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1.
Respirology ; 25(8): 816-826, 2020 08.
Article in English | MEDLINE | ID: mdl-32363736

ABSTRACT

PAP is an ultra-rare disease in which surfactant components, that impair gas exchange, accumulate in the alveolae. There are three types of PAP. The most frequent form, primary PAP, includes autoimmune PAP which accounts for over 90% of all PAP, defined by the presence of circulating anti-GM-CSF antibodies. Secondary PAP is mainly due to haematological disease, infections or inhaling toxic substances, while genetic PAP affects almost exclusively children. PAP is suspected if investigation for ILD reveals a crazy-paving pattern on chest CT scan, and is confirmed by a milky looking BAL that gives a positive PAS reaction indicating extracellular proteinaceous material. PAP is now rarely confirmed by surgical lung biopsy. WLL is still the first-line treatment, with an inhaled GM-CSF as second-line treatment. Inhalation has been found to be better than subcutaneous injections. Other treatments, such as rituximab or plasmapheresis, seem to be less efficient or ineffective. The main complications of PAP are due to infections by standard pathogens (Streptococcus, Haemophilus and Enterobacteria) or opportunistic pathogens such as mycobacteria, Nocardia, Actinomyces, Aspergillus or Cryptococcus. The clinical course of PAP is unpredictable and spontaneous improvement can occur. The 5-year actuarial survival rate is 95%.


Subject(s)
Pulmonary Alveolar Proteinosis/pathology , Autoimmune Diseases/complications , Humans , Lung/pathology , Pulmonary Alveolar Proteinosis/classification , Pulmonary Alveolar Proteinosis/epidemiology , Pulmonary Alveolar Proteinosis/immunology , Tomography, X-Ray Computed
2.
Am J Ind Med ; 58 Suppl 1: S31-8, 2015 Nov.
Article in English | MEDLINE | ID: mdl-26509752

ABSTRACT

The 1930 International Labour Office Conference on silicosis in Johannesburg identified silicosis by setting a medicolegal framework to its nosology: as with other occupational illnesses, its medical content was fixed under economic pressure. This article follows a reading of all the proceedings of this conference (debates and reports of experts) to examine their potential impact on the etiology and nosology of other diseases, specifically sarcoidosis and pulmonary alveolar proteinosis (PAP), "idiopathic" diseases in which inorganic particles may be involved. We propose renewed study of the role of inorganic particles in these diseases. To do this, we propose to mobilize detection means such as mineralogical analysis and electron microscopy and in depth interviewing that are currently seldom used in France, in order to establish diagnosis and the potential occupational and environmental origin of these diseases.


Subject(s)
Congresses as Topic/history , Pulmonary Alveolar Proteinosis/history , Sarcoidosis/history , Silicosis/history , History, 20th Century , Humans , Pneumoconiosis/classification , Pneumoconiosis/diagnosis , Pneumoconiosis/history , Pulmonary Alveolar Proteinosis/classification , Pulmonary Alveolar Proteinosis/diagnosis , Sarcoidosis/classification , Sarcoidosis/diagnosis , Silicosis/classification , Silicosis/diagnosis , South Africa
3.
Rev Mal Respir ; 31(10): 975-91, 2014 Dec.
Article in French | MEDLINE | ID: mdl-25496792

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.


Subject(s)
Pulmonary Alveolar Proteinosis , Animals , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Biopsy , Bronchoalveolar Lavage , Disease Progression , Granulocyte-Macrophage Colony-Stimulating Factor/therapeutic use , Humans , Lung Transplantation , Pulmonary Alveolar Proteinosis/classification , Pulmonary Alveolar Proteinosis/diagnosis , Pulmonary Alveolar Proteinosis/etiology , Pulmonary Alveolar Proteinosis/therapy , Radiography, Thoracic , Rare Diseases , Rituximab
4.
Pathologe ; 25(4): 299-309, 2004 Jul.
Article in German | MEDLINE | ID: mdl-15241624

ABSTRACT

Pulmonary alveolar proteinoses are rare pulmonary diseases characterised by an intraalveolar accumulation of surfactant protein A. Subtyping of alveolar proteinoses: Type I alveolar proteinoses: severe respiratory insufficiency in newborns, which will take a lethal course without lung transplant; hereditary SP-B deficiency and an intraalveolar accumulation of N-terminal incompletely processed SP-C. Type II alveolar proteinoses: occur in newborns and infants; often take a lethal course; show intraalveolar accumulation of precursors of SP-B and mature SP-B as well as an accompanying interstitial lung disease of variable severity. Type III alveolar proteinoses: in infants and children; do not generally take a lethal course; they are characterised by an intraalveolar accumulation of precursors of SP-B and mature SP-B without accompanying interstitial lung disease. "Cryptogenic" congenital, acquired (idiopathic), and secondary type III alveolar proteinoses can be distinguished. In newborns, infants, and children with pulmonary alveolar proteinosis, a detailed pathological-anatomical examination including immunohistochemical and molecular genetic analyses, should be performed in order to optimise the therapeutical management.


Subject(s)
Pulmonary Alveolar Proteinosis/pathology , Child , Diagnosis, Differential , Humans , Infant , Infant, Newborn , Pulmonary Alveolar Proteinosis/classification , Pulmonary Alveolar Proteinosis/congenital
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