Transfusion-related acute lung injury after intravenous immunoglobulin treatment in a lung transplant recipient.

Three weeks after single-lung transplantation for pulmonary fibrosis, a patient with high serum levels of de novo donor-specific antibodies received high-dose intravenous immunoglobulin (IVIG) infusion (scheduled dose: 2 g/kg on 2 days) to prevent antibody-mediated rejection. Within the first hours after completion of infusions, he experienced acute lung injury involving the transplanted lung. Given the clinical evolution and the absence of an alternative diagnosis, transfusion-related acute lung injury (TRALI) was diagnosed. The IVIG administered on each day was from the same batch. At day 110, because of an increase in the serum titers of donor-specific antibodies, IVIG therapy was reintroduced but from a different batch, with excellent clinical tolerance. The lung injury was explored biologically, but no mechanism was revealed. Given the increasing use of IVIG in solid-organ recipients, clinicians should be aware of possible TRALI after IVIG infusion.

[Pulmonary arterial hypertension related to HIV: is inflammation related to IL-6 the cornerstone?]. / Hypertension artérielle pulmonaire liée à l'infection VIH : de la pression artérielle pulmonaire à l'interleukine-6.

Vascular diseases have become the leading cause of mortality in the population treated for HIV infection. Pulmonary arterial hypertension (PAH) related to HIV (PAH-HIV), the fourth cause of PAH in France, has the same histological pattern as other PAH from the group 1 of Dana Point classification. But, conversely to idiopathic PAH in the general population, PAH-HIV is particular by its high frequency in HIV-infected population. This raises the question for the role of inflammation in the PAH-HIV pathophysiology. Its constant occurrence over the decades, despite introduction of combination antiretroviral therapy (CAT), does not preclude the hypothesis of an involvement of inflammation in the genesis of PAH-HIV. Indeed, it is well known that normalization of CD4+ by the CAT does not mean no inflammation. Especially, it persists an increased and continuous production of IL-6, a main cytokine in the genesis of PAH lesions. This inflammation mainly involves the endothelin-1 pathway, which has an action on endothelium and macrophages, leading to high production of IL-6. Moreover, plasmatic level of IL-6 has a prognostic value in PAH-HIV, independently from conventional (functional or hemodynamic) parameters. The use of endothelin receptor antagonist permits major effect on IL-6 production and dramatic effect on PAH in so-called "bosentan responders".

[Pulmonary cystic adenomatoid malformation in an adult patient: an underdiagnosed disease]. / Malformation kystique adénomatoïde pulmonaire de l'adulte : une pathologie méconnue.

Currently, most congenital lower respiratory tract malformations are detected during pregnancy or at birth, thanks to antenatal imaging. However, a pulmonary congenital cystic adenomatoid disease may be found in adulthood. The diagnosis is difficult, due to its rarity. We present the case of a patient whose diagnosis of pulmonary cystic adenomatoid malformation was confirmed when she had tuberculosis. A lobectomy was performed, which enabled identification of tuberculosis and also multiple cysts of adenomatoid malformation. The risk posed by this malformation, i.e. the risk of developing bronchioloalveolar carcinoma and of infection or pneumothorax, is the incentive for proposing formal surgical removal.

Combined pancreatic islets-lung transplantation in cystic fibrosis-related diabetes: case reports.

UNLABELLED: We report two cases of percutaneous portal embolization of pancreatic islets performed after double lung transplantation in cystic fibrosis (CF) patients using the pancreas of the same donor. CASE 1: A 19-year-old man with CF had insulin-dependent diabetes, which was poorly controlled despite an external insulin pump (96 IU/d): HbA(1c) = 9.8% and 1 to 3 hypoglycemic events per day. On October 29, 2007, he received a double lung graft because of chronic respiratory failure. For days after lung transplantation, 149,000 cultured IEQ (Islet EQuivalent) were injected by percutaneous intraportal infusion under local anesthesia. Immunosuppression consisted of steroids, cyclosporine, and azathioprine. Two years later, the forced expiratory volume (FEV) was 83%; C peptide level reached 1.4 µg/L, and the diabetes was satisfactorily controlled with an HbA(1c) of 7.5% and a decrease in insulin requirements to 30 U/d in the absence of hypoglycemic events. CASE 2: On July 10, 2006, a 32-year-old man with CF-related diabetes received a double lung graft because of chronic respiratory failure. Under multiple insulin injections, the HbA(1c) was 9.6% with numerous hypoglycemic events. On March 11, 2008, he again received a double lung graft because of persistent humoral rejection. Despite severe bleeding during the postoperative course, 234,000 IEQ were injected via the portal vein one week after lung transplantation. Immunosuppression consisted of steroids, tacrolimus, and mycophenolate mofetil. Eighteen months after the combined graft, the FEV was 52%; the plasma C-peptide reached 0.79 µg/L, the HbA(1c), 6% and the insulin requirements decreased to 55 U/d in the absence of hypoglycemic events. CONCLUSION: Combined lung-islet transplantation for patients with CF-related diabetes improved pulmonary and metabolic function.

[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.

[When should patients be referred by the physician to the lung transplant team? Patient selection, indications, timing of referral and preparation for lung transplantation]. / Quand le pneumologue doit-il envisager la greffe pulmonaire pour un de ses patients adulte? Indications, critères de sélection, préparation à la greffe.

INTRODUCTION: Lung transplantation (LT) is accepted as a therapeutic option in a wide range of end stage lung diseases, with evidence supporting survival and quality of life benefits in transplant recipients. Appropriate patients who have good chance of survival with transplantation should be identified carefully. STATE OF THE ART: Four diagnoses account for approximately 80% of transplant recipients: chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis and alpha-1-antitrypsin deficiency emphysema. The aim of this review is to discuss the selection process of potential candidates and to assist physicians in referring these patients to a transplant team. The decision to refer patients for transplantation is difficult and depends on several parameters such as the results of transplantation, the referring physician's view of survival prospects with actual medical therapy according to the pathology, and also the patient's physical, nutritional and psychological status. The timing of listing patients remains a difficult decision which is imposed by both defined criteria and uncertain events such as the rapid worsening of the lung disease and the likely waiting time. PERSPECTIVES: The optimal modalities for pre-surgical rehabilitation programs and their postoperative impact should be evaluated. CONCLUSIONS: Careful selection of potential candidates for lung transplantation at the most appropriate time should lead to an improvement of survival of such patients.

[Pituitary metastases from lung cancer. Five cases]. / Métastases hypophysaires de cancers bronchiques. A propos de 5 cas.

Pituitary metastases are rare and generally asymptomatic. We studied 5 patients with pituitary metastases from lung cancer, illustrating the different clinical features. These metastases were in these cases symptomatic with the manifestation being diabetes insipidus or visual field defect. Histological subtypes from our five patients were as well small cell or non small cell lung cancer. After diagnosis of pituitary metastasis, prognosis seems to be linked to the histological subtype and the stage of lung cancer, rather than to the presence of such metastases.