Pneumocystosis Explained

Pneumocystosis
Synonyms:Pneumocystis jiroveci pneumonia,[1] Pneumocystis pneumonia,[2] PCP, Pneumocystis carinii pneumonia
Field:Infectious diseases
Symptoms:
Complications:
Types:
Causes:Pneumocystis jirovecii
Risks:Poor immunity, HIV/AIDS
Diagnosis:Medical imaging, bronchoalveolar lavage, immunofluorescence assay, biopsy
Prevention:Trimethoprim/sulfamethoxazole (co-trimoxazole) in high risk groups
Medication:Trimethoprim/sulfamethoxazole (co-trimoxazole)
Frequency:Uncommon, 97% in lungs

Pneumocystosis is a fungal infection that most often presents as Pneumocystis pneumonia in people with HIV/AIDS or poor immunity.[1] It usually causes cough, difficulty breathing and fever, and can lead to respiratory failure.[3] 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] If occurring in the skin, it usually presents as nodular growths in the ear canals or underarms.

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.

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

The prevalence is unknown. Less than 3% of cases do not involve the lungs. The first cases of pneumocystosis affecting lungs were described in premature infants in Europe following the Second World War.[6]

Signs and symptoms

Pneumocystosis is generally an infection in the lungs.[3] Involvement outside the lungs is rare but, can occur as a disseminated type affecting lymph nodes, bone marrow,[5] liver[5] [7] or spleen.[5] [8] It may also affect skin, eyes,[9] kidneys, thyroid, heart, adrenals and gastrointestinal tract.[5] [10]

Lungs

See main article: article and Pneumocystis pneumonia. When the lungs are affected there is usually a dry cough, difficulty breathing and fever, usually present for longer than four weeks.[2] There may be chest pain, shivering or tiredness.[11] The oxygen saturation is low.[2] The lungs may fail to function.[3]

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.[9] Vision is usually not affected and it is typically found by chance.[9]

Skin

If occurring in the skin, pneumocystosis most often presents as nodular growths in the ear canals of a person with HIV/AIDS.[4] There may be fluid in the ear.[4] Skin involvement may appear outside the ear, usually palms, soles or underarms; as a rash, or small bumps with a dip.[4] It can occur on the face as brownish bumps and plaques.[4] The bumps may be tender and the ulcerate. Infection in the ear may result in a perforated ear drum or destruction of the mastoid bone.[4] The nerves in the head may be affected.[4]

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.[12]

Risk factors

Pneumocystosis occurs predominantly in people with HIV/AIDS.[11] Other risk factors include chronic lung disease, cancer, autoimmune diseases, organ transplant, or taking corticosteroids.[11]

Diagnosis

Diagnosis of Pneumocystis pneumonia is by identifying the organism from a sample of sputum, fluid from affected lungs or a biopsy.[3] [13] A chest X-ray of affected lungs show widespread shadowing in both lungs, with a "bat-wing" pattern and ground glass appearance.[2] [12] Giemsa or silver stains can be used to identify the organism, as well as direct immunofluorescence of infected cells.[13]

Diagnosis in the eye involves fundoscopy.[9] A biopsy of the retina and choroid layer may be performed.[9] In affected liver, biopsy shows focal areas of necrosis and sinusoidal widening.[7] H&E staining show extracellular frothy pink material.[7] Typical cysts with a solid dark dot can be seen using a Grocott silver stain.[7]

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]

Prevention

There is no vaccine that prevents pneumocystosis.[11] Trimethoprim/sulfamethoxazole (co-trimoxazole) might be prescribed for people at high risk.[11]

Treatment

Treatment is usually with co-trimoxazole.[3] [11] Other options include pentamidine, dapsone and atovaquone.[2]

Outcomes

It is fatal in 10-20% of people with HIV/AIDS.[13] Pneumocystosis in people without HIV/AIDS is frequently diagnosed late and the death rate is therefore higher; 30-50%.[13]

Epidemiology

The exact number of people in the world affected is not known.[12] Pneumocystosis affects lungs in around 97% of cases and is often fatal without treatment.[12]

History

The first cases of pneumocystosis affecting lungs were described in premature infants in Europe following the Second World War.[6] It was then known as plasma cellular interstitial pneumonitis of the newborn.[6]

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

Notes and References

  1. Web site: ICD-11 - ICD-11 for Mortality and Morbidity Statistics . icd.who.int . 30 June 2021.
  2. Book: Russell-Goldman . Eleanor . Milner . Dan A. . Solomon . Isaac . Milner . Danny A. . Diagnostic Pathology: Infectious Diseases . 2020 . Elsevier . 978-0-323-61138-1 . Pneumocystosis. 310–313 . https://books.google.com/books?id=172gDwAAQBAJ&pg=PA310 . en.
  3. Web site: Orphanet: Pneumocystosis . www.orpha.net . 24 July 2021 . https://web.archive.org/web/20210724075314/https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=723 . 24 July 2021 . en.
  4. Book: Calonje . J. Eduardo . Grayson . Wayne . Calonje . J. Eduardo . Brenn . Thomas . Lazar . Alexander J. . Billings . Steven D. . McKee's Pathology of the Skin, 2 Volume Set . 2020 . Elsevier . 978-0-7020-6983-3 . 964 . 5th . https://books.google.com/books?id=pMN1DwAAQBAJ&pg=PA964 . en . 18. Infectious diseases of the skin.
  5. Book: Ioachim . Harry L. . Medeiros . L. Jeffrey . Ioachim's Lymph Node Pathology . 2009 . Lippincott Williams & Wilkins . Philadelphia . 978-0-7817-7596-0 . 156–157 . 4th . https://books.google.com/books?id=-hmfiFfdNbwC&pg=PA156 . en . 28. Pneumocystosis lymphadenitis.
  6. Carmona . Eva M. . Limper . Andrew H. . Update on the diagnosis and treatment of Pneumocystis pneumonia . Therapeutic Advances in Respiratory Disease . February 2011 . 5 . 1 . 41–59 . 10.1177/1753465810380102 . 20736243 . 1753-4666. 6886706 .
  7. Book: Zaki . Sherif R. . Alves . Venancio A. F. . Hale . Gillian L. . Burt . Alastair D. . Ferrell . Linda D. . Hubscher . Stefan G. . MacSween's Pathology of the Liver . 2018 . Elsevier . Philadelphia . 978-0-323-50869-8 . 7th . https://books.google.com/books?id=kMUmDwAAQBAJ&pg=PA477 . en . 7. Non-hepatotropic viral, bacterial, and parasitic infections of the liver. 477.
  8. Book: O'Malley . Dennis P. . Atlas of Spleen Pathology . 2013 . Springer . 978-1-4614-4671-2 . 151–155 . https://books.google.com/books?id=zy8t7IhoTIUC&pg=PA154 . en . 7.4. Infections: Fungi.
  9. Book: Papaliodis, George N. . George N. Papaliodis. Uveitis: A Practical Guide to the Diagnosis and Treatment of Intraocular Inflammation. https://books.google.com/books?id=rvUODgAAQBAJ&pg=PA71. 2017. Springer. 978-3-319-09125-9. 71. 9. Pneumocystis jirovecii.
  10. Book: Gigliotti . Frances . Wright . Terry W. . Hospenthal . Duane R. . Rinaldi . Michael G. . Diagnosis and Treatment of Human Mycoses . 2008 . Humana Press . Totowa, New Jersey . 978-1-59745-325-7 . https://books.google.com/books?id=4e5xrKOOsNEC&pg=PA249 . en . 13. Pneumocystosis.
  11. Web site: Pneumocystis pneumonia Fungal Diseases CDC . www.cdc.gov . 26 July 2021 . https://web.archive.org/web/20210726083526/https://www.cdc.gov/fungal/diseases/pneumocystis-pneumonia/index.html . 26 July 2021 . en-us . 1 December 2020.
  12. Web site: CDC - DPDx - Pneumocystis . www.cdc.gov . 25 July 2021 . https://web.archive.org/web/20210725110001/https://www.cdc.gov/dpdx/pneumocystis/index.html . 25 July 2021 . en-us . 22 January 2019.
  13. Web site: Pneumocystosis DermNet NZ . dermnetnz.org . 24 July 2021 . https://web.archive.org/web/20210724110801/https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=723 . 24 July 2021.