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Pneumocystis pneumonia

pneumocystis pneumonia, pneumocystis pneumonia (pcp)

ICD9 = 1363 DiseasesDB 10160 MedlinePlus 000671 eMedicine med/1850 MeSH D011020 edit on Wikidata

Pneumocystis pneumonia PCP is a form of pneumonia, caused by the yeast-like fungus Pneumocystis jirovecii1

Pneumocystis pneumonia is not commonly found in the lungs of healthy people, but, being a source of opportunistic infection, it can cause a lung infection in people with a weak immune system Pneumocystis pneumonia is especially seen in people with cancer undergoing chemotherapy, HIV/AIDS, and the use of medications that suppress the immune system


  • 1 Signs and symptoms
    • 11 Disease course
  • 2 Diagnosis
  • 3 Prevention and treatment
  • 4 Epidemiology
    • 41 PCP and AIDS
  • 5 Nomenclature
  • 6 References

Signs and symptomsedit

Signs and symptoms of PCP include fever, non-productive cough because sputum is too viscous to become productive, shortness of breath especially on exertion, weight loss, and night sweats There is usually not a large amount of sputum with PCP unless the patient has an additional bacterial infection The fungus can invade other visceral organs such as the liver, spleen, and kidney, but only in a minority of cases

Pneumothorax is a well-known complication of PCP2 An acute history of chest pain with breathlessness and diminished breath sounds is typical of pneumothoraxcitation needed

Disease courseedit

The risk of PCP increases when CD4 positive T-cell levels are less than 200 cells/μL In these immunosuppressed individuals the manifestations of the infection are highly variable3 The disease attacks the interstitial, fibrous tissue of the lungs, with marked thickening of the alveolar septa and alveoli, leading to significant hypoxia which can be fatal if not treated aggressively In this situation LDH levels increase and gas exchange is compromised Oxygen is less able to diffuse into the blood, leading to hypoxia Hypoxia, along with high arterial carbon dioxide CO2 levels, stimulates hyper-ventilatory effort, thereby causing dyspnea breathlessness


X-ray of Pneumocystis jirovecii pneumonia There is increased opacification whiteness in the lower lungs on both sides, characteristic of Pneumocystis pneumonia

The diagnosis can be confirmed by the characteristic appearance of the chest x-ray, which shows widespread pulmonary infiltrates, and an arterial oxygen level PaO2 that is strikingly lower than would be expected from symptoms Gallium 67 scans are also useful in the diagnosis They are abnormal in approximately 90% of cases and are often positive before the chest x-ray becomes abnormal The diagnosis can be definitively confirmed by histological identification of the causative organism in sputum or bronchio-alveolar lavage lung rinse Staining with toluidine blue, silver stain, periodic-acid schiff stain, or an immunofluorescence assay will show the characteristic cysts4 The cysts resemble crushed ping-pong balls and are present in aggregates of 2 to 8 and not to be confused with Histoplasma or Cryptococcus, which typically do not form aggregates of spores or cells A lung biopsy would show thickened alveolar septa with fluffy eosinophilic exudate in the alveoli Both the thickened septa and the fluffy exudate contribute to dysfunctional diffusion capacity which is characteristic of this pneumonia

Pneumocystis infection can also be diagnosed by immunofluorescent or histochemical staining of the specimen, and more recently by molecular analysis of polymerase chain reaction products comparing DNA samples Notably, simple molecular detection of Pneumocystis jirovecii in lung fluids does not mean that a person has Pneumocystis pneumonia or infection by HIV The fungus appears to be present in healthy individuals in the general population5

Prevention and treatmentedit

In immunocompromised patients, prophylaxis with co-trimoxazole trimethoprim/sulfamethoxazole,6 atovaquone, or regular pentamidine inhalations may help prevent PCP

Antipneumocystic medication is used with concomitant steroids in order to avoid inflammation, which causes an exacerbation of symptoms about four days after treatment begins if steroids are not used By far the most commonly used medication is trimethoprim/sulfamethoxazole, but some patients are unable to tolerate this treatment due to allergies Other medications that are used, alone or in combination, include pentamidine, trimetrexate, dapsone, atovaquone, primaquine, pafuramidine maleate under investigation, and clindamycin Treatment is usually for a period of about 21 days

Pentamidine is less often used as its major limitation is the high frequency of side effects These include acute pancreatic inflammation, kidney failure, liver toxicity, decreased white blood cell count, rash, fever, and low blood sugar


Pneumocystis jirovecii

The disease PCP is relatively rare in people with normal immune systems, but common among people with weakened immune systems, such as premature or severely malnourished children, the elderly, and especially persons living with HIV/AIDS in whom it is most commonly observed17 PCP can also develop in patients who are taking immunosuppressive medications It can occur in patients who have undergone solid organ transplantation or bone marrow transplantation and after surgery8 Infections with Pneumocystis pneumonia are also common in infants with hyper IgM syndrome, an X-linked or autosomal recessive trait

The causative organism of PCP is distributed worldwide9 and Pneumocystis pneumonia has been described in all continents except Antarctica9 Greater than 75% of children are seropositive by the age of 4, which suggests a high background exposure to the organism A post-mortem study conducted in Chile of 96 persons who died of unrelated causes suicide, traffic accidents, and so forth found that 65 68% of them had pneumocystis in their lungs, which suggests that asymptomatic pneumocystis infection is extremely common10

Pneumocystis jirovecii was originally described as a rare cause of pneumonia in neonates It is commonly believed to be a commensal organism dependent upon its human host for survival The possibility of person-to-person transmission has recently gained credence, with supporting evidence coming from many different genotyping studies of Pneumocystis jirovecii isolates from human lung tissue1112 For example, in one outbreak of 12 cases among transplant patients in Leiden, it was suggested as likely, but not proven, that human-to-human spread may have occurred13

PCP and AIDSedit

Since the start of the AIDS epidemic, PCP has been closely associated with AIDS Because it only occurs in an immunocompromised host, it may be the first clue to a new AIDS diagnosis if the patient has no other reason to be immunocompromised eg taking immunosuppressive drugs for organ transplant An unusual rise in the number of PCP cases in North America, noticed when physicians began requesting large quantities of the rarely used antibiotic pentamidine, was the first clue to the existence of AIDS in the early 1980s1415

Prior to the development of more effective treatments, PCP was a common and rapid cause of death in persons living with AIDS Much of the incidence of PCP has been reduced by instituting a standard practice of using oral co-trimoxazole Bactrim / Septra to prevent the disease in people with CD4 counts less than 200/μL In populations that do not have access to preventive treatment, PCP continues to be a major cause of death in AIDScitation needed


Both Pneumocystis pneumonia and pneumocystis pneumonia16 are orthographically correct; one uses the genus name per se and the other uses the common noun based on it This is the same reason, for example, why "group A Streptococcus" and "group A streptococcus" are both valid Synonyms for PCP include pneumocystosis16 pneumocystis + -osis, pneumocystiasis16 pneumocystis + -iasis, and interstitial plasma cell pneumonia16

The older species name Pneumocystis carinii which now applies only to the Pneumocystis species that is found in rats17 is still in common usage As a result, Pneumocystis pneumonia PCP is also known as Pneumocystis jirovecii pneumonia and incorrectly as Pneumocystis carinii pneumonia181920

Regarding nomenclature, when the name of Pneumocystis pneumonia PCP changed from P carinii pneumonia to P jirovecii pneumonia, it was at first felt that "PJP" should replace "PCP" However, because the term PCP was already used among physicians that managed patients with Pneumocystis infection, it was rationalized that the term PCP could continue to be used, as it could stand for PneumoCystis jirovecii Pneumonia21


  1. ^ a b Aliouat-Denis, C-M; et al 2008 "Pneumocystis species, co-evolution and pathogenic power" Infection, Genetics and Evolution 8 5: 708–726 PMID 18565802 doi:101016/jmeegid200805001 
  2. ^ Kanne JP1, Yandow DR, Meyer CA June 2012 "Pneumocystis jiroveci pneumonia: high-resolution CT findings in patients with and without HIV infection" AJR American Journal of Roentgenology 198 6: W555–61 PMID 22623570 doi:102214/AJR117329 CS1 maint: Multiple names: authors list link
  3. ^ Hughes WT 1996 "Pneumocystis Carinii" In Barron S; et al Barron's Medical Microbiology 4th ed University of Texas Medical Branch ISBN 0-9631172-1-1 
  4. ^ "Supplementary Information: Microscopic appearance of Pneumocystis jiroveci from bronchial washings" Retrieved 5 June 2009 
  5. ^ Medrano FJ, Montes-Cano M, Conde M, et al February 2005 "Pneumocystis jirovecii in general population" Emerging Infect Dis 11 2: 245–50 PMID 15752442 doi:103201/eid1102040487 
  6. ^ Stern A, Green H, Paul M, Vidal L, Leibovici L October 2014 "Prophylaxis for Pneumocystis pneumonia PCP in non-HIV immunocompromised patients" Cochrane Database Syst Rev 10 CD005590 PMID 25269391 doi:101002/14651858CD005590pub3 
  7. ^ Ryan KJ; Ray CG editors 2004 Sherris Medical Microbiology 4th ed McGraw Hill ISBN 0-8385-8529-9 CS1 maint: Extra text: authors list link
  8. ^ Puzio J, Kucewicz E, Sioła M, et al 2009 "Atypical and opportunistic pulmonary infections after cardiac surgery" Anestezjologia Intensywna Terapia in Polish 41 1: 41–5 PMID 19517677 
  9. ^ a b Morris A, Lundgren JD, Masur H, et al October 2004 "Current epidemiology of Pneumocystis pneumonia" Emerging Infect Dis 10 10: 1713–20 PMID 15504255 doi:103201/eid1010030985 
  10. ^ Ponce CA, Gallo M, Bustamante R, Vargas SL 2010 "Pneumocystis colonization is highly prevalent in the autopsied lungs of the general population" Clin Infect Dis 50 3: 347–353 PMID 20047487 doi:101086/649868 
  11. ^ Schmoldt S, Schuhegger R, Wendler T, et al March 2008 "Molecular evidence of nosocomial Pneumocystis jirovecii transmission among 16 patients after kidney transplantation" J Clin Microbiol 46 3: 966–71 PMC 2268360  PMID 18216217 doi:101128/JCM02016-07 
  12. ^ Morris A, Beard CB, Huang L January 2002 "Update on the epidemiology and transmission of Pneumocystis carinii" Microbes Infect 4 1: 95–103 PMID 11825780 doi:101016/S1286-45790101514-3 
  13. ^ de Boer M, Bruijnesteijn van Coppenraet L, Gaasbeek A, et al 2007 "An outbreak of Pneumocystis jiroveci pneumonia with 1 predominant genotypeamong renal transplant recipients: interhuman transmission or a common environmental source" Clin Infect Dis 44 9: 1143–9 PMID 17407029 doi:101086/513198 
  14. ^ Fannin S, Gottlieb MS, Weisman JD, et al 1982 "A Cluster of Kaposi's Sarcoma and Pneumocystis carinii pneumonia among homosexual male residents of Los Angeles and Range Counties, California" MMWR Weekly 31 32: 305–7 
  15. ^ Masur H, Michelis MA, Greene JB, et al 10 December 1981 "An outbreak of community-acquired Pneumocystis carinii pneumonia" N Engl J Med 305 24: 1431–8 PMID 6975437 doi:101056/NEJM198112103052402 
  16. ^ a b c d Elsevier, Dorland's Illustrated Medical Dictionary, Elsevier 
  17. ^ Stringer JR, Beard CB, Miller RF, Wakefield AE September 2002 "A new name Pneumocystis jiroveci for Pneumocystis from humans" Emerging Infect Dis 8 9: 891–6 PMC 2732539  PMID 12194762 doi:103201/eid0809020096 
  18. ^ Cushion MT 1998 "Ch 34: Pneumocystis carinii" In Collier, L; Balows, A; Sussman, M Topley and Wilson's Microbiology and Microbial Infections 9th ed New York: Arnold and Oxford Press pp 645–683 
  19. ^ Cushion MT 1998 "Taxonomy, genetic organization, and life cycle of Pneumocystis carinii" Semin Respir Infect 13 4: 304–312 PMID 9872627 
  20. ^ Cushion MT 2004 "Pneumocystis: unraveling the cloak of obscurity" Trends Microbiol 12 5: 243–9 PMID 15120144 doi:101016/jtim200403005 
  21. ^ Stringer, James R; Beard, Charles B; Miller, Robert F; Wakefield, Ann E Sep 2002 "A New Name for Pneumocystis from Humans and New Perspectives on the Host-Pathogen Relationship" Emerg Infect Dis 8: 891–896 PMC 2732539  PMID 12194762 doi:103201/eid0809020096 

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