced rapidly progressive respiratory failure. Acute rejection and opportunistic lung infections were suspected. The clinical conditions rapidly deteriorated despite treatment with broad-spectrum antibiotics and high-dose steroids. Therefore, AFOP was suspected owing to: 1) acute clinical presentation; 2) pulmonary computerized tomographic data; 3) typical histopathologic findings on transbronchial biopsieseconds, and 4) lack of response to different treatments. The patient required an emergency bilateral lung retransplantation 44 months after the initial transplantation. The histopathologic analysis of the explanted lungs confirmed the diagnosis of AFOP. Two years after the 2nd transplant, the patient is alive and well. To the best of our knowledge, this is the 1st case of a patient experiencing AFOP following lung transplantation who was successfully rescued by a 2nd bilateral lung retransplantation.

Clinical usefulness of oral immunoglobulins in lung transplant recipients with norovirus gastroenteritis: a case series.

Viral gastroenteritis causing diarrhea is a common complication observed in lung transplant recipients. Differently from the mild and typically self-limited disease seen in immunocompetent subjects, immunocompromised patients frequently have a more severe course. Norovirus and rotavirus are among the leading causes of severe gastroenteritis in transplant recipients. Specific treatment is unavailable, although good supportive treatment can significantly reduce morbidity. Previous studies have suggested that oral immunoglobulins may be used for the treatment of acute viral gastroenteritis after solid-organ transplantation. Herein, we conducted a retrospective chart review of 12 lung transplant recipients with norovirus-induced gastroenteritis who were treated with oral immunoglobulins for 2 days. Eleven patients were successfully treated, whereas 1 subject was only mildly improved. Four patients had at least 1 recurrence. No significant adverse effects were observed. We conclude that oral immunoglobulins may be clinically useful for lung transplant recipients with norovirus-induced gastroenteritis.

[Pleural effusion in hereditary haemorrhagic telangiectasia: hemothorax or empyema?]. / Pleurésie dans le cadre d'une maladie de Rendu-Osler: hémothorax ou pyothorax?

INTRODUCTION: In hereditary hemorrhagic telangiectasia the pulmonary arteriovenous malformations frequently lead to complications. In a case of pleural effusion in a patient with known pulmonary arterio-venous malformations, the first diagnosis to consider is a hemothorax even though alternatives such as empyema are possible. CASE REPORT: We report the case of a 35-year-old woman with a known diagnosis of hereditary hemorrhagic telangiectasia with bilateral arteriovenous malformations, who was admitted to the emergency department with subacute dyspnoea and left thoracic pain. The clinical examination suggested a hemothorax. Pulmonary angiography was performed and showed an arteriovenous malformation in the left lower lobe. Embolisation was undertaken and at the same time a chest tube was inserted. Pus was evacuated leading to the final diagnosis of pleural empyema. Progress was good despite an episode acute respiratory distress due to a pulmonary embolism. CONCLUSION: Pleural empyema is rarely described in the context of hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations. In the case of pleural effusion this diagnosis should be considered even though hemothorax is more common.

[Thoracic manifestations of Castleman's disease]. / Maladie de Castleman à expression thoracique.

INTRODUCTION: Castleman's disease is a rare orphan disease. The prevalence is estimated at less than 1/100 000. Respirologists may encounter this disease when its thoracic manifestations occur. CASE REPORT: The authors report two cases of Castleman's disease with two different thoracic involvements. The first patient was a 20-year-old man without a previous medical history. A chance chest X-ray revealed right basal opacity. A lung biopsy demonstrated giant lymph node polyclonal hyperplasia leading to the diagnosis of a thoracic form of Castleman's disease. Since the patient was completely symptom free, no treatment was proposed. The patient was stable after 10months of medical supervision. The second patient, a 34-year-old woman, had a medical history of myasthenia gravis, autoimmune thrombopenic purpura and haemolytic anaemia. Her general condition deteriorated and upper mediastinal enlargement was noted. A diagnosis of multicentric Castleman's disease was established by means of the biopsy of an axillary lymph node. As the symptoms persisted, she was treated by rituximab. The clinical response was dramatic. CONCLUSION: The authors call to mind the difficult diagnostic features and therapeutic strategies of Castleman's disease, a rare disease which may involve the thorax.