La protéinose alvéolaire pulmonaire

Abstract

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.

Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF

“…The reasons for this discrepancy are unclear but might be associated with patient selection, ethnic differences or simply the heterogeneity of the disease itself. Also, 81.8% of patients had primary PAP [1,2], and 84.8% presented with a typical BAL fluid sufficient for diagnosis as in other Caucasian series [1], compared to only 58.7% in a Japanese cohort [24]. In our cohort, only 2 HRCT were atypical, and all our patients with primary PAP had positive GM-CSF antibodies, showing that the combination of HRCT, BAL and GM-CSF antibodies is sufficient to diagnose primitive PAP.…”

“…This high rate of relapses is well known [2,6]. The mechanism is not fully understood, yet the accumulation of surfactant according to disease severity and cause is a determining factor [2].…”

“…PAP is characterized by the accumulation of proteins and lipids of the surfactant in the alveolar duct, which are stained positively by periodic acid-Schiff, resulting in impairment of gas exchange [1,2,3]. Patients commonly present with cough and dyspnea [1].…”

Tos e hipoxemia como manifestación clínica de la proteinosis alveolar. Reporte de caso clínico

“…1 Si bien la biopsia de pulmón a cielo abierto es el estándar de oro para confirmar el diagnóstico, la endoscopía respiratoria con toma de muestras del LBA permite observar, en el 75% de los casos, 2 fluido de aspecto opaco y lechoso, macrófagos alveolares espumosos, grandes cuerpos eosinófilos acelulares y detrito granular positivo para la tinción de Schiff.…”

“…1 Se presenta un caso clínico de una paciente derivada al Hospital "Prof. Dr. Juan P. Garrahan" para el estudio de una neumonía con evolución desfavorable por hipoxemia persistente.…”