Pneumocystis pneumonia Accordion Q&A Notes

Pneumocystis pneumonia Active Recall Accordion Q&A Revision Notes

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Definition of Pneumocystis Pneumonia (PCP)

• Severe fungal lung infection caused by Pneumocystis jirovecii (formerly Pneumocystis carinii).
• Affects mainly individuals with weakened immune systems.

• People with HIV/AIDS, those who have undergone organ transplants, and individuals on immunosuppression therapy.

• Pneumocystis jirovecii is a unicellular eukaryote with features of both a fungus and a protozoa.

• It is the most common opportunistic infection in AIDS patients and is considered AIDS-defining.

• Can result in severe respiratory symptoms and is potentially fatal.

• Essential for managing PCP in at-risk populations, helping to prevent severe outcomes and complications.

Aetiology of Pneumocystis Pneumonia (PCP)

Pneumocystis jirovecii, a fungal organism, is the causative agent of PCP.

Transmission occurs through inhalation of airborne fungal spores.

Immunocompromised individuals (e.g. those with HIV/AIDS or undergoing immunosuppressive therapy) are primarily affected.

The fungus is widespread in the environment but does not cause illness in individuals with healthy immune systems.

Risk Factors for Pneumocystis Pneumonia (PCP)

• Weakened immune system, particularly in HIV-positive individuals and those on immunosuppressive therapy.

• When the CD4 count drops below 200 cells/mm³.

1. HIV-related factors:
– Oral thrush, persistent fever, or an AIDS-defining diagnosis.
2. Immunosuppressive therapy:
– Use of steroids or other immunosuppressants
3. Haematological malignancies and organ transplants
4. Connective tissue diseases
5. Congenital immune deficiencies such as thymic aplasia, severe combined immune deficiency (SCID), hypogammaglobulinemia

Pathophysiology of Pneumocystis Pneumonia (PCP)

• Targets the lung alveoli, leading to inflammation, compromised gas exchange, lung tissue damage, fluid buildup, and potentially respiratory failure.

• Involves a dysregulated immune response primarily affecting T-cell-mediated immunity, especially in individuals with impaired T-cell function.

Differential Diagnosis of Pneumocystis Pneumonia (PCP)

Differential diagnoses include:
– Bacterial/Viral pneumonia
– Fungal infections
– Tuberculosis
– Atypical pneumonia
– Pulmonary Kaposi’s sarcoma
– Influenza/Cytomegalovirus infection
– Measles/Varicella pneumonitis
– Coxiella burnetii (Q Fever)
– Thoracic actinomycosis/Nocardiosis
– Lymphocytic interstitial pneumonia
– Pulmonary embolism

Epidemiology of Pneumocystis Pneumonia (PCP) in the UK

• Individuals with weakened immune systems, particularly those with HIV/AIDS
• Transplant recipients
• Individuals with malignancies.

• The incidence of PCP has been significantly reduced due to Highly Active Antiretroviral Therapy (HAART).

• Lower rates due to poor HIV survival, underdiagnosis, and the presence of prior pulmonary infections such as TB.

Clinical Presentation of Pneumocystis Pneumonia (PCP)

• Respiratory Symptoms: Progressive dyspnea, non-productive cough, chest discomfort
• Systemic Symptoms: Fever, fatigue, cyanosis, respiratory distress

• Pulmonary: Pneumothorax
• Rare Extrapulmonary Manifestations: Hepatosplenomegaly, lymphadenopathy, choroid lesions

• Respiratory: Exertional dyspnea, tachypnea, chest pain
• Oral Manifestations: Thrush, oral hairy leukoplakia
• Cutaneous: Kaposi’s sarcoma

Investigations for Pneumocystis Pneumonia (PCP)

• Induced sputum
• Bronchoalveolar lavage (BAL)
• Lung biopsy (less common)

• Chest X-ray (CXR): May show bilateral interstitial infiltrates, perihilar fluffy shadows, or pneumothorax
• CT scan: High-resolution CT often reveals ground-glass infiltrates, offering high sensitivity and specificity

• Elevated lactate dehydrogenase (LDH)
• Arterial blood gases (ABG): Show hypoxia and increased alveolar-arterial oxygen tension gradient
• Serum (1→3) beta-D-glucan: Elevated levels are indicative of PCP

• PCR or staining can detect Pneumocystis jirovecii DNA or antigens in sputum or BAL samples

No, it cannot be routinely cultured.

• Mild: Breathlessness on mild exercise; PaO2 >11 kPa; SaO2 >96%; CXR normal or minor perihilar infiltrates
• Moderate: Breathlessness on minimal exercise; PaO2 8-11 kPa; SaO2 91-96%; CXR diffuse interstitial shadowing
• Severe: Breathlessness at rest; PaO2 <8 kPa; SaO2 <91%; CXR extensive interstitial shadowing, possibly with alveolar shadowing

• Modest reduction in vital capacity (VC) and total lung capacity (TLC)
• Consistent decrease in single-breath diffusing capacity for carbon monoxide (DLCO) with high sensitivity

Management of Pneumocystis Pneumonia (PCP) in the UK

• Co-trimoxazole (trimethoprim-sulfamethoxazole), particularly for mild-to-moderate cases.

• IV pentamidine is used for severe cases.
• Corticosteroids are added if hypoxia is present (PO2 < 9.3 kPa) to reduce the risk of respiratory failure and death.

• Oxygen therapy
• Corticosteroids
• Optimization of antiretroviral therapy (ART) for HIV/AIDS patients.

• 7 days of treatment.

• Atovaquone
• Dapsone with trimethoprim
• Clindamycin with primaquine.

• Due to its lower efficacy, risk of pneumothorax, and relapse.

• Oral co-trimoxazole is recommended for prophylaxis, especially for those with a history of PCP or severe immunosuppression.
• Prophylaxis can be discontinued when CD4 T-cell counts are above 200 cells/mm³ in HIV-positive patients, indicating successful ART.

Prognosis of Pneumocystis Pneumonia (PCP)

The prognosis depends on:
– Immune status (e.g., advanced HIV or severe immunosuppression).
– Timeliness of treatment.

Early diagnosis, appropriate treatment, and antiretroviral therapy (ART) significantly improve the prognosis in HIV-related PCP.

With early diagnosis and treatment, the prognosis is generally favourable.

– Overall: 25.4%.
– In ICU-admitted cases: 58%.

PCP can become life-threatening in cases of advanced HIV/AIDS or severe immunosuppression, leading to complications like respiratory failure.

ART has led to a significant improvement in the prognosis of HIV-related PCP and reduced the incidence of severe cases.

Complications of Pneumocystis Pneumonia (PCP)

• Respiratory failure
• Pneumothorax
• Acute respiratory distress syndrome (ARDS)
• Pulmonary cyst formation
• Secondary bacterial infections

• Haematogenous spread of the infection can affect the bone marrow, liver, spleen, lymph nodes, gut, and eyes.

• Restricted access to care, which can significantly impact outcomes.