Pneumocystosis

What is pneumocystosis?

Pneumocystosis is disease caused by infection with pneumocystis organisms. Pneumocystosis is an opportunistic infection, primarily seen in individuals with impaired immunity.

Pneumocystosis is also known as PJP (Pneumocystis jiroveci pneumonia, previously known as PCP or Pneumocystis carinii pneumonia). The parasite was once considered a protozoan, but has recently been reclassified as a fungus, although debate still exists over the correct taxonomic classification. Pneumocystis organisms are commonly found in the lungs of healthy humans and other mammals. Recent DNA analysis has shown the existence of multiple species of Pneumocystis. This has led to the renaming of the organism that causes human disease from P. carinii to P. jirovecii.

P. jirovecii is a ubiquitous parasite that is present worldwide. Most children have antibodies to Pneumocystis by age 3 or 4, suggesting prior exposure to the organism. In healthy individuals, P. jirovecii rarely causes disease. Pneumocystosis usually occurs in immunodeficient patients with:

• HIV – pneumocystosis is the most common opportunistic infection in patients with human immunodeficiency virus infection
• Chronic mucocutaneous candidiasis
• Granulomatosis with polyangiitis
• Lupus erythematosus
• Long-term treatment with immunosuppressant medications, for example in organ transplant recipients
• Other congenital and acquired immune deficiencies
• Severe malnutrition
• Patients with cancer
• Premature malnourished infants

What are the clinical features of pneumocystosis?

P. jirovecii infection is usually limited to the lungs, but can occasionally spread to other organs, including the skin. It has been estimated that 66% to 85% of all HIV infected individuals will have one or more episodes of pneumocystosis in their lifetime. Approximately 1–2.5% of all P. jirovecii infections are extrapulmonary (they spread outside the lungs). Involvement of the skin by P. jirovecii is even rarer.

• Symptoms of pneumocystosis are relatively non-specific and include breathlessness, fever, and a nonproductive (dry) cough. These symptoms tend to develop more slowly (over several weeks) in patients with HIV infection. The physical signs of pneumocystosis are also non-specific. Increased respiratory rate and heart rate are present in most patients, and lung examination may reveal mild crackles and rhonchi in around 50% of patients.
• Extrapulmonary disease — sites most frequently involved are the lymph nodes, spleen, liver, and bone marrow. Extrapulmonary disease is usually more severe in HIV-infected individuals.
• Skin disease — patients usually present with red or skin-coloured non-tender papules (small bumps, less than 0.5 cm diameter) or nodules (larger than 0.5cm). Secondary erosion (loss of the surface of the lesion) or ulceration may be seen. The lesions can be solitary or widespread. The ear, external auditory canal, and axilla (armpit) are the most common sites of involvement.

How is pneumocystosis diagnosed?

The diagnosis of pneumocystosis is usually based on microscopic identification of P. jirovecii from bronchopulmonary (lung) secretions. Bronchopulmonary secretions can be obtained by induced sputum (patient inhales a saltwater mist to encourage a deep cough) bronchoalveolar lavage (a small tube is passed through the mouth or nose into the lungs, fluid is squirted into a small area of the lung and is then recollected for examination) or lung biopsy.

Different techniques can aid microscopic identification of P. jirovecii:

• Special stains (such as Giemsa or silver stains) can highlight the organism
• Direct immunofluorescence using monoclonal antibodies – antibodies to P. jirovecii are produced in the laboratory and dyed with fluorescein. These antibodies bind to antigen in infected cells and the cells display fluorescence under the microscope.

Biopsy of any skin lesions, with subsequent staining and microscopy, can also reveal the organism.

What is the treatment for pneumocystosis?

The drug of choice for treating P. jirovecii infection is trimethoprim-sulphamethoxazole (TMP-SMZ). Patients infected with HIV tend to respond to treatment more slowly than patients without HIV and require a longer duration of therapy (3 weeks of treatment as opposed to 2).

Alternative treatments for pneumocystosis include pentamidine and atovaquone.

TMP-SMZ can also be used as a prophylactic (preventive) agent in patients with HIV and in other patients with impaired immunity. In patients who cannot tolerate TMP-SMX, other options include dapsone, dapsone plus pyrimethamine, atovaquone, and aerosolised pentamidine.

What is the prognosis in pneumocystosis?

Early diagnosis and treatment of pneumocystosis increase the likelihood of successful treatment. In patients with HIV infection, around 10–20% of cases will be fatal.

In patients without HIV infection, around 30–50% of cases will be fatal; the higher mortality rate is likely to be due to delayed diagnosis in this group.