Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95%. The most frequent complications are infectious etiology.
Complications are easily detected by the appearance of respiratory symptoms and do not necessitate systematic flexible bronchoscopy, but only preventive measures such as regular aerosol therapy, adapted respiratory physiotherapy and regular clinical follow-up.
Introduction. About 10% of the 300 million people worldwide who suffer from asthma have a severe disease that is uncontrolled despite treatment with inhaled corticosteroids and long-acting beta agonists. The eosinophilic inflammation pathway in the respiratory tract and blood is involved and interleukin-5 (IL-5) has recently been identified as a major promotor of this pathway. The anti-IL-5 antibodies reduce the incidence of exacerbation and allowed steroid sparing in severe asthma patients but only two case reports have been published on their use in critical care. Case Presentation. This report describes the extraordinary clinical improvement of a young patient with steroid-refractory eosinophilic acute severe asthma who required mechanical ventilation, VV-ECMO followed by treatment with mepolizumab. The salient point in this case is the use of an anti-IL-5 monoclonal antibody for a critically ill patient whose condition was deteriorating despite mechanical ventilation and VV-ECMO. The usual steroid treatment failed to control the increase in blood eosinophils or his bronchial inflammation and constriction. Conclusion. Anti-IL-5 antibodies are now a standard treatment for severe eosinophilic asthma that can also be useful in an emergency to treat steroid-refractory eosinophilic acute severe asthma.
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