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The most frequent form, primary PAP, includes autoimmune PAP which accounts for over 90% of all PAP, defined by the presence of circulating anti\u2010GM\u2010CSF 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\u2010paving 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\u2010line treatment, with an inhaled GM\u2010CSF as second\u2010line 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\u2010year actuarial survival rate is 95%.<\/jats:p>","DOI":"10.1111\/resp.13831","type":"journal-article","created":{"date-parts":[[2020,5,4]],"date-time":"2020-05-04T04:14:25Z","timestamp":1588565665000},"page":"816-826","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":87,"title":["Pulmonary alveolar proteinosis"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1949-3461","authenticated-orcid":false,"given":"St\u00e9phane","family":"Jouneau","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine, Competence Centre for Rare Pulmonary Diseases, CHU Rennes Univ Rennes  Rennes France"},{"name":"IRSET UMR108 Univ Rennes  Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9dric","family":"M\u00e9nard","sequence":"additional","affiliation":[{"name":"Service d'Immunologie, de Th\u00e9rapie Cellulaire et d'H\u00e9matopo\u00ef\u00e8se H\u00f4pital Pontchaillou  Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Lederlin","sequence":"additional","affiliation":[{"name":"Department of Radiology, CHU Rennes Univ Rennes  Rennes France"},{"name":"LTSI, INSERM U1099 Univ Rennes  Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,5,3]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195806052582301"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-019-0066-3"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00001311"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2011.08.018"},{"key":"e_1_2_14_6_1","first-page":"166","article-title":"Pulmonary alveolar proteinosis","volume":"19","author":"Briens E","year":"2002","journal-title":"Rev. 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