MCID: ACT029
MIFTS: 42

Acute Interstitial Pneumonia

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Acute Interstitial Pneumonia

MalaCards integrated aliases for Acute Interstitial Pneumonia:

Name: Acute Interstitial Pneumonia 12 53 15 73
Hamman-Rich Syndrome 12 53 73
Acute Interstitial Pneumonitis 12 53
Idiopathic Pulmonary Fibrosis, Acute Fatal Form 12
Accelerated Interstitial Pneumonia 12
Idiopathic Pulmonary Fibrosis 73
Hamman-Rich Disease 12
Aip 12

Classifications:



External Ids:

Disease Ontology 12 DOID:2800
ICD10 33 J84.114
ICD9CM 35 516.33
NCIt 50 C35806
UMLS 73 C1279945

Summaries for Acute Interstitial Pneumonia

NIH Rare Diseases : 53 Acute interstitial pneumonia (AIP) is a rare and serious condition that affects the lungs. The signs and symptoms generally develop and progress rapidly. In the early stages of the condition, affected people may experience upper respiratory and/or viral-like symptoms such as cough, shortness of breath, and fever. This is followed by the rapid onset of respiratory failure and the need for mechanical ventilation in the majority of cases. The underlying cause of AIP is unknown. Most cases occur sporadically in people with no family history of the condition. There is, unfortunately, no proven treatment for AIP. Supportive care is generally recommended to address the signs and symptoms of the condition. Glucocorticoid therapy and other immunosuppressive therapies are often used; however, the benefits of these treatments remain unclear.

MalaCards based summary : Acute Interstitial Pneumonia, also known as hamman-rich syndrome, is related to pulmonary fibrosis, idiopathic and pulmonary fibrosis, and has symptoms including dyspnea on exertion and dry cough. An important gene associated with Acute Interstitial Pneumonia is SFTPD (Surfactant Protein D), and among its related pathways/superpathways are Bacterial infections in CF airways and Phagosome. The drugs Cabergoline and Dopamine have been mentioned in the context of this disorder. Affiliated tissues include lung and placenta, and related phenotype is Increased virus production.

Disease Ontology : 12 A idiopathic interstitial pneumonia which develops suddenly and is severe. Initially, the lung shows edema, hyaline membranes, and interstitial acute inflammation. Later,it develops loose organizing fibrosis, mostly within alveolar septa and type II pneumocyte hyperplasia. Fever, cough, and difficulty breathing develop over 1 to 2 weeks, typically progressing to acute respiratory failure.

Related Diseases for Acute Interstitial Pneumonia

Diseases related to Acute Interstitial Pneumonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 83)
# Related Disease Score Top Affiliating Genes
1 pulmonary fibrosis, idiopathic 30.8 MUC1 SFTPA1 SFTPA2 SFTPD
2 pulmonary fibrosis 29.5 SFTPA1 SFTPA2 SFTPD
3 aip-related familial isolated pituitary adenomas 12.1
4 porphyria, acute intermittent 12.1
5 pituitary adenoma 1, multiple types 11.9
6 acromegaly 11.8
7 autoimmune pancreatitis 11.8
8 pituitary adenoma, prolactin-secreting 11.6
9 adenoma 11.6
10 pituitary adenoma 11.6
11 familial isolated pituitary adenoma 11.5
12 growth hormone secreting pituitary adenoma 11.5
13 acroleukopathy, symmetric 11.4
14 gigantism 11.4
15 pituitary tumors 11.4
16 pituitary apoplexy 11.3
17 hepatitis b 11.1
18 chester porphyria 11.0
19 carney complex variant 10.9
20 multiple endocrine neoplasia, type iv 10.9
21 cell type benign neoplasm 10.9
22 organ system benign neoplasm 10.9
23 endocrine organ benign neoplasm 10.9
24 hyperpituitarism 10.9
25 functioning pituitary adenoma 10.9
26 hormone producing pituitary cancer 10.9
27 cancer-associated retinopathy 10.9
28 autoimmune pancreatitis type 2 10.9
29 autoimmune pancreatitis type 1 10.9
30 acth-secreting pituitary adenoma 10.9
31 silent pituitary adenoma 10.8
32 null pituitary adenoma 10.8
33 pneumonia 10.6
34 porphyria 10.2
35 pancreatitis 10.2
36 splenic artery aneurysm 10.0 SFTPA1 SFTPA2
37 pleuroparenchymal fibroelastosis 10.0 MUC1 SFTPD
38 thymus adenocarcinoma 10.0 SFTPA1 SFTPA2
39 rheumatoid arthritis 10.0
40 arthritis 10.0
41 acute respiratory distress syndrome 10.0
42 allergic bronchopulmonary aspergillosis 10.0 SFTPA2 SFTPD
43 multiple endocrine neoplasia 10.0
44 poland syndrome 10.0 SFTPA1 SFTPA2
45 pneumocystosis 9.9 MUC1 SFTPD
46 dermatomyositis 9.9
47 antiphospholipid syndrome 9.9
48 lung sarcoma 9.9 SFTPA1 SFTPA2
49 aspergillosis 9.8 SFTPA2 SFTPD
50 bronchiolitis obliterans 9.8

Graphical network of the top 20 diseases related to Acute Interstitial Pneumonia:



Diseases related to Acute Interstitial Pneumonia

Symptoms & Phenotypes for Acute Interstitial Pneumonia

UMLS symptoms related to Acute Interstitial Pneumonia:


dyspnea on exertion, dry cough

GenomeRNAi Phenotypes related to Acute Interstitial Pneumonia according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased virus production GR00378-A 8.62 MUC1 SFTPD

Drugs & Therapeutics for Acute Interstitial Pneumonia

Drugs for Acute Interstitial Pneumonia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cabergoline Approved 81409-90-7 54746
2
Dopamine Approved 51-61-6, 62-31-7 681
3
Levodopa Approved 59-92-7 6047
4 Autonomic Agents
5 Dopamine Agents
6 Dopamine agonists
7 Neurotransmitter Agents
8 Peripheral Nervous System Agents
9 Protective Agents
10 Antibodies
11 Immunoglobulins

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo
2 The Association Between Dopamine Agonists and Cardiac Valvulopathy, Fibrosis and Other Cardiopulmonary Events Completed NCT01052948
3 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416

Search NIH Clinical Center for Acute Interstitial Pneumonia

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Genetic Tests for Acute Interstitial Pneumonia

Anatomical Context for Acute Interstitial Pneumonia

MalaCards organs/tissues related to Acute Interstitial Pneumonia:

41
Lung, Placenta

Publications for Acute Interstitial Pneumonia

Articles related to Acute Interstitial Pneumonia:

(show top 50) (show all 85)
# Title Authors Year
1
Pneumocystis jirovecii infection in patients with acute interstitial pneumonia. ( 29891174 )
2018
2
Acute interstitial pneumonia as first presentation of anti-synthetase syndrome: an atypical case. ( 29644083 )
2018
3
Extracorporeal Membrane Oxygenation for Refractory Severe Respiratory Failure in Acute Interstitial Pneumonia. ( 29323413 )
2018
4
A 45-Year-Old Woman with Acute Interstitial Pneumonia (Hamman-Rich Syndrome). ( 28868178 )
2017
5
Abruptio Placentae with Type II Respiratory Failure Secondary to Acute Interstitial Pneumonia Responsive to Steroids. ( 28969741 )
2017
6
Successful treatment of gemcitabine-induced acute interstitial pneumonia with imatinib mesylate: a case report. ( 27733144 )
2016
7
Feedlot Acute Interstitial Pneumonia. ( 26253266 )
2015
8
Leflunomide-induced acute interstitial pneumonia in a patient treated for rheumatoid arthritis. ( 25577534 )
2015
9
Acute interstitial pneumonia caused by interleukin-2 and interferon I+-2b therapy for renal cell carcinoma: a case report and clinical study. ( 25430469 )
2014
10
A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan. ( 24533549 )
2014
11
Concurrent acute interstitial pneumonia and pulmonary embolism during treatment with peginterferon alpha-2a and ribavirin in a patient with hepatitis C. ( 25097288 )
2014
12
High-resolution computed tomography findings of acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis. ( 24480142 )
2014
13
Acute interstitial pneumonia following heroin inhalation. ( 25065663 )
2014
14
Alveolar epithelial cells undergo epithelial-mesenchymal transition in acute interstitial pneumonia: a case report. ( 24755111 )
2014
15
Serum heat shock protein 47 levels are elevated in acute interstitial pneumonia. ( 24650086 )
2014
16
[Acute interstitial pneumonia in patient with rheumatoid arthritis treated with leflunomide]. ( 25339568 )
2014
17
A fatal Case of Acute Interstitial Pneumonia (AIP) in a Woman Affected by Glioblastoma. ( 24410308 )
2014
18
Successful combination therapy with corticosteroids, biweekly intravenous pulse cyclophosphamide and cyclosporin A for acute interstitial pneumonia in patients with dermatomyositis : report of three cases. ( 23629432 )
2013
19
Efficacy of infliximab in the treatment for dermatomyositis with acute interstitial pneumonia: a study of fourteen cases and literature review. ( 23715693 )
2013
20
A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan. ( 23553659 )
2013
21
Clinical management of acute interstitial pneumonia: a case report. ( 23316404 )
2012
22
Sorafenib-induced acute interstitial pneumonia in patients with advanced hepatocellular carcinoma: report of three cases. ( 24672586 )
2012
23
Acute interstitial pneumonia (AIP): relationship to Hamman-Rich syndrome, diffuse alveolar damage (DAD), and acute respiratory distress syndrome (ARDS). ( 23001802 )
2012
24
Coadministration of cyclosporin a with prednisolone in acute interstitial pneumonia complicating polymyositis/dermatomyositis. ( 22442637 )
2012
25
Pathological changes preceding radiological changes in a patient with acute interstitial pneumonia. ( 23257539 )
2012
26
Lymphatic fluctuation in the parenchymal remodeling stage of acute interstitial pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis. ( 22488224 )
2012
27
Fatal acute interstitial pneumonia in a worker making chips from wooden debris generated by the Great East Japan earthquake and tsunami. ( 23199976 )
2012
28
Acute interstitial pneumonia (Hamman-Rich syndrome) in idiopathic pulmonary fibrosis and bronchoalveolar carcinoma: a case report. ( 22336655 )
2011
29
Acute interstitial pneumonia (hamman-rich syndrome) as a cause of idiopathic acute respiratory distress syndrome. ( 21687544 )
2011
30
Successful erlotinib rechallenge after gefitinib-induced acute interstitial pneumonia. ( 20581580 )
2010
31
Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. ( 19095855 )
2009
32
Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis? ( 18399864 )
2008
33
Acute interstitial pneumonia in siblings: a case report. ( 18583894 )
2008
34
Acute interstitial pneumonia in feedlot cattle: effects of feeding feather meal or vitamin E. ( 17479779 )
2007
35
Leflunomide-related acute interstitial pneumonia in two patients with rheumatoid arthritis: autopsy findings with a mosaic pattern of acute and organizing diffuse alveolar damage. ( 17039315 )
2006
36
Acute interstitial pneumonia and acute exacerbations of idiopathic pulmonary fibrosis. ( 17195142 )
2006
37
Acute interstitial pneumonia following surgery for primary lung cancer. ( 16893654 )
2006
38
Early intervention can improve clinical outcome of acute interstitial pneumonia. ( 16537878 )
2006
39
Effect of dietary melengestrol acetate on the incidence of acute interstitial pneumonia in feedlot heifers. ( 16850945 )
2006
40
Leflunomide induced acute interstitial pneumonia. ( 15940779 )
2005
41
A case of lepidic pulmonary metastasis from adenocarcinoma of the gallbladder mimicking acute interstitial pneumonia. ( 16223618 )
2005
42
Rheumatoid arthritis complicated with acute interstitial pneumonia induced by leflunomide as an adverse reaction. ( 15645660 )
2004
43
Acute interstitial pneumonia. ( 15564019 )
2004
44
Microbiologic findings in feedlot cattle with acute interstitial pneumonia. ( 15566091 )
2004
45
Video-assisted thoracoscopic lung biopsy as a possible cause of acute interstitial pneumonia in a patient with nonspecific interstitial pneumonia. ( 15510251 )
2004
46
Severe acute interstitial pneumonia and gefitinib. ( 12531582 )
2003
47
Acute interstitial pneumonia: report of a series. ( 12570127 )
2003
48
Low hospital mortality in patients with acute interstitial pneumonia. ( 12907542 )
2003
49
Acute interstitial pneumonia: comparison of high-resolution computed tomography findings between survivors and nonsurvivors. ( 12045132 )
2002
50
Nitric oxide and prostacyclin in acute interstitial pneumonia. ( 11773351 )
2002

Variations for Acute Interstitial Pneumonia

Expression for Acute Interstitial Pneumonia

Search GEO for disease gene expression data for Acute Interstitial Pneumonia.

Pathways for Acute Interstitial Pneumonia

Pathways related to Acute Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.76 MUC1 SFTPA1 SFTPD
2 11.68 SFTPA1 SFTPA2 SFTPD
3
Show member pathways
11.4 SFTPA1 SFTPA2 SFTPD
4 11.31 SFTPA1 SFTPA2
5 11.2 SFTPA1 SFTPA2
6
Show member pathways
10.99 SFTPA1 SFTPA2 SFTPD
7
Show member pathways
10.77 SFTPA1 SFTPA2 SFTPD
8
Show member pathways
10.2 SFTPA1 SFTPA2 SFTPD

GO Terms for Acute Interstitial Pneumonia

Cellular components related to Acute Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.71 MUC1 SFTPA1 SFTPA2 SFTPD
2 endoplasmic reticulum membrane GO:0005789 9.67 SFTPA1 SFTPA2 SFTPD SPTLC1
3 collagen trimer GO:0005581 9.5 SFTPA1 SFTPA2 SFTPD
4 rough endoplasmic reticulum GO:0005791 9.43 SFTPA1 SFTPA2 SFTPD
5 clathrin-coated endocytic vesicle GO:0045334 9.33 SFTPA1 SFTPA2 SFTPD
6 multivesicular body GO:0005771 9.13 SFTPA1 SFTPA2 SFTPD
7 lamellar body GO:0042599 8.8 SFTPA1 SFTPA2 SFTPD

Biological processes related to Acute Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.54 SFTPA1 SFTPA2 SFTPD
2 toll-like receptor signaling pathway GO:0002224 9.5 SFTPA1 SFTPA2 SFTPD
3 developmental process GO:0032502 9.43 SFTPA1 SFTPA2 SFTPD
4 respiratory gaseous exchange GO:0007585 9.33 SFTPA1 SFTPA2 SFTPD
5 positive regulation of phagocytosis GO:0050766 9.13 SFTPA1 SFTPA2 SFTPD
6 surfactant homeostasis GO:0043129 8.8 SFTPA1 SFTPA2 SFTPD

Molecular functions related to Acute Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 carbohydrate binding GO:0030246 9.33 SFTPA1 SFTPA2 SFTPD
2 lipopolysaccharide binding GO:0001530 9.13 SFTPA1 SFTPA2 SFTPD
3 monosaccharide binding GO:0048029 8.8 SFTPA1 SFTPA2 SFTPD

Sources for Acute Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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