Pneumocystis pneumonia (PCP), also referred to as Pneumocystis jirovecii pneumonia (PJP), is an atypical pulmonary infection and the most common opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS).
Classically, PCP was the acronym for Pneumocystis carinii pneumonia, but the causative organism was reclassified as Pneumocystis jirovecii. Strictly speaking, Pneumocystis carinii refers to a species found in rats, while Pneumocystis jirovecii refers to the human isolate. However, physicians have been slow to adopt the updated terminology, and there continues to be widespread use of the term PCP; the post-hoc justification for its use is that it stands for pneumocystis pneumonia.
Pneumocystis pneumonia is virtually never present in immunocompetent individuals. It is seen particularly in patients with AIDS; therefore, the demographics closely match those of the AIDS population. Typically, non-AIDS patients are severely immunosuppressed due to other causes, such as haematological malignancy or in bone marrow transplant recipients.
In order to be susceptible to PCP, the patient's CD4 count usually has to fall below 200 cells/mm3 8.
Presentation is usually non-specific and insidious, the most common symptoms being dyspnoea and/or a non-productive cough. In patients who are profoundly immunocompromised, onset may be more dramatic and resemble other pulmonary infections 7,9.
The diagnosis can often be confirmed with bronchoalveolar lavage which has a sensitivity of 85-90% 8.
Pneumocystis jirovecii is an atypical yeast-like fungus of the genus Pneumocystis 10 that was previously thought to be a protozoan.
Histology of infected lung demonstrates intra-alveolar eosinophilic masses with a foamy appearance, due to small cysts within which the Pneumocystis jirovecii organism is found 9.
Culturing Pneumocystis jirovecii can be very difficult. Diagnostic confirmation requires identification of organisms in sputum or bronchoalveolar lavage fluid. Monoclonal antibodies for detecting Pneumocystis jirovecii are available and have a sensitivity greater than 90% for detecting Pneumocystis jirovecii in induced sputum from HIV-infected patients 10.
Although up to 90% of chest radiographs in patients with PCP are abnormal, appearances are often non-specific. Between 10-15% of patients with PCP have normal chest radiographs and close to 30% have non-specific or inconclusive findings 2-4,6,7 .
Features which are highly suggestive of PCP in patients with CD4 counts below 200/mm3 include 5:
- small pneumatoceles, subpleural blebs
- a fine reticular interstitial pulmonary pattern
- there is often a perihilar distribution
Pleural effusions are normally not a feature, being seen in less than 5% of cases 9.
High-resolution computed tomography (HRCT) is more sensitive and may be used to exclude PCP in patients with clinical suspicion for PCP but normal or inconclusive chest radiographs 3.
Features include 2,3,7:
- considered a principal finding
- predominantly involving perihilar or mid zones
- there may be a mid, upper or lower zone predilection depending on whether the patient is on prophylactic aerosolised medication
- if they are, then the poorly ventilated upper zones are prone to infection 9, whereas in those who are not, the lower zones are more frequently involved
- there may be a relative preservation of previously irradiated areas
- may show some peripheral sparing in a considerable number of patients (~40%) 10
- reticular opacities or septal thickening may also be present; a crazy paving pattern may, therefore, be seen when both ground-glass opacities and septal thickening are superimposed on one another
- varying shape, size, and wall thickness
- are seen in up to 30% of cases 7
- pleural effusions are rare 2,3
- lymphadenopathy is uncommon (10%)
Especially in patients treated prophylactically, features are more frequently atypical including 7:
- consolidation: can be more common in patients without HIV infection and tends to develop more rapidly, reflecting pulmonary damage from the host immune response.
- nodules (granulomas)
- pleural effusions are also more frequently encountered in this group of patients
A cystic form of PCP is also recognised, again more frequently in patients receiving aerosolised prophylaxis. In this pattern, features include 7:
- thin walled cysts: in most cases these are pneumatoceles
- upper lobes
- may be bilateral
- increased risk of pneumothorax
Gallium67 lung scintigraphy is highly sensitive for PCP, and a normal gallium scan renders the diagnosis of PCP very unlikely. The gallium scan in patients with PCP demonstrates diffuse pulmonary uptake, which may be heterogeneous or homogeneous.
Despite this, the specificity of the gallium scan is low; hence, it is most useful in patients in whom bronchoalveolar lavage may be less diagnostic (e.g. in suspected relapse).
Treatment and prognosis
Most patients with an acute infection are treated with trimethoprim-sulfamethoxazole (co-trimoxazole or TMP-SMZ), combined with corticosteroids in patients with moderate to severe infections 8. The same agent may be used as prophylaxis. A number of alternative agents may also be employed, both for acute treatment and prophylaxis, although these are beyond the scope of this article.
Overall, with prompt treatment, survival is good (50-95%), although relapses are common 9.
The differential diagnosis on HRCT is strongly influenced by knowledge of HIV status and CD4 count. In these patients the differential includes:
- viral pneumonitis: similar, but cysts are absent
- mycobacterium tuberculosis: when upper zone cysts are prominent
- angioinvasive aspergillosis
- immune reconstitution inflammatory syndrome (IRIS): patients receiving HAART
Other conditions to be considered, which also occur in non-AIDS patients, include:
- for prominent cysts consider
- for a reticulonodular pattern consider
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