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Secondary pulmonary alveolar proteinosis

MedGen UID:
845105
•Concept ID:
C3873302
•
Disease or Syndrome
Synonyms: Secondary PAP; secondary PAP; secondary pulmonary alveolar proteinosis; SPAP
SNOMED CT: Secondary pulmonary alveolar proteinosis (707510005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018483
Orphanet: ORPHA420259

Definition

A rare, acquired, interstitial lung disease, characterized by alveolar surfactant accumulation, cough, progressive dyspnea and respiratory insufficiency. The disease may be secondary to hematological disorder, toxic inhalation, and infection or may occur within the setting of immunosuppression after transplantation. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSecondary pulmonary alveolar proteinosis

Professional guidelines

PubMed

Secondary Pulmonary Alveolar Proteinosis Associated with Primary Myelofibrosis and Ruxolitinib Treatment: An Autopsy Case.
Sugiura H, Nishimori H, Nishii K, Toji T, Fujii K, Fujii N, Matsuoka KI, Nakata K, Kiura K, Maeda Y
Intern Med 2020 Aug 15;59(16):2023-2028. Epub 2020 May 23 doi: 10.2169/internalmedicine.4082-19. PMID: 32448830Free PMC Article

Recent clinical studies

Etiology

Intestinal Behçet's disease complicated by myelodysplastic syndrome and secondary pulmonary alveolar proteinosis: a case report.
Shimizu H, Sato S, Suzuki T, Sasajima T, Takahata Y, Shinohara N, Hideshima K, Yokokawa Y, Matsuhashi N, Ichii O, Tai M, Ejiri Y, Yano K, Ikezoe T, Ohira H, Migita K
BMC Gastroenterol 2021 Dec 20;21(1):488. doi: 10.1186/s12876-021-02065-0. PMID: 34930121Free PMC Article
Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review).
Zhang D, Tian X, Feng R, Guo X, Wang P, Situ Y, Xiao Y, Xu KF
BMC Pulm Med 2018 Jan 25;18(1):15. doi: 10.1186/s12890-018-0590-z. PMID: 29368649Free PMC Article
Pulmonary alveolar proteinosis: time to shift?
Papiris SA, Tsirigotis P, Kolilekas L, Papadaki G, Papaioannou AI, Triantafillidou C, Papaporfyriou A, Karakatsani A, Kagouridis K, Griese M, Manali ED
Expert Rev Respir Med 2015 Jun;9(3):337-49. Epub 2015 Apr 12 doi: 10.1586/17476348.2015.1035259. PMID: 25864717
Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis.
Ishii H, Trapnell BC, Tazawa R, Inoue Y, Akira M, Kogure Y, Tomii K, Takada T, Hojo M, Ichiwata T, Goto H, Nakata K; Japanese Center of the Rare Lung Disease Consortium
Chest 2009 Nov;136(5):1348-1355. doi: 10.1378/chest.09-0097. PMID: 19892674
Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy.
Chung JH, Pipavath SJ, Myerson DH, Godwin D
J Thorac Imaging 2009 May;24(2):115-8. doi: 10.1097/RTI.0b013e3181930ed6. PMID: 19465834Free PMC Article

Diagnosis

Images: Secondary pulmonary alveolar proteinosis in brucellosis.
Yan L, Wang Z, Zhao J, Liu J
Pulmonology 2023 Jul-Aug;29(4):351-352. Epub 2022 Oct 21 doi: 10.1016/j.pulmoe.2022.09.008. PMID: 36274047
Secondary pulmonary alveolar proteinosis in GATA-2 deficiency (MonoMAC syndrome).
Raziq FI, Abubaker A, Smith E, Uddin M
BMJ Case Rep 2020 Nov 30;13(11) doi: 10.1136/bcr-2020-238290. PMID: 33257389Free PMC Article
Secondary pulmonary alveolar proteinosis in acute myeloid leukemia.
Hirakawa T, Taniwaki M, Yamasaki M, Imanaka R, Hattori N
QJM 2019 Apr 1;112(4):293-294. doi: 10.1093/qjmed/hcy224. PMID: 30295888
Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review).
Zhang D, Tian X, Feng R, Guo X, Wang P, Situ Y, Xiao Y, Xu KF
BMC Pulm Med 2018 Jan 25;18(1):15. doi: 10.1186/s12890-018-0590-z. PMID: 29368649Free PMC Article
Pulmonary Manifestations of Lymphoma and Leukemia.
Bashoura L, Eapen GA, Faiz SA
Clin Chest Med 2017 Jun;38(2):187-200. doi: 10.1016/j.ccm.2016.12.003. PMID: 28477632

Therapy

Secondary Pulmonary Alveolar Proteinosis Associated with Primary Myelofibrosis and Ruxolitinib Treatment: An Autopsy Case.
Sugiura H, Nishimori H, Nishii K, Toji T, Fujii K, Fujii N, Matsuoka KI, Nakata K, Kiura K, Maeda Y
Intern Med 2020 Aug 15;59(16):2023-2028. Epub 2020 May 23 doi: 10.2169/internalmedicine.4082-19. PMID: 32448830Free PMC Article
Secondary Pulmonary Alveolar Proteinosis Following Treatment with Azacitidine for Myelodysplastic Syndrome.
Hashimoto M, Itonaga H, Nannya Y, Taniguchi H, Fukuda Y, Furumoto T, Fujioka M, Kasai S, Taguchi M, Taniguchi H, Sato S, Sawayama Y, Atogami S, Iwasaki K, Hata T, Soda H, Moriuchi Y, Nakata K, Ogawa S, Miyazaki Y
Intern Med 2020 Apr 15;59(8):1081-1086. Epub 2019 Dec 26 doi: 10.2169/internalmedicine.3770-19. PMID: 31875636Free PMC Article
Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease.
Imura Y, Yukawa N, Handa T, Nakashima R, Murakami K, Yoshifuji H, Ohmura K, Ishii H, Nakata K, Mimori T
Mod Rheumatol 2018 Jul;28(4):724-729. Epub 2016 Mar 11 doi: 10.3109/14397595.2016.1153443. PMID: 26872621
Pulmonary alveolar proteinosis: time to shift?
Papiris SA, Tsirigotis P, Kolilekas L, Papadaki G, Papaioannou AI, Triantafillidou C, Papaporfyriou A, Karakatsani A, Kagouridis K, Griese M, Manali ED
Expert Rev Respir Med 2015 Jun;9(3):337-49. Epub 2015 Apr 12 doi: 10.1586/17476348.2015.1035259. PMID: 25864717
Clinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease.
Handa T, Nakatsue T, Baba M, Takada T, Nakata K, Ishii H
Respir Investig 2014 Jan;52(1):75-9. Epub 2013 Jul 3 doi: 10.1016/j.resinv.2013.05.005. PMID: 24388375

Prognosis

Secondary pulmonary alveolar proteinosis during corticosteroid therapy for organising pneumonia associated with myelodysplastic syndrome.
Inoue D, Marumo S, Ishii H, Fukui M
BMJ Case Rep 2019 Sep 18;12(9) doi: 10.1136/bcr-2019-231055. PMID: 31537595Free PMC Article
Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review).
Zhang D, Tian X, Feng R, Guo X, Wang P, Situ Y, Xiao Y, Xu KF
BMC Pulm Med 2018 Jan 25;18(1):15. doi: 10.1186/s12890-018-0590-z. PMID: 29368649Free PMC Article
Clinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease.
Handa T, Nakatsue T, Baba M, Takada T, Nakata K, Ishii H
Respir Investig 2014 Jan;52(1):75-9. Epub 2013 Jul 3 doi: 10.1016/j.resinv.2013.05.005. PMID: 24388375
Secondary pulmonary alveolar proteinosis after unrelated cord blood hematopoietic cell transplantation.
Ansari M, Rougemont AL, Le Deist F, Ozsahin H, Duval M, Champagne MA, Fournet JC
Pediatr Transplant 2012 Aug;16(5):E146-9. Epub 2011 Mar 13 doi: 10.1111/j.1399-3046.2011.01487.x. PMID: 21395954
Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy.
Chung JH, Pipavath SJ, Myerson DH, Godwin D
J Thorac Imaging 2009 May;24(2):115-8. doi: 10.1097/RTI.0b013e3181930ed6. PMID: 19465834Free PMC Article

Clinical prediction guides

Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan.
Ishii H, Seymour JF, Tazawa R, Inoue Y, Uchida N, Nishida A, Kogure Y, Saraya T, Tomii K, Takada T, Itoh Y, Hojo M, Ichiwata T, Goto H, Nakata K
BMC Pulm Med 2014 Mar 5;14:37. doi: 10.1186/1471-2466-14-37. PMID: 24597668Free PMC Article
Secondary pulmonary alveolar proteinosis after unrelated cord blood hematopoietic cell transplantation.
Ansari M, Rougemont AL, Le Deist F, Ozsahin H, Duval M, Champagne MA, Fournet JC
Pediatr Transplant 2012 Aug;16(5):E146-9. Epub 2011 Mar 13 doi: 10.1111/j.1399-3046.2011.01487.x. PMID: 21395954
Pulmonary alveolar proteinosis associated with Pneumocystis carinii. Ultrastructural identification in bronchoalveolar lavage in AIDS and immunocompromised non-AIDS patients.
Tran Van Nhieu J, Vojtek AM, Bernaudin JF, Escudier E, Fleury-Feith J
Chest 1990 Oct;98(4):801-5. doi: 10.1378/chest.98.4.801. PMID: 2209133

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