Pneumocystosis

disease
On this page

Also known as PCPPJPPneumocystisPneumocystis carinii pneumoniaPneumocystis jirovecii pneumoniaPneumocystis Pneumonia

Summary

Pneumocystosis (MONDO:0019121) is a disease and 32 clinical trials. Top therapeutic interventions include caspofungin, clindamycin, and primaquine. A subtype of opportunistic mycosis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: Unknown (Worldwide) [Orphanet-validated]
  • Phenotypes (HPO): 23
  • Clinical trials: 32

Clinical features

Signs & symptoms

Clinical features (HPO)

23 HPO clinical features (Orphanet curated; top 23 by frequency):

HPO IDTermFrequency
HP:0001945FeverVery frequent (80-99%)
HP:0002094DyspneaVery frequent (80-99%)
HP:0011949Acute infectious pneumoniaVery frequent (80-99%)
HP:0012418HypoxemiaVery frequent (80-99%)
HP:0020102Pneumocystis jirovecii pneumoniaVery frequent (80-99%)
HP:0001824Weight lossFrequent (30-79%)
HP:0002093Respiratory insufficiencyFrequent (30-79%)
HP:0002202Pleural effusionFrequent (30-79%)
HP:0002721ImmunodeficiencyFrequent (30-79%)
HP:0002875Exertional dyspneaFrequent (30-79%)
HP:0002878Respiratory failureFrequent (30-79%)
HP:0004887Respiratory failure requiring assisted ventilationFrequent (30-79%)
HP:0006515Interstitial pneumonitisFrequent (30-79%)
HP:0010702Increased circulating antibody levelFrequent (30-79%)
HP:0011991Abnormal neutrophil countFrequent (30-79%)
HP:0025395Combined cystic and ground-glass pattern on pulmonary HRCTFrequent (30-79%)
HP:0031246Nonproductive coughFrequent (30-79%)
HP:0002664NeoplasmOccasional (5-29%)
HP:0005948Multiple pulmonary cystsOccasional (5-29%)
HP:0009098Chronic oral candidiasisOccasional (5-29%)
HP:0025435Increased circulating lactate dehydrogenase concentrationOccasional (5-29%)
HP:0031863Bloodstream infectious agentOccasional (5-29%)
HP:0032177Parenchymal consolidationOccasional (5-29%)

Identifiers

Disease identifiers

FieldValue
Canonical namepneumocystosis
Mondo IDMONDO:0019121
EFOEFO:0007448
MeSHD011020
Orphanet723
DOIDDOID:11339
ICD-10-CMB59
ICD-11404370038
NCITC3334
SNOMED CT415125002
UMLSC1535939
MedGen777048
GARD0004386
NORD1923
Is cancer (heuristic)no

Also known as: PCP · PJP · Pneumocystis · Pneumocystis carinii pneumonia · Pneumocystis jirovecii pneumonia · Pneumocystis Pneumonia · Pneumocystis pneumonia · pneumocystosis

Disease family

This is a subtype of opportunistic mycosis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › disease of primarily extrinsic mechanism › infectious diseasefungal infectious diseaseopportunistic mycosispneumocystosis

Related subtypes (11): phaeohyphomycosis, candidiasis, maple bark strippers’ lung, aspergillosis, cryptococcosis, geotrichosis, microsporidiosis, fusariosis, Zygomycosis, opportunistic systemic mycosis, hyalohyphomycosis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

3 approved, 10 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugDevelopment status
AtovaquoneApproved (phase 4)
SulfamethoxazoleApproved (phase 4)
TrimethoprimApproved (phase 4)
ClindamycinPhase 3 (in late-stage trials)
CobicistatPhase 3 (in late-stage trials)
DapsonePhase 3 (in late-stage trials)
DarunavirPhase 3 (in late-stage trials)
HydroxychloroquinePhase 3 (in late-stage trials)
MethylprednisolonePhase 3 (in late-stage trials)
PrimaquinePhase 3 (in late-stage trials)
PyrimethaminePhase 3 (in late-stage trials)
SulfadoxinePhase 3 (in late-stage trials)
ZidovudinePhase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Co-Trimoxazole.

Clinical trials & evidence

Clinical trials

Clinical trials: 32.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified18
PHASE47
PHASE34
PHASE21
PHASE1/PHASE21
PHASE11

Top trials by phase / activity

NCTPhaseStatusTitle
NCT07328984PHASE4NOT_YET_RECRUITINGHow Long Should we Give Steroids for Patients With Severe PCP
NCT07357103PHASE4NOT_YET_RECRUITINGPositioning Second-line Therapies for Pneumocystis Jirovecii Pneumonia (PCP Alternatives)
NCT00636935PHASE4WITHDRAWNCorticosteroids Therapy and Pneumocystis Jirovecii Pneumonia (PCP)
NCT01167452PHASE4COMPLETEDEffect of Weight and/or Obesity on Sulfamethoxazole and Trimethoprim Concentrations
NCT02550080PHASE4UNKNOWNClinical Utility Of Genetic Screening For HLA-B*1301, On Susceptibility To Dapsone Hypersensitivity Syndrome
NCT03087890PHASE4COMPLETEDImpact of Cotrimoxazole Use in Immunocompetent HIV Patients on Carriage of Antimicrobial Resistant Bacteria
NCT03978559PHASE4UNKNOWNTrimethoprim/Sulfamethoxazole Combined With Caspofungin as First-line Therapy in PCP
NCT04851015PHASE3RECRUITINGLow Dose Trimethoprim-Sulfamethoxazole for the Treatment of Pneumocystis Jirovecii Pneumonia
NCT00302341PHASE3TERMINATEDDB289 Versus TMP-SMX for the Treatment of Acute Pneumocystis Jiroveci Pneumonia (PCP)
NCT02944045PHASE3UNKNOWNEffect of Steroids During Pneumocystis Infection Among Non HIV Immunocompromised Patients
NCT04368559PHASE3COMPLETEDStudy of Rezafungin Compared to Standard Antimicrobial Regimen for Prevention of Invasive Fungal Diseases in Adults Undergoing Allogeneic Blood and Marrow Transplantation
NCT05835479PHASE2RECRUITINGRezafungin for Treatment of Pneumocystis Pneumonia in HIV Adults
NCT06892951PHASE1/PHASE2NOT_YET_RECRUITINGCaspoNEB: Efficacy and Safety of Caspofungin Aerosols for the Curative Treatment of Pneumocystis Pneumonia
NCT01479361PHASE1COMPLETEDAntiretroviral Drug Interaction Study in Volunteers With HIV
NCT03856229Not specifiedENROLLING_BY_INVITATIONShort-term Steroid Therapy in Patients With P. Jirovecii Pneumonia Due to HIV / AIDS
NCT06431958Not specifiedACTIVE_NOT_RECRUITINGDroplet Digital PCR and PCR-free BIOSensors for the Detection of Resistance-associated SNPs in Pneumocystis Jirovecii
NCT06442345Not specifiedNOT_YET_RECRUITINGPneumocystis Jirovecii Genotyping
NCT06691321Not specifiedRECRUITINGCaspofungin for Pneumocystis Pneumonia in PLWHIV.
NCT06890078Not specifiedRECRUITINGOptimizing the Diagnosis of Pneumocystis in Immunocompromised Patients
NCT00869544Not specifiedCOMPLETEDPneumocystis in Pathogenesis of HIV-associated Emphysema
NCT00870857Not specifiedCOMPLETEDPrevalence and Pathogenesis of Lung Disease in a Large HIV Cohort-coordinating Center
NCT02045160Not specifiedUNKNOWNA Pharmacokinetic and Pharmacogenetic Study in Patients Receiving Sulfamethoxazole-trimethoprim Therapy
NCT02603575Not specifiedUNKNOWNEffects and Safety of Caspofungin and Corticosteroids in Pneumocystis Pneumonia in Non-HIV Patients
NCT03613025Not specifiedCOMPLETEDNon Invasive Diagnosis of Pneumocystis Pneumonia
NCT04328688Not specifiedCOMPLETEDClindamycin-trimethoprim/Sulfamethoxazole for PCP After Solid Organ Transplantation Population.
NCT04358419Not specifiedCOMPLETEDNon-invasive Diagnosis of Invasive Pulmonary Aspergillosis by Use of Biomarkers in Exhaled Breath Condensate
NCT05077150Not specifiedCOMPLETEDA Case-control Study on Risk Factors, Timing, and PCR Use, for Pneumocystis Pneumonia (PcP) After Allogeneic HSCT
NCT05605145Not specifiedUNKNOWNPCP in Immunocompromised Population in Southern China
NCT05701631Not specifiedUNKNOWNRadiomics Model for the Diagnosis of Pneumocystis Jirovecii Pneumonia in Non-HIV Patients
NCT05707156Not specifiedCOMPLETEDProspective Observational Study on the Incidence of Opportunistic Fungal Infections
NCT06654076Not specifiedCOMPLETEDAssessing Diagnostic Methods for Invasive Fungal Disease in Lung Transplant Recipients
NCT07004127Not specifiedCOMPLETEDPneumocystis in Patients Over 75 Year

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
CASPOFUNGIN43
CLINDAMYCIN43
PRIMAQUINE43
METHYLPREDNISOLONE42
REZAFUNGIN42
ATOVAQUONE41
DAPSONE41
FLUCONAZOLE41
PENTAMIDINE41
POSACONAZOLE41
SULFAMETHOXAZOLE41
TRIMETHOPRIM41
CO-TRIMOXAZOLE32
PAFURAMIDINE MALEATE31
CHEMBL475094103
CHEMBL49980803
CHEMBL474320502
CHEMBL451967101
CHEMBL47179201