General Information of Disease (ID: DISFSOM3)

Disease Name Pneumocystis pneumonia
Synonyms
pneumocystosis pneumonia; Pneumocystis carinii pneumonia; PCP; Pneumocystis; pneumocystosis; Pneumocystis pneumonia; pulmonary pneumocystosis; Pneumocystis Pneumonia; Pneumocystis jirovecii pneumonia; PJP
Disease Class CA40: Pneumonia
Definition
Human pneumocystosis is caused by an infectious agent, which (after recent nomenclature and taxonomy revisions) is now classed as the fungus Pneumocystis jiroveci. The prevalence is unknown. Pneumocystis jiroveci is an opportunistic infectious agent, developing in immunosuppressed patients. It is an air-borne infection, localized to the lungs. However, extrapulmonary involvement is seen in AIDS patients. The disease manifests progressively with coughing, respiratory problems (dyspnea) and fever, followed by acute respiratory insufficiency and death within a few weeks in untreated cases. The most reliable diagnostic method is bronchoalveolar lavage. The treatment of choice is cotrimoxazole.
Disease Hierarchy
DIS8EF3M: Pneumonia
DISH0JV6: Fungal lung infectious disease
DIS67GEL: Opportunistic mycosis
DISOX2NY: Pneumocystis infectious disease
DISFSOM3: Pneumocystis pneumonia
ICD Code
ICD-11
ICD-11: CA40.20
ICD-10
ICD-10: B59
Disease Identifiers
MONDO ID
MONDO_0019121
MESH ID
D011020
UMLS CUI
C1535939
MedGen ID
777048
HPO ID
HP:0020102
Orphanet ID
723
SNOMED CT ID
415125002

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 7 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Dapsone DM4LT8A Approved Small molecular drug [1]
Levoleucovorin DMH5LME Approved Small molecular drug [2]
Methylprednisolone DM4BDON Approved Small molecular drug [3]
Pentamidine DMHZJCG Approved Small molecular drug [4]
Prednisolone DMQ8FR2 Approved Small molecular drug [5]
Sulfamethoxazole DMB08GE Approved Small molecular drug [6]
Trimethoprim DMM7CHK Approved Small molecular drug [7]
------------------------------------------------------------------------------------
⏷ Show the Full List of 7 Drug(s)
This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Aerucin DMCK28R Phase 2 Antibody [8]
------------------------------------------------------------------------------------
This Disease is Treated as An Indication in 2 Discontinued Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
CGP-40215A DM69YWS Terminated Small molecular drug [9]
FR-901379 DM0VR6H Terminated NA [10]
------------------------------------------------------------------------------------

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 11 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
CEL TTTRNQW Limited Genetic Variation [12]
CLEC6A TTQ02HK Limited Biomarker [13]
DAAM2 TTN0Z6H Limited Biomarker [14]
NEK2 TT3VZ24 Limited Biomarker [15]
PKN1 TTSL41O Limited Posttranslational Modification [16]
RORC TTGV6LY Limited Genetic Variation [17]
AGRP TT4DE1O Strong Biomarker [18]
C3 TTJGY7A Strong Biomarker [19]
DHFR TTYZVDJ Strong Genetic Variation [20]
DHPS TTBO2A9 Strong Genetic Variation [21]
ROR2 TTUDPCI Strong Biomarker [22]
------------------------------------------------------------------------------------
⏷ Show the Full List of 11 DTT(s)
This Disease Is Related to 2 DTP Molecule(s)
Gene Name DTP ID Evidence Level Mode of Inheritance REF
SLC27A5 DT0TQS3 Limited Genetic Variation [12]
SLC9A6 DTN0JXW Strong Biomarker [18]
------------------------------------------------------------------------------------
This Disease Is Related to 2 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
MAT1A DEQ6NC9 Limited Genetic Variation [23]
UAP1 DETYWG5 Limited Altered Expression [24]
------------------------------------------------------------------------------------
This Disease Is Related to 39 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
ALKBH3 OTS1CD9Z Limited Biomarker [25]
APOBEC3F OT750IJM Limited Genetic Variation [26]
ATMIN OTEADE76 Limited Biomarker [27]
BTBD8 OT3A3RD7 Limited Genetic Variation [28]
CCRL2 OT5PX0RX Limited Biomarker [29]
CLEC4E OT7P8ICY Limited Biomarker [30]
DAAM1 OT0VHIYZ Limited Biomarker [14]
GSC OT4DH7PR Limited Biomarker [31]
LRRFIP1 OTN7XAUD Limited Biomarker [32]
LURAP1 OTJ1EZDJ Limited Biomarker [33]
MPP1 OTA2ENZQ Limited Biomarker [34]
NR6A1 OTFZOOQ9 Limited Genetic Variation [35]
OFD1 OTAZW5TK Limited Biomarker [36]
PARP9 OT7K4494 Limited Genetic Variation [12]
PKHD1 OTAH8SMF Limited Altered Expression [27]
PLEKHA4 OTNOCQTF Limited Biomarker [37]
POLG2 OTDBMZJB Limited Biomarker [34]
PRICKLE1 OT9HHEM9 Limited Biomarker [38]
PSMD12 OTWICA51 Limited Biomarker [34]
RECQL4 OT59LSW7 Limited Genetic Variation [39]
SEC24B OT6W8CWP Limited Genetic Variation [40]
SMURF1 OT5UIZR8 Limited Biomarker [41]
STATH OTQHBHM9 Limited Biomarker [42]
TES OTL8PP6V Limited Biomarker [38]
CYTB OTAHB98A Disputed Biomarker [43]
ARTN OTWIWGL6 Strong Biomarker [18]
AZIN1 OTX5W77I Strong Biomarker [44]
CELSR1 OT7PS8O1 Strong Genetic Variation [45]
CLEC7A OTRTBH27 Strong Biomarker [46]
EMP1 OTSZHUHQ Strong Biomarker [47]
FZD6 OTBCPII8 Strong Altered Expression [48]
HLA-DQB1 OTVVI3UI Strong Biomarker [49]
MAX OTKZ0YKM Strong Biomarker [50]
MUC5B OTPW6K5C Strong Biomarker [51]
SFTPA2 OT6SFOMU Strong Altered Expression [52]
SFTPB OTOHS07E Strong Altered Expression [52]
CELSR2 OTON6JSZ Definitive Genetic Variation [53]
DVL3 OTPRROHJ Definitive Genetic Variation [53]
SCRIB OTW4N3FV Definitive Genetic Variation [54]
------------------------------------------------------------------------------------
⏷ Show the Full List of 39 DOT(s)

References

1 The fight against drug-resistant malaria: novel plasmodial targets and antimalarial drugs. Curr Med Chem. 2008;15(2):161-71.
2 Levoleucovorin FDA Label
3 Methylprednisolone FDA Label
4 Pentamidine FDA Label
5 Prednisolone FDA Label
6 Sulfamethoxazole FDA Label
7 Trimethoprim FDA Label
8 ClinicalTrials.gov (NCT03027609) Adjunctive Therapeutic Treatment With Human Monoclonal Antibody AR-105 (Aerucin) in P. Aeruginosa Pneumonia. U.S. National Institutes of Health.
9 Antileishmanial effect of a potent S-adenosylmethionine decarboxylase inhibitor: CGP 40215A. Pharmacol Res. 1996 Jan;33(1):67-70.
10 Trusted, scientifically sound profiles of drug programs, clinical trials, safety reports, and company deals, written by scientists. Springer. 2015. Adis Insight (drug id 800006606)
11 Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
12 Diagnosis of Pneumocystis pneumonia by real-time PCR in patients with various underlying diseases.J Microbiol Immunol Infect. 2020 Oct;53(5):785-790. doi: 10.1016/j.jmii.2019.08.012. Epub 2019 Oct 7.
13 Dectin-2 Is a C-Type Lectin Receptor that Recognizes Pneumocystis and Participates in Innate Immune Responses.Am J Respir Cell Mol Biol. 2018 Feb;58(2):232-240. doi: 10.1165/rcmb.2016-0335OC.
14 WNT/PCP signaling pathway and human cancer (review).Oncol Rep. 2005 Dec;14(6):1583-8.
15 Nemo-like kinase 1 (Nlk1) and paraxial protocadherin (PAPC) cooperatively control Xenopus gastrulation through regulation of Wnt/planar cell polarity (PCP) signaling.Differentiation. 2017 Jan-Feb;93:27-38. doi: 10.1016/j.diff.2016.10.002. Epub 2016 Oct 28.
16 Cdc42 and RhoB activation are required for mannose receptor-mediated phagocytosis by human alveolar macrophages.Mol Biol Cell. 2005 Feb;16(2):824-34. doi: 10.1091/mbc.e04-06-0463. Epub 2004 Dec 1.
17 m-TOR inhibitors and risk of Pneumocystis pneumonia after solid organ transplantation: a systematic review and meta-analysis.Eur J Clin Pharmacol. 2019 Nov;75(11):1471-1480. doi: 10.1007/s00228-019-02730-0. Epub 2019 Aug 3.
18 Paediatric deaths in a tertiary government hospital setting, Malawi.Paediatr Int Child Health. 2019 Nov;39(4):240-248. doi: 10.1080/20469047.2018.1536873. Epub 2018 Nov 19.
19 Expression and activation of complement protein and alveolar damage during Pneumocystis pneumonia.J Eukaryot Microbiol. 2006;53 Suppl 1:S136-7. doi: 10.1111/j.1550-7408.2006.00204.x.
20 Genetic polymorphisms associated with treatment failure and mortality in pediatric Pneumocystosis.Sci Rep. 2019 Feb 4;9(1):1192. doi: 10.1038/s41598-018-38052-x.
21 Novel dihydropteroate synthase gene mutation in Pneumocystis jirovecii among HIV-infected patients in India: Putative association with drug resistance and mortality.J Glob Antimicrob Resist. 2019 Jun;17:236-239. doi: 10.1016/j.jgar.2019.01.007. Epub 2019 Jan 15.
22 Wnt/PCP controls spreading of Wnt/-catenin signals by cytonemes in vertebrates.Elife. 2018 Jul 31;7:e36953. doi: 10.7554/eLife.36953.
23 Comparative genomics suggests primary homothallism of Pneumocystis species.mBio. 2015 Jan 13;6(1):e02250-14. doi: 10.1128/mBio.02250-14.
24 Characterization of N-Acetylglucosamine Biosynthesis in Pneumocystis species. A New Potential Target for Therapy.Am J Respir Cell Mol Biol. 2017 Feb;56(2):213-222. doi: 10.1165/rcmb.2016-0155OC.
25 Immunization with Pneumocystis Cross-Reactive Antigen 1 (Pca1) Protects Mice against Pneumocystis Pneumonia and Generates Antibody to Pneumocystis jirovecii.Infect Immun. 2017 Mar 23;85(4):e00850-16. doi: 10.1128/IAI.00850-16. Print 2017 Apr.
26 Role of APOBEC3F Gene Variation in HIV-1 Disease Progression and Pneumocystis Pneumonia.PLoS Genet. 2016 Mar 4;12(3):e1005921. doi: 10.1371/journal.pgen.1005921. eCollection 2016 Mar.
27 Atmin modulates Pkhd1 expression and may mediate Autosomal Recessive Polycystic Kidney Disease (ARPKD) through altered non-canonical Wnt/Planar Cell Polarity (PCP) signalling.Biochim Biophys Acta Mol Basis Dis. 2019 Feb 1;1865(2):378-390. doi: 10.1016/j.bbadis.2018.11.003. Epub 2018 Nov 7.
28 Pneumocystis jirovecii pneumonia (PCP) PCR-negative conversion predicts prognosis of HIV-negative patients with PCP and acute respiratory failure.PLoS One. 2018 Oct 25;13(10):e0206231. doi: 10.1371/journal.pone.0206231. eCollection 2018.
29 Role of exonic variation in chemokine receptor genes on AIDS: CCRL2 F167Y association with pneumocystis pneumonia.PLoS Genet. 2011 Oct;7(10):e1002328. doi: 10.1371/journal.pgen.1002328. Epub 2011 Oct 27.
30 The Interaction of Pneumocystis with the C-Type Lectin Receptor Mincle Exerts a Significant Role in Host Defense against Infection.J Immunol. 2017 May 1;198(9):3515-3525. doi: 10.4049/jimmunol.1600744. Epub 2017 Mar 15.
31 Transcriptomic and Proteomic Approaches to Finding Novel Diagnostic and Immunogenic Candidates in Pneumocystis.mSphere. 2019 Sep 4;4(5):e00488-19. doi: 10.1128/mSphere.00488-19.
32 GC-binding factor 2 interacts with dishevelled and regulates Wnt signaling pathways in human carcinoma cell lines.Int J Cancer. 2011 Oct 1;129(7):1599-610. doi: 10.1002/ijc.25837. Epub 2011 Mar 8.
33 Leucine repeat adaptor protein 1 interacts with Dishevelled to regulate gastrulation cell movements in zebrafish.Nat Commun. 2017 Nov 7;8(1):1353. doi: 10.1038/s41467-017-01552-x.
34 Synthetic p55 tandem DNA vaccine against Pneumocystis carinii in rats.Microbiol Immunol. 2016 Jun;60(6):397-406. doi: 10.1111/1348-0421.12386.
35 Outbreak of pneumocystis pneumonia in renal and liver transplant patients caused by genotypically distinct strains of Pneumocystis jirovecii.Transplantation. 2013 Nov 15;96(9):834-42. doi: 10.1097/TP.0b013e3182a1618c.
36 Convergent extension movements and ciliary function are mediated by ofd1, a zebrafish orthologue of the human oral-facial-digital type 1 syndrome gene.Hum Mol Genet. 2009 Jan 15;18(2):289-303. doi: 10.1093/hmg/ddn356. Epub 2008 Oct 29.
37 PLEKHA4/kramer Attenuates Dishevelled Ubiquitination to Modulate Wnt and Planar Cell Polarity Signaling.Cell Rep. 2019 May 14;27(7):2157-2170.e8. doi: 10.1016/j.celrep.2019.04.060.
38 Prickle1 regulates neurite outgrowth of apical spiral ganglion neurons but not hair cell polarity in the murine cochlea.PLoS One. 2017 Aug 24;12(8):e0183773. doi: 10.1371/journal.pone.0183773. eCollection 2017.
39 Successful umbilical cord blood stem cell transplantation in a patient with Rothmund-Thomson syndrome and combined immunodeficiency.Clin Genet. 2006 Apr;69(4):337-43. doi: 10.1111/j.1399-0004.2006.00592.x.
40 Planar cell polarity defects and defective Vangl2 trafficking in mutants for the COPII gene Sec24b.Development. 2010 Apr;137(7):1067-73. doi: 10.1242/dev.041434.
41 The E3 ubiquitin ligase SMURF1 regulates cell-fate specification and outflow tract septation during mammalian heart development.Sci Rep. 2018 Jun 22;8(1):9542. doi: 10.1038/s41598-018-27854-8.
42 Diversity of Pneumocystis jirovecii Across Europe: A Multicentre Observational Study.EBioMedicine. 2017 Aug;22:155-163. doi: 10.1016/j.ebiom.2017.06.027. Epub 2017 Jun 29.
43 Polymorphisms involving the Pneumocystis jirovecii-related genes in AIDS patients in eastern China.Infect Genet Evol. 2019 Nov;75:103955. doi: 10.1016/j.meegid.2019.103955. Epub 2019 Jul 5.
44 Pneumocystis mediates overexpression of antizyme inhibitor resulting in increased polyamine levels and apoptosis in alveolar macrophages.J Biol Chem. 2009 Mar 20;284(12):8174-84. doi: 10.1074/jbc.M805787200. Epub 2009 Jan 20.
45 Spina bifida-predisposing heterozygous mutations in Planar Cell Polarity genes and Zic2 reduce bone mass in young mice.Sci Rep. 2018 Feb 20;8(1):3325. doi: 10.1038/s41598-018-21718-x.
46 The 14th International Workshops on Opportunistic Protists (IWOP 14).J Eukaryot Microbiol. 2018 Nov;65(6):934-939. doi: 10.1111/jeu.12631. Epub 2018 May 25.
47 Anidulafungin as an alternative treatment for Pneumocystis jirovecii pneumonia in patients who cannot tolerate trimethoprim/sulfamethoxazole.Int J Antimicrob Agents. 2020 Jan;55(1):105820. doi: 10.1016/j.ijantimicag.2019.10.001. Epub 2019 Oct 14.
48 Molecular cloning, characterization and expression analysis of Frizzled 6 in the small intestine of pigs (Sus scrofa).PLoS One. 2017 Jun 14;12(6):e0179421. doi: 10.1371/journal.pone.0179421. eCollection 2017.
49 Microarray studies on effects of Pneumocystis carinii infection on global gene expression in alveolar macrophages.BMC Microbiol. 2010 Apr 8;10:103. doi: 10.1186/1471-2180-10-103.
50 Evaluation of the automated Becton Dickinson MAX real-time PCR platform for detection of Pneumocystis jirovecii.Future Microbiol. 2017 Jan;12:29-37. doi: 10.2217/fmb-2016-0115. Epub 2016 Dec 12.
51 Increase in secreted airway mucins and partial Muc5b STAT6/FoxA2 regulation during Pneumocystis primary infection.Sci Rep. 2019 Feb 14;9(1):2078. doi: 10.1038/s41598-019-39079-4.
52 Inhibition of lung surfactant protein B expression during Pneumocystis carinii pneumonia in mice.J Lab Clin Med. 1999 May;133(5):423-33. doi: 10.1016/s0022-2143(99)90019-7.
53 Genetic analysis of Wnt/PCP genes in neural tube defects.BMC Med Genomics. 2018 Apr 4;11(1):38. doi: 10.1186/s12920-018-0355-9.
54 Scribble1 plays an important role in the pathogenesis of neural tube defects through its mediating effect of Par-3 and Vangl1/2 localization.Hum Mol Genet. 2017 Jun 15;26(12):2307-2320. doi: 10.1093/hmg/ddx122.