Pneumocystosis
diseaseOn 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 ID | Term | Frequency |
|---|---|---|
| HP:0001945 | Fever | Very frequent (80-99%) |
| HP:0002094 | Dyspnea | Very frequent (80-99%) |
| HP:0011949 | Acute infectious pneumonia | Very frequent (80-99%) |
| HP:0012418 | Hypoxemia | Very frequent (80-99%) |
| HP:0020102 | Pneumocystis jirovecii pneumonia | Very frequent (80-99%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0002093 | Respiratory insufficiency | Frequent (30-79%) |
| HP:0002202 | Pleural effusion | Frequent (30-79%) |
| HP:0002721 | Immunodeficiency | Frequent (30-79%) |
| HP:0002875 | Exertional dyspnea | Frequent (30-79%) |
| HP:0002878 | Respiratory failure | Frequent (30-79%) |
| HP:0004887 | Respiratory failure requiring assisted ventilation | Frequent (30-79%) |
| HP:0006515 | Interstitial pneumonitis | Frequent (30-79%) |
| HP:0010702 | Increased circulating antibody level | Frequent (30-79%) |
| HP:0011991 | Abnormal neutrophil count | Frequent (30-79%) |
| HP:0025395 | Combined cystic and ground-glass pattern on pulmonary HRCT | Frequent (30-79%) |
| HP:0031246 | Nonproductive cough | Frequent (30-79%) |
| HP:0002664 | Neoplasm | Occasional (5-29%) |
| HP:0005948 | Multiple pulmonary cysts | Occasional (5-29%) |
| HP:0009098 | Chronic oral candidiasis | Occasional (5-29%) |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | Occasional (5-29%) |
| HP:0031863 | Bloodstream infectious agent | Occasional (5-29%) |
| HP:0032177 | Parenchymal consolidation | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | pneumocystosis |
| Mondo ID | MONDO:0019121 |
| EFO | EFO:0007448 |
| MeSH | D011020 |
| Orphanet | 723 |
| DOID | DOID:11339 |
| ICD-10-CM | B59 |
| ICD-11 | 404370038 |
| NCIT | C3334 |
| SNOMED CT | 415125002 |
| UMLS | C1535939 |
| MedGen | 777048 |
| GARD | 0004386 |
| NORD | 1923 |
| 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 disease › fungal infectious disease › opportunistic mycosis › pneumocystosis
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.
| Drug | Development status |
|---|---|
| Atovaquone | Approved (phase 4) |
| Sulfamethoxazole | Approved (phase 4) |
| Trimethoprim | Approved (phase 4) |
| Clindamycin | Phase 3 (in late-stage trials) |
| Cobicistat | Phase 3 (in late-stage trials) |
| Dapsone | Phase 3 (in late-stage trials) |
| Darunavir | Phase 3 (in late-stage trials) |
| Hydroxychloroquine | Phase 3 (in late-stage trials) |
| Methylprednisolone | Phase 3 (in late-stage trials) |
| Primaquine | Phase 3 (in late-stage trials) |
| Pyrimethamine | Phase 3 (in late-stage trials) |
| Sulfadoxine | Phase 3 (in late-stage trials) |
| Zidovudine | Phase 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)
| Phase | Trials |
|---|---|
| Not specified | 18 |
| PHASE4 | 7 |
| PHASE3 | 4 |
| PHASE2 | 1 |
| PHASE1/PHASE2 | 1 |
| PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT07328984 | PHASE4 | NOT_YET_RECRUITING | How Long Should we Give Steroids for Patients With Severe PCP |
| NCT07357103 | PHASE4 | NOT_YET_RECRUITING | Positioning Second-line Therapies for Pneumocystis Jirovecii Pneumonia (PCP Alternatives) |
| NCT00636935 | PHASE4 | WITHDRAWN | Corticosteroids Therapy and Pneumocystis Jirovecii Pneumonia (PCP) |
| NCT01167452 | PHASE4 | COMPLETED | Effect of Weight and/or Obesity on Sulfamethoxazole and Trimethoprim Concentrations |
| NCT02550080 | PHASE4 | UNKNOWN | Clinical Utility Of Genetic Screening For HLA-B*1301, On Susceptibility To Dapsone Hypersensitivity Syndrome |
| NCT03087890 | PHASE4 | COMPLETED | Impact of Cotrimoxazole Use in Immunocompetent HIV Patients on Carriage of Antimicrobial Resistant Bacteria |
| NCT03978559 | PHASE4 | UNKNOWN | Trimethoprim/Sulfamethoxazole Combined With Caspofungin as First-line Therapy in PCP |
| NCT04851015 | PHASE3 | RECRUITING | Low Dose Trimethoprim-Sulfamethoxazole for the Treatment of Pneumocystis Jirovecii Pneumonia |
| NCT00302341 | PHASE3 | TERMINATED | DB289 Versus TMP-SMX for the Treatment of Acute Pneumocystis Jiroveci Pneumonia (PCP) |
| NCT02944045 | PHASE3 | UNKNOWN | Effect of Steroids During Pneumocystis Infection Among Non HIV Immunocompromised Patients |
| NCT04368559 | PHASE3 | COMPLETED | Study of Rezafungin Compared to Standard Antimicrobial Regimen for Prevention of Invasive Fungal Diseases in Adults Undergoing Allogeneic Blood and Marrow Transplantation |
| NCT05835479 | PHASE2 | RECRUITING | Rezafungin for Treatment of Pneumocystis Pneumonia in HIV Adults |
| NCT06892951 | PHASE1/PHASE2 | NOT_YET_RECRUITING | CaspoNEB: Efficacy and Safety of Caspofungin Aerosols for the Curative Treatment of Pneumocystis Pneumonia |
| NCT01479361 | PHASE1 | COMPLETED | Antiretroviral Drug Interaction Study in Volunteers With HIV |
| NCT03856229 | Not specified | ENROLLING_BY_INVITATION | Short-term Steroid Therapy in Patients With P. Jirovecii Pneumonia Due to HIV / AIDS |
| NCT06431958 | Not specified | ACTIVE_NOT_RECRUITING | Droplet Digital PCR and PCR-free BIOSensors for the Detection of Resistance-associated SNPs in Pneumocystis Jirovecii |
| NCT06442345 | Not specified | NOT_YET_RECRUITING | Pneumocystis Jirovecii Genotyping |
| NCT06691321 | Not specified | RECRUITING | Caspofungin for Pneumocystis Pneumonia in PLWHIV. |
| NCT06890078 | Not specified | RECRUITING | Optimizing the Diagnosis of Pneumocystis in Immunocompromised Patients |
| NCT00869544 | Not specified | COMPLETED | Pneumocystis in Pathogenesis of HIV-associated Emphysema |
| NCT00870857 | Not specified | COMPLETED | Prevalence and Pathogenesis of Lung Disease in a Large HIV Cohort-coordinating Center |
| NCT02045160 | Not specified | UNKNOWN | A Pharmacokinetic and Pharmacogenetic Study in Patients Receiving Sulfamethoxazole-trimethoprim Therapy |
| NCT02603575 | Not specified | UNKNOWN | Effects and Safety of Caspofungin and Corticosteroids in Pneumocystis Pneumonia in Non-HIV Patients |
| NCT03613025 | Not specified | COMPLETED | Non Invasive Diagnosis of Pneumocystis Pneumonia |
| NCT04328688 | Not specified | COMPLETED | Clindamycin-trimethoprim/Sulfamethoxazole for PCP After Solid Organ Transplantation Population. |
| NCT04358419 | Not specified | COMPLETED | Non-invasive Diagnosis of Invasive Pulmonary Aspergillosis by Use of Biomarkers in Exhaled Breath Condensate |
| NCT05077150 | Not specified | COMPLETED | A Case-control Study on Risk Factors, Timing, and PCR Use, for Pneumocystis Pneumonia (PcP) After Allogeneic HSCT |
| NCT05605145 | Not specified | UNKNOWN | PCP in Immunocompromised Population in Southern China |
| NCT05701631 | Not specified | UNKNOWN | Radiomics Model for the Diagnosis of Pneumocystis Jirovecii Pneumonia in Non-HIV Patients |
| NCT05707156 | Not specified | COMPLETED | Prospective Observational Study on the Incidence of Opportunistic Fungal Infections |
| NCT06654076 | Not specified | COMPLETED | Assessing Diagnostic Methods for Invasive Fungal Disease in Lung Transplant Recipients |
| NCT07004127 | Not specified | COMPLETED | Pneumocystis in Patients Over 75 Year |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CASPOFUNGIN | 4 | 3 |
| CLINDAMYCIN | 4 | 3 |
| PRIMAQUINE | 4 | 3 |
| METHYLPREDNISOLONE | 4 | 2 |
| REZAFUNGIN | 4 | 2 |
| ATOVAQUONE | 4 | 1 |
| DAPSONE | 4 | 1 |
| FLUCONAZOLE | 4 | 1 |
| PENTAMIDINE | 4 | 1 |
| POSACONAZOLE | 4 | 1 |
| SULFAMETHOXAZOLE | 4 | 1 |
| TRIMETHOPRIM | 4 | 1 |
| CO-TRIMOXAZOLE | 3 | 2 |
| PAFURAMIDINE MALEATE | 3 | 1 |
| CHEMBL4750941 | 0 | 3 |
| CHEMBL499808 | 0 | 3 |
| CHEMBL4743205 | 0 | 2 |
| CHEMBL4519671 | 0 | 1 |
| CHEMBL471792 | 0 | 1 |