PAP
MCID: PLM150
MIFTS: 39

Pulmonary Alveolar Proteinosis, Acquired (PAP)

Categories: Blood diseases, Immune diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Alveolar Proteinosis, Acquired

MalaCards integrated aliases for Pulmonary Alveolar Proteinosis, Acquired:

Name: Pulmonary Alveolar Proteinosis, Acquired 57 70
Autoimmune Pulmonary Alveolar Proteinosis 20 58
Pulmonary Alveolar Lipoproteinosis, Acquired 57
Pulmonary Alveolar Lipoproteinosis Acquired 20
Pulmonary Alveolar Proteinosis, Autoimmune 57
Pulmonary Alveolar Proteinosis Autoimmune 20
Idiopathic Pulmonary Alveolar Proteinosis 58
Acquired Pulmonary Alveolar Proteinosis 20
Pulmonary Alveolar Proteinosis Acquired 20
Autoimmune Pap 58
Idiopathic Pap 58
Pap, Acquired 57
Pap Acquired 20
Apap 58
Ipap 58
Pap 20

Characteristics:

Orphanet epidemiological data:

58
autoimmune pulmonary alveolar proteinosis
Inheritance: Not applicable; Prevalence: <1/1000000 (Japan),1-9/1000000 (Japan),1-9/1000000 (Worldwide),<1/1000000 (Worldwide); Age of onset: Adult;

OMIM®:

57 (Updated 20-May-2021)
Miscellaneous:
insidious onset
acquired autoimmune disorder
usually adult onset
associated with smoking
rare spontaneous improvement occurs (8%)
death due to respiratory failure or infection

Inheritance:
isolated cases


HPO:

31
pulmonary alveolar proteinosis, acquired:
Inheritance sporadic
Onset and clinical course insidious onset


Classifications:

Orphanet: 58  
Rare respiratory diseases


Summaries for Pulmonary Alveolar Proteinosis, Acquired

GARD : 20 Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare autoimmune lung disorder. It is the most common form (90% of the cases) of pulmonary alveolar proteinosis (PAP). Most cases affect adults between the ages of 20-50 years. Some people may not show symptoms, while others may have progressive difficulty breathing and shortness of breath upon exertion. Other signs and symptoms may include a dry, chronic cough; fatigue; weight loss; chest pain; and a general feeling of ill health. In rare cases, coughing up of blood, rounding and swelling of the tips of the fingers, and cyanosis may be present. Autoimmune PAP is caused by an immune system malfunction, due to IgG antibodies that block the granulocyte-Macrophage Colony Stimulating Factor ( GM-CSF) effect. GM-CSF is a protein that regulates clearance of surfactant (a mix of protein and fat) by alveolar macrophages. The surfactant pile up in the air sacs of the lungs (alveoli), and eventually lead to an inability to breath. The standard treatment is a procedure called lung lavage. A drug called GM-CSF might effectively treat aPAP. Other PAP forms include congenital PAP, a very rare form that occurs in some newborns, caused by d isorders of surfactant production; hereditary PAP, where people are born with genetic mutations that destroy the function of the proteins (receptors) on alveolar macrophages that interact with GM-CSF; and, secondary PAP, which occur in people with another disease or dust exposure.

MalaCards based summary : Pulmonary Alveolar Proteinosis, Acquired, also known as autoimmune pulmonary alveolar proteinosis, is related to surfactant metabolism dysfunction, pulmonary, 4 and surfactant metabolism dysfunction, pulmonary, 5, and has symptoms including hemoptysis, chest pain and coughing. The drugs Guaifenesin and Hydromorphone have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and cervix, and related phenotypes are intraalveolar phospholipid accumulation and dyspnea

OMIM® : 57 Pulmonary alveolar proteinosis is a pathologic entity characterized by intraalveolar surfactant accumulation. There are 3 clinically distinct forms: hereditary (usually congenital), secondary, and acquired. The acquired form of pulmonary alveolar proteinosis is the most common form, accounting for approximately 90% of cases. The mean age at diagnosis is 39 years and it is associated with smoking in 72% of cases. The estimated incidence and prevalence are 0.36 and 3.70 cases per million, respectively (Trapnell et al., 2003; Seymour and Presneill, 2002). Secondary pulmonary alveolar proteinosis develops in association with conditions involving functional impairment or reduced numbers of alveolar macrophages. Such conditions include some hematologic cancers, pharmacologic immunosuppression, inhalation of inorganic dust or toxic fumes, and certain infections. Congenital pulmonary alveolar proteinosis is a rare, severe, often fatal disorder of newborns associated with pulmonary surfactant metabolism dysfunction caused by mutations in genes involved in surfactant metabolism (see, e.g., SMDP1, 265120) (Trapnell et al., 2003). See 300770 for information on congenital PAP due to CSF2RA (306250) deficiency. (610910) (Updated 20-May-2021)

Related Diseases for Pulmonary Alveolar Proteinosis, Acquired

Diseases in the Pulmonary Alveolar Proteinosis family:

Pulmonary Alveolar Proteinosis, Acquired Congenital Pulmonary Alveolar Proteinosis
Hereditary Pulmonary Alveolar Proteinosis Secondary Pulmonary Alveolar Proteinosis

Diseases related to Pulmonary Alveolar Proteinosis, Acquired via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 417)
# Related Disease Score Top Affiliating Genes
1 surfactant metabolism dysfunction, pulmonary, 4 11.5
2 surfactant metabolism dysfunction, pulmonary, 5 11.3
3 surfactant metabolism dysfunction, pulmonary, 1 11.3
4 cervical cancer 11.2
5 polydactyly, postaxial, type a1 11.2
6 congenital pulmonary alveolar proteinosis 11.2
7 pituitary adenoma 1, multiple types 11.0
8 polydactyly, postaxial, type a10 11.0
9 pulmonary alveolar proteinosis 11.0
10 vaginal cancer 10.9
11 hereditary pulmonary alveolar proteinosis 10.9
12 human papillomavirus infectious disease 10.9
13 surfactant metabolism dysfunction, pulmonary, 2 10.9
14 surfactant metabolism dysfunction, pulmonary, 3 10.9
15 interstitial lung and liver disease 10.9
16 cervix disease 10.9
17 chondrodysplasia with joint dislocations, gpapp type 10.9
18 mycoplasma pneumoniae pneumonia 10.9
19 cervix uteri carcinoma in situ 10.8
20 cervical intraepithelial neoplasia 10.8
21 lung disease 10.6
22 sleep apnea 10.6
23 squamous cell papilloma 10.5
24 papilloma 10.5
25 sarcoidosis 1 10.5
26 suppressor of tumorigenicity 3 10.5
27 respiratory failure 10.5
28 pancreatitis 10.4
29 pyelonephritis 10.4
30 chlamydia 10.4
31 in situ carcinoma 10.4
32 endometrial cancer 10.4
33 autoimmune disease 10.4
34 pulmonary fibrosis 10.4
35 rare pulmonary disease 10.4
36 acute pancreatitis 10.3
37 bacterial vaginosis 10.3
38 adenocarcinoma 10.3
39 prostatic hypertrophy 10.3
40 endometrial adenocarcinoma 10.3
41 cervicitis 10.3
42 fibrosis of extraocular muscles, congenital, 1 10.3
43 aspergillosis 10.3
44 secondary pulmonary alveolar proteinosis 10.3
45 vaginal discharge 10.3
46 anus cancer 10.2
47 vaginitis 10.2
48 cystic fibrosis 10.2
49 endocervical adenocarcinoma 10.2
50 acute cystitis 10.2

Graphical network of the top 20 diseases related to Pulmonary Alveolar Proteinosis, Acquired:



Diseases related to Pulmonary Alveolar Proteinosis, Acquired

Symptoms & Phenotypes for Pulmonary Alveolar Proteinosis, Acquired

Human phenotypes related to Pulmonary Alveolar Proteinosis, Acquired:

31 58 (show all 30)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 intraalveolar phospholipid accumulation 31 hallmark (90%) HP:0006517
2 dyspnea 58 31 frequent (33%) Frequent (79-30%) HP:0002094
3 hypoxemia 58 31 very rare (1%) Frequent (79-30%) HP:0012418
4 cyanosis 58 31 very rare (1%) Frequent (79-30%) HP:0000961
5 foam cells 58 31 frequent (33%) Frequent (79-30%) HP:0003651
6 autoimmune antibody positivity 58 31 frequent (33%) Frequent (79-30%) HP:0030057
7 clubbing 58 31 very rare (1%) Frequent (79-30%) HP:0001217
8 abnormality of the upper respiratory tract 58 31 frequent (33%) Frequent (79-30%) HP:0002087
9 decreased dlco 58 31 frequent (33%) Frequent (79-30%) HP:0045051
10 restrictive ventilatory defect 31 very rare (1%) HP:0002091
11 increased lactate dehydrogenase level 31 frequent (33%) HP:0025435
12 abnormal circulating protein concentration 31 frequent (33%) HP:0010876
13 cough 58 31 occasional (7.5%) Occasional (29-5%) HP:0012735
14 crackles 58 31 occasional (7.5%) Occasional (29-5%) HP:0030830
15 crazy paving pattern on pulmonary hrct 58 31 occasional (7.5%) Occasional (29-5%) HP:0025391
16 fatigue 58 31 very rare (1%) Very rare (<4-1%) HP:0012378
17 fever 58 31 very rare (1%) Very rare (<4-1%) HP:0001945
18 hemoptysis 58 31 very rare (1%) Very rare (<4-1%) HP:0002105
19 chest pain 58 31 very rare (1%) Very rare (<4-1%) HP:0100749
20 weight loss 58 31 very rare (1%) Very rare (<4-1%) HP:0001824
21 brain abscess 31 very rare (1%) HP:0030049
22 lung abscess 31 very rare (1%) HP:0025044
23 anti-granulocyte-macrophage colony stimulating factor antibody positivity 31 very rare (1%) HP:0020050
24 inspiratory crackles 31 very rare (1%) HP:0031996
25 recurrent respiratory infections 31 HP:0002205
26 pneumonia 31 HP:0002090
27 restrictive deficit on pulmonary function testing 58 Frequent (79-30%)
28 increased lactate dehydrogenase activity 58 Frequent (79-30%)
29 alveolar proteinosis 58 Very frequent (99-80%)
30 abnormality of circulating protein level 58 Frequent (79-30%)

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Respiratory:
hemoptysis
cough
chest pain
dyspnea, progressive

Immunology:
systemic infections
serum igg autoantibodies to granulocyte/macrophage colony-stimulating factor (gm-csf, csf2, )
defective macrophage function
defective neutrophil function
secondary respiratory infections
more
Skeletal Hands:
digital clubbing due to chronic hypoxia

Laboratory Abnormalities:
polycythemia
hypoxemia

Respiratory Lung:
alveoli accumulate pas-positive eosinophilic lipoproteinaceous material
accumulation of surfactant protein
inspiratory crackles (50%)
radiology shows bilateral airspace disease with ill-defined nodular or confluent pattern with perihilar predominance
radiology suggestive of pneumonia
more
Skin Nails Hair Skin:
cyanosis (25%)

Clinical features from OMIM®:

610910 (Updated 20-May-2021)

UMLS symptoms related to Pulmonary Alveolar Proteinosis, Acquired:


hemoptysis; chest pain; coughing

Drugs & Therapeutics for Pulmonary Alveolar Proteinosis, Acquired

Drugs for Pulmonary Alveolar Proteinosis, Acquired (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 70)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Guaifenesin Approved, Investigational, Vet_approved Phase 4 93-14-1 3516
2
Hydromorphone Approved, Illicit Phase 4 466-99-9 5284570
3
Phenylpropanolamine Approved, Vet_approved, Withdrawn Phase 4 14838-15-4 26934
4
Tramadol Approved, Investigational Phase 4 27203-92-5 33741
5
Aceclofenac Approved, Investigational Phase 4 89796-99-6 71771
6
Oxycodone Approved, Illicit, Investigational Phase 4 76-42-6 5284603
7
Hydrocodone Approved, Illicit, Investigational Phase 4 125-29-1 5284569
8
Acetaminophen Approved Phase 4 103-90-2 1983
9
Cysteine Approved, Nutraceutical Phase 4 52-90-4 5862
10 Chlorpheniramine, phenylpropanolamine drug combination Phase 4
11 Antitussive Agents Phase 4
12 Respiratory System Agents Phase 4
13 Liver Extracts Phase 4
14 Anti-Asthmatic Agents Phase 4
15 Analgesics Phase 4
16 Antirheumatic Agents Phase 4
17 Anti-Inflammatory Agents Phase 4
18 Analgesics, Non-Narcotic Phase 4
19 Anti-Inflammatory Agents, Non-Steroidal Phase 4
20 Acetaminophen, hydrocodone drug combination Phase 4
21 Analgesics, Opioid Phase 4
22 Narcotics Phase 4
23
Diphenhydramine Approved, Investigational Phase 3 147-24-0, 58-73-1 3100
24
Promethazine Approved, Investigational Phase 3 60-87-7 4927
25
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
26
Molgramostim Investigational Phase 3 99283-10-0
27 Pulmonary Surfactants Phase 2, Phase 3
28 Antibiotics, Antitubercular Phase 2, Phase 3
29 Autoantibodies Phase 2, Phase 3
30 Pharmaceutical Solutions Phase 3
31
Pioglitazone Approved, Investigational Phase 1 111025-46-8 4829
32
Trazodone Approved, Investigational Phase 1 19794-93-5 5533
33 Hypoglycemic Agents Phase 1
34 Psychotropic Drugs Phase 1
35 Anti-Anxiety Agents Phase 1
36 Serotonin Uptake Inhibitors Phase 1
37 Neurotransmitter Agents Phase 1
38 Antidepressive Agents Phase 1
39
Serotonin Investigational, Nutraceutical Phase 1 50-67-9 5202
40
Cocaine Approved, Illicit 50-36-2 5760 446220
41
Meperidine Approved Early Phase 1 57-42-1 4058
42
Pregabalin Approved, Investigational Early Phase 1 148553-50-8 5486971
43
Amikacin Approved, Investigational, Vet_approved 37517-28-5 37768
44
Fomepizole Approved, Vet_approved Early Phase 1 7554-65-6 3406
45
tannic acid Approved 1401-55-4
46
Benzocaine Approved, Investigational 1994-09-7, 94-09-7 2337
47
Mefenamic acid Approved Early Phase 1 61-68-7 4044
48
Ibuprofen Approved 15687-27-1 3672
49
Dehydroepiandrosterone Approved, Investigational, Nutraceutical Early Phase 1 53-43-0 5881
50 Anti-Bacterial Agents

Interventional clinical trials:

(show top 50) (show all 85)
# Name Status NCT ID Phase Drugs
1 Exacerbation's Prevention in Patients With COPD in GOLD IV Stage (Very Severe) With Non Invasive Mechanical Ventilation Unknown status NCT01481727 Phase 4
2 Prospective Aleatory Study of Non Invasive Mechanical Ventilation Versus Respiratory Rehabilitation in Hypercapnic Stable Severe COPD Completed NCT01377818 Phase 4
3 Evaluating the Pharmacokinetic Profile of Hydromorphone in Chronic Pain Patients Taking Hydrocodone/APAP Completed NCT01517295 Phase 4 Hydrocodone
4 Open-Label Parallel Group Flexible Dosing & Titration Study to Evaluate the Efficacy, Safety of Oxycodone/APAP Extended Release Formulation Xartemisxr® in the Management of Post-Operative Pain Following Outpatient Arthroscopic Knee Surgery Completed NCT02391844 Phase 4 Oxycodone
5 A Randomized, Open Label,Prospective Study of the Analgesic Efficacy of Oral Xartemis Compared to Generic Oxycodone/APAP (Acetaminophen) in the Treatment of Moderate to Severe Post Operative Pain Completed NCT02101476 Phase 4 Percocet;Xartemis
6 Chung Shan Medical University Hospital, Taiwan Completed NCT00647517 Phase 4 Ultracet
7 Serum Acetaminophen-Cysteine (APAP-CYS) Adduct Concentrations in Subjects Expected to Develop Aminotransferase Elevations With Liver-Directed Therapy Intended to Treat Hepatic Tumors Recruiting NCT02911961 Phase 4 Acetaminophen
8 Effect of the Noninvasive Mechanical Ventilation on the Daily Physical Activity and the Inflammatory Biomarkers in Stable Patients With COPD Recruiting NCT01037387 Phase 4 Control
9 A Phase 4, Open-Label Study of the Pharmacokinetics and Safety of XARTEMIS® XR (7.5 Oxycodone Hydrochloride/325 mg Acetaminophen) in Postsurgical Adolescent Subjects (Ages 12 to 17) With Moderate to Severe Acute Pain Terminated NCT02508935 Phase 4 XARTEMIS XR
10 Whole Lung Lavage Followed by Inhaled Sargramostim in the Treatment of Autoimmune Pulmonary Alveolar Proteinosis Unknown status NCT00901511 Phase 2, Phase 3 GM-CSF [Leukine (Sargramostim)]
11 Therapeutic Efficacy Evaluation of the CPAP/autoCPAP Sandman in the Treatment of New Patients With Obstructive Sleep Apnoea Syndrome Unknown status NCT00646477 Phase 3
12 Prospective, Open-labeled, Randomized Controlled Trial of Comparison Between High-flow Nasal Cannula System and Non-invasive Ventilation in Acute Hypoxemic Respiratory Failure Unknown status NCT01166256 Phase 2, Phase 3
13 Phase III Single-blind Randomized Controlled Trial of Bipap Versus CPAP in Overlap Syndrome Unknown status NCT01427673 Phase 3
14 A Randomised, Double-Blind, Placebo-Controlled Multicentre Clinical Trial of Inhaled Molgramostim in Autoimmune Pulmonary AlveoLAr Proteinosis Patients "IMPALA" Completed NCT02702180 Phase 2, Phase 3 molgramostim;placebo
15 An Open-label, Non-controlled, Multicentre Clinical Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis Patients Completed NCT03482752 Phase 3 Molgramostim
16 A Phase 3, Double-blind, Randomized Safety And Efficacy Study Comparing Multiple Administrations Of Ibu 250 Mg/Apap 500 Mg (Administered As Two Tablets Of Ibu/Apap 125 Mg/250 Mg) To Placebo In The Treatment Of Post Surgical Dental Pain In Adult Subjects Completed NCT02837952 Phase 3 FDC IBU/APAP 250 mg/500 mg;Placebo
17 A Phase 3, Double-blind, Randomized, Placebo-controlled, Full Factorial, Safety And Efficacy Study Comparing The Antipyretic Effects Of A Single Oral Dose Of Ibuprofen (Ibu) 250 Mg/ Acetaminophen (Apap) 500 Mg Caplets To Ibu 250 Mg And Apap 500 Mg Caplets In Healthy Male Volunteers With Fever Induced By An Endotoxin Completed NCT02761980 Phase 3 Ibuprofen 250 mg / Acetaminophen 500 mg;Ibuprofen 250 mg;Acetaminophen 500 mg;Placebo
18 A Phase 3, Double-Blind, Randomized, Safety And Efficacy Study Comparing A Single Oral Dose Of Ibuprofen (IBU) 250 Mg/Acetaminophen (APAP) 500 Mg (Administered As Two Tablets Of IBU/APAP 125 Mg/250 Mg) To Each Active Drug Monocomponent Alone And To Placebo In The Treatment Of Post-Surgical Dental Pain Completed NCT02912650 Phase 3 Ibuprofen 250 mg / Acetaminophen 500 mg;Ibuprofen 250 mg;Acetaminophen 650 mg;Placebo
19 A Randomized, Double-Blind, Placebo- and Active-Controlled Study to Determine the Efficacy and Safety of CL-108 5 mg (Hydrocodone 5 mg/Acetaminophen 325 mg/Promethazine 12.5 mg) as a Treatment for Acute Pain and the Prevention of Opioid-Induced Nausea and Vomiting (OINV) Completed NCT03657810 Phase 3 CL-108 5 mg;Norco;Placebo
20 Phase 3 Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Parallel, Multiple-Dose Study of the Analgesic Efficacy and Safety of IV Acetaminophen (APAP) Versus Placebo Over 48 Hours(Hrs) for the Treatment of Postoperative Pain After Gynecologic Surgery Completed NCT00399568 Phase 3 IV Acetaminophen;IV Placebo 100 mL solution
21 A Phase III Multi-Center, Open-Label, Prospective, Repeated Dose, Multi-Day Study of the Safety & Efficacy of Intravenous Acetaminophen in Pediatric Inpatients. Completed NCT00598702 Phase 3 IV Acetaminophen
22 A Phase III, Randomized, Double-Blind, Double-Dummy, Single-Dose Study of the Efficacy and Safety of Intravenous Acetaminophen Versus Oral Acetaminophen for the Treatment of Endotoxin-Induced Fever in Healthy Adult Males Completed NCT00564629 Phase 3 Intravenous acetaminophen plus oral placebo;Oral acetaminophen plus IV placebo
23 Metabolism and Toxicity of Acetaminophen in Preterm Infants Active, not recruiting NCT01328808 Phase 2, Phase 3 Acetaminophen/APAP
24 A Randomized, Double-blind, Placebo-controlled Clinical Trial of Once-daily Inhaled Molgramostim Nebulizer Solution in Adult Subjects With Autoimmune Pulmonary Alveolar Proteinosis (aPAP) Not yet recruiting NCT04544293 Phase 3 Molgramostim;Placebo
25 A Prospective Study of Inhaling Granulocyte-macrophage Colony Stimulating Factor in Adult Patients With Mild-to-moderate Autoimmune Pulmonary Alveolar Proteinosis in China: a Randomized Open-label Study Unknown status NCT02243228 Phase 2 GM-CSF
26 A Multicenter Clinical Study of the Sequential Therapy With Whole Lung Lavage/Inhaling Granulocyte-macrophage Colony Stimulating Factor in Adult Patients With Severe Autoimmune Pulmonary Alveolar Proteinosis in China Unknown status NCT03316651 Phase 2 GM-CSF
27 Pulmonary Alveolar Proteinosis GM-CSF Inhalation Efficacy Trial in Japan Completed NCT02835742 Phase 2 Sargramostim;Placebo
28 A Phase 2 Randomized, Double-blind, Placebo-controlled, 3-arm, Parallel-design Study of the Efficacy and Safety of VX-150 for Acute Pain Following Bunionectomy Completed NCT03206749 Phase 2 VX-150;HB/APAP;Placebo
29 IBUPAP - Combination of Oral Ibuprofen and Acetaminophen (APAP) is Superior to Either Analgesic Alone for Pediatric Emergency Department (ED) Patients With Acute Pain Completed NCT03088800 Phase 2 Ibuprofen;APAP
30 SK-1401 (rhGM-CSF Agent for Inhalation) GM-CSF Inhalation Pharmacokinetic Study Completed NCT02840708 Phase 1 Sargramostim
31 First in Human Study of Pioglitazone Therapy of Autoimmune Pulmonary Alveolar Proteinosis Completed NCT03231033 Phase 1 Pioglitazone
32 To Compare the Relative Bioavailability of the Kali's Tramadol APAP Tablets With Ortho-McNeil's Ultracet Tablets Under Fed Conditions. Completed NCT00652821 Phase 1 Tramadol/ APAP;Ultracet
33 To Compare the Relative Bioavailability of the Kali's Tramadol APAP Tablets With Ortho-McNeil's Ultracet Tablets Under Fasting Conditions. Completed NCT00653315 Phase 1 Tramadol APAP;Ultracet
34 Non Invasive Ventilation in Acute Myocardial Infarction Completed NCT01484795 Phase 1
35 Evaluation of a Single Dose of Inhaled Sargramostim in Patients With Autoimmune Pulmonary Alveolar Proteinosis Recruiting NCT03006146 Phase 1 Sargramostim
36 Randomized Controlled Trial of Trazodone Dose Tolerance and APAP Adherence Suspended NCT02945644 Phase 1 Trazodone;Placebo
37 Safety and Efficacy of Early Initiation of Noninvasive Positive Pressure Ventilation in Pediatric Patients Admitted With Status Asthmaticus Withdrawn NCT01188473 Phase 1
38 Biphasic Intermittent Positive Airway Pressure (BIPAP) Versus Airway Pressure Release Ventilation (APRV) in Patients With Multiple Fracture Ribs Unknown status NCT03314701
39 Therapy Adherence in Different Settings of Automatic Positive Airway Pressure (APAP) Therapy Initiation in Patients With Obstructive Sleep Apnea (OSA): Comparison Between in Sleep-lab vs. at Home Environment. Unknown status NCT02339597
40 Adherence and Preference of Continuous Positive Airway Pressure Versus Mandibular Advancement Splints in Obstructive Sleep Apnea Patients: A Randomized Trial Unknown status NCT02242617
41 Extended Evaluation of the Efficacy of a Proactive FOT-based Auto-CPAP Algorithm in Patients With Predominantly Obstructive Sleep Apnea Unknown status NCT03070756
42 The Effects of Non-invasive Ventilation During Cycle Exercise Within a 3-week Pulmonary Rehabilitation Program in COPD Patients With Chronic Hypercapnic Respiratory Failure - a Randomized Controlled Trial Unknown status NCT03803358
43 Effects of Non-invasive Positive Pressure Ventilation at Different Pressure on Respiratory Mechanics and Neural Respiratory Drive(NRD)in Patients With Stable Chronic Obstructive Pulmonary Disease(COPD) Unknown status NCT03555097
44 Acute Effect of EPAP and Noninvasive Ventilation on Pulmonary Function, Regional Lung Ventilation and Respiratory Mechanics on Patients Submitted Bariatric Surgery Evaluated by Spirometry and Electric Impedance Tomography: A Clinical Trial Controlled Randomized Unknown status NCT02577744
45 Prospective Trial of the Bilevel Positive Airway Pressure (by Boussignac) in Emergency Department of Non-invasive Patients Presenting With Acute Hypercapnic Respiratory Failure Unknown status NCT01788345
46 Effects of High-intensity Versus Low-intensity Noninvasive Positive Pressure Ventilation in Acute Exacerbation of Chronic Obstructive Pulmonary Disease: A Randomized Controlled Trial Completed NCT04044625
47 The Role of Pregabalin in the Treatment of Post-Operative Pain in Fracture Patients Completed NCT00583869 Early Phase 1 Placebo;Pregabalin;Pregabalin
48 Effect of Noninvasive Ventilation in Thoracic-abdominal Kinematics of Individuals With Heart Failure Associated With Cardiomegaly: a Randomized Controlled Trial Completed NCT02073253
49 Impact of Noninvasive Ventilation With Two Levels of Pressure on Exercise Tolerance in Subjects With Heart Failure: Randomized and Controlled Clinical Trial. Completed NCT02085122
50 Validation of a New Automatic Bi-level Algorithm in the Treatment of Sleep-disordered Breathing Completed NCT00910195

Search NIH Clinical Center for Pulmonary Alveolar Proteinosis, Acquired

Genetic Tests for Pulmonary Alveolar Proteinosis, Acquired

Anatomical Context for Pulmonary Alveolar Proteinosis, Acquired

MalaCards organs/tissues related to Pulmonary Alveolar Proteinosis, Acquired:

40
Lung, Liver, Cervix, Brain, Heart, Colon, Pancreas

Publications for Pulmonary Alveolar Proteinosis, Acquired

Articles related to Pulmonary Alveolar Proteinosis, Acquired:

(show top 50) (show all 87)
# Title Authors PMID Year
1
GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis. 57
17287477 2007
2
Pulmonary alveolar proteinosis--is host defense awry? 57
17287475 2007
3
Pulmonary alveolar proteinosis. 57
14695413 2003
4
Images in clinical medicine. Pulmonary alveolar proteinosis. 57
12501225 2002
5
Pulmonary alveolar proteinosis: progress in the first 44 years. 57
12119235 2002
6
Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. 57
8202532 1994
7
Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. 57
8171324 1994
8
Pulmonary alveolar proteinosis in two siblings with decreased immunoglobulin A. 57
7435514 1980
9
Simultaneous bilateral lung lavage (alveolar washing) using partial cardiopulmonary bypass. 57
5419972 1970
10
Pulmonary alveolar proteinosis. 57
13552931 1958
11
Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology. 61
33811096 2021
12
B cell-activating factors in autoimmune pulmonary alveolar proteinosis. 61
33653382 2021
13
Genetic determinants of risk in autoimmune pulmonary alveolar proteinosis. 61
33589587 2021
14
Autoimmune Pulmonary Alveolar Proteinosis Complicated with Sarcoidosis: the Clinical Course and Serum Levels of Anti-granulocyte-macrophage colony-stimulating Factor Autoantibody. 61
32611952 2020
15
Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis. 61
32897035 2020
16
Clinical significance of serum anti-granulocyte-macrophage colony-stimulating factor autoantibodies in patients with sarcoidosis and hypersensitivity pneumonitis. 61
32993757 2020
17
Autoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome. 61
32377347 2020
18
Reduction in Alveolar Macrophage Size in Refractory Autoimmune Pulmonary Alveolar Proteinosis After Treatment With Pioglitazone. 61
32569076 2020
19
A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features. 61
32336733 2020
20
Inhaled granulocyte-macrophage colony stimulating factor for mild-to-moderate autoimmune pulmonary alveolar proteinosis - a six month phase II randomized study with 24 months of follow-up. 61
32615994 2020
21
Autoimmune pulmonary alveolar proteinosis developed during immunosuppressive treatment in polymyositis with interstitial lung disease: a case report. 61
32252718 2020
22
Proteogenomic analysis of granulocyte macrophage colony- stimulating factor autoantibodies in the blood of a patient with autoimmune pulmonary alveolar proteinosis. 61
32188922 2020
23
Natural Autoantibodies in Chronic Pulmonary Diseases. 61
32046322 2020
24
Validation of a new serum granulocyte-macrophage colony-stimulating factor autoantibody testing kit. 61
32010720 2020
25
Alveolar proteinosis due to toxic inhalation at workplace. 61
32868988 2020
26
Autoimmune pulmonary alveolar proteinosis mimicking Mycoplasma pneumonia in an adolescent. 61
32518746 2020
27
Does maternal autoantibody that transfer to newborn cause disease? 61
31632678 2019
28
Pulmonary alveolar proteinosis (PAP) in idiopathic hypoparathyroidism. 61
31712233 2019
29
Removal of lung lavage fluid during whole-lung lavage using biphasic cuirass ventilation chest percussion in a patient with autoimmune pulmonary alveolar proteinosis. 61
31670303 2019
30
Inhaled GM-CSF for Pulmonary Alveolar Proteinosis. 61
31483963 2019
31
Memory B cell pool of autoimmune pulmonary alveolar proteinosis patients contains higher frequency of GM-CSF autoreactive B cells than healthy subjects. 61
31195018 2019
32
Autoimmune Pulmonary Alveolar Proteinosis Diagnosed after Exposure to a Fire Extinguisher Containing Silica Powder. 61
30996157 2019
33
Autoimmune pulmonary alveolar proteinosis in a patient with sarcoidosis. 61
30997074 2019
34
Incidence of autoimmune pulmonary alveolar proteinosis estimated using Poisson distribution. 61
30895185 2019
35
Autoimmune pulmonary alveolar proteinosis presenting peripheral ground-glass opacities. 61
30459948 2019
36
[Crazy Paving Pattern of the Lung]. 61
30536247 2019
37
Assay system development to measure the concentration of sargramostim with high specificity in patients with autoimmune pulmonary alveolar proteinosis after single-dose inhalation. 61
30003894 2018
38
Pulmonary Alveolar Proteinosis with Ulcerative Colitis. 61
29709938 2018
39
Better approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses. 61
30165864 2018
40
Pulmonary alveolar proteinosis following cryptococcal meningitis: a possible cause? 61
29592982 2018
41
Clinical significance of serum anti-GM-CSF autoantibody levels in autoimmune pulmonary alveolar proteinosis. 61
29202602 2018
42
Peripheral alveolar nitric oxide concentration reflects alveolar inflammation in autoimmune pulmonary alveolar proteinosis. 61
29577041 2018
43
Autoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim). 61
29719809 2018
44
Pathological features of explant lungs with fibrosis in autoimmune pulmonary alveolar proteinosis. 61
28748093 2017
45
Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis. 61
28966213 2017
46
Sequential Granulocyte-Macrophage Colony-Stimulating Factor Inhalation after Whole-Lung Lavage for Pulmonary Alveolar Proteinosis. A Report of Five Intractable Cases. 61
28421817 2017
47
Two cases of autoimmune pulmonary alveolar proteinosis with rheumatoid arthritis. 61
28242199 2017
48
Classification of idiopathic interstitial pneumonias using anti-myxovirus resistance-protein 1 autoantibody. 61
28230086 2017
49
A case of autoimmune pulmonary alveolar proteinosis with fluctuating lung shadows in parallel with cigarette smoke burden. 61
32476854 2017
50
Temporary remission of autoimmune pulmonary alveolar proteinosis after infectious episodes. 61
32476827 2017

Variations for Pulmonary Alveolar Proteinosis, Acquired

Expression for Pulmonary Alveolar Proteinosis, Acquired

Search GEO for disease gene expression data for Pulmonary Alveolar Proteinosis, Acquired.

Pathways for Pulmonary Alveolar Proteinosis, Acquired

GO Terms for Pulmonary Alveolar Proteinosis, Acquired

Sources for Pulmonary Alveolar Proteinosis, Acquired

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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