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Pneumocystosis

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Pneumocystosis
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Pneumocystosis is a fungal infection that most often presents as Pneumocystis pneumonia in people with HIV/AIDS or poor immunity.[1][7] It usually causes cough, difficulty breathing and fever, and can lead to respiratory failure.[4] Involvement outside the lungs is rare but, can occur as a disseminated type affecting lymph nodes, spleen, liver, bone marrow, eyes, kidneys, thyroid, gastrointestinal tract or other organs.[5][7] If occurring in the skin, it usually presents as nodular growths in the ear canals or underarms.[3]

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It is caused by Pneumocystis jirovecii, a fungus which is usually breathed in and found in the lungs of healthy people without causing disease, until the person's immune system becomes weakened.[7]

Diagnosis is by identifying the organism from a sample of fluid from affected lungs or a biopsy.[3][4] Prevention in high risk people, and treatment in those affected is usually with trimethoprim/sulfamethoxazole (co-trimoxazole).[4][8]

The prevalence is unknown.[7] Less than 3% of cases do not involve the lungs.[7] The first cases of pneumocystosis affecting lungs were described in premature infants in Europe following the Second World War.[9]

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Signs and symptoms

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Pneumocystosis is generally an infection in the lungs.[4] Involvement outside the lungs is rare but, can occur as a disseminated type affecting lymph nodes, bone marrow,[5][7] liver[5][10] or spleen.[5][11] It may also affect skin,[3] eyes,[12] kidneys, thyroid, heart, adrenals and gastrointestinal tract.[5][13]

Lungs

When the lungs are affected there is usually a dry cough, difficulty breathing and fever, usually present for longer than four weeks.[2][7] There may be chest pain, shivering or tiredness.[8] The oxygen saturation is low.[2] The lungs may fail to function.[4]

Eyes

Pneumocystosis in eyes may appear as a single or multiple (up to 50) yellow-white plaques in the eye's choroid layer or just beneath the retina.[12] Vision is usually not affected and it is typically found by chance.[12]

Skin

If occurring in the skin, pneumocystosis most often presents as nodular growths in the ear canals of a person with HIV/AIDS.[3][6] There may be fluid in the ear.[6] Skin involvement may appear outside the ear, usually palms, soles or underarms; as a rash, or small bumps with a dip.[6] It can occur on the face as brownish bumps and plaques.[6] The bumps may be tender and the ulcerate.[3] Infection in the ear may result in a perforated ear drum or destruction of the mastoid bone.[6] The nerves in the head may be affected.[6]

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Cause

Pneumocystosis is caused by Pneumocystis jirovecii, a fungus which is generally found in the lungs of healthy people, without causing disease until the person's immune system becomes weakened.[7]

Risk factors

Pneumocystosis occurs predominantly in people with HIV/AIDS.[8] Other risk factors include chronic lung disease, cancer, autoimmune diseases, organ transplant, or taking corticosteroids.[8]

Diagnosis

Thumb
X-ray and CT of ground glass opacities and pneumothorax in pneumocystis pneumonia

Diagnosis of Pneumocystis pneumonia is by identifying the organism from a sample of sputum, fluid from affected lungs or a biopsy.[4][3] A chest X-ray of affected lungs show widespread shadowing in both lungs, with a "bat-wing" pattern and ground glass appearance.[2][7] Giemsa or silver stains can be used to identify the organism, as well as direct immunofluorescence of infected cells.[3]

Diagnosis in the eye involves fundoscopy.[12] A biopsy of the retina and choroid layer may be performed.[12] In affected liver, biopsy shows focal areas of necrosis and sinusoidal widening.[10] H&E staining show extracellular frothy pink material.[10] Typical cysts with a solid dark dot can be seen using a Grocott silver stain.[10]

Differential diagnosis

Pneumocystosis may appear similar to pulmonary embolism or adult respiratory distress syndrome.[2] Other infections can present similarly such as tuberculosis, Legionella, and severe flu.[2]

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Prevention

There is no vaccine that prevents pneumocystosis.[8] Trimethoprim/sulfamethoxazole (co-trimoxazole) might be prescribed for people at high risk.[8]

Treatment

Treatment is usually with co-trimoxazole.[4][8] Other options include pentamidine, dapsone and atovaquone.[2]

Outcomes

It is fatal in 10-20% of people with HIV/AIDS.[3] Pneumocystosis in people without HIV/AIDS is frequently diagnosed late and the death rate is therefore higher; 30-50%.[3]

Epidemiology

The exact number of people in the world affected is not known.[7] Pneumocystosis affects lungs in around 97% of cases and is often fatal without treatment.[7]

History

The first cases of pneumocystosis affecting lungs were described in premature infants in Europe following the Second World War.[9] It was then known as plasma cellular interstitial pneumonitis of the newborn.[9]

Pneumocystis jirovecii (previously called Pneumocystis carinii) is named for Otto Jírovec, who first described it in 1952.[2]

References

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