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ORPHA:723Disease
What it is
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, localised 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.
Key facts
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Common30–79%
12- Abnormal neutrophil count
- Combined cystic and ground-glass pattern on pulmonary HRCT
- Exertional dyspnea
- Immunodeficiency
- Increased circulating immunoglobulin concentration
- Interstitial pneumonitis
- Nonproductive cough
- Pleural effusion
- Respiratory failure
- Respiratory failure requiring assisted ventilation
- Respiratory insufficiency
- Weight loss
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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