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respiratory disease characterized by inflammation of the lung parenchyma (excluding the bronchi) with congestion caused by viruses or bacteria or irritants

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肺炎

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/07/14 11:02:40」(JST)

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Pneumocystis pneumonia (PCP) or pneumocystosis is a form of pneumonia, caused by the yeast-like fungus (which had previously been erroneously classified as a protozoan) Pneumocystis jirovecii. ^[1]

Pneumocystis is 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 affect the immune system.

Signs and symptoms

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 PCP. An acute history of chest pain with breathlessness and diminished breath sounds is typical of pneumothorax.^{[citation needed]}

Disease course

The risk of PCP increases when CD4 positive cell levels are less than 200 cells/μL. In these immunosuppressed individuals the manifestations of the infection are highly variable.^[2] 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 (CO
2) levels, stimulates hyper-ventilatory effort, thereby causing dyspnea (breathlessness).

Diagnosis

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 cysts.^[3] 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 population.^[4]

Prevention and treatment

In immunocompromised patients, prophylaxis with co-trimoxazole (trimethoprim/sulfamethoxazole), 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 pancreatitis, renal failure, hepatotoxicity, leukopenia, rash, fever, and hypoglycemia.

Epidemiology

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 observed).^[1]^[5] 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 surgery.^[6] 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 worldwide^[7] and Pneumocystis pneumonia has been described in all continents except Antarctica.^[7] Greater than 75% of children are seropositive by the age of 4, which suggest 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 common.^[8]

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 tissue.^[9]^[10] 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 occurred.^[11]

PCP and AIDS

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 (e.g. 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 1980s.^[12]^[13]

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 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 AIDS^{[citation needed]}.

Nomenclature

The older name Pneumocystis carinii (which now only applies to the Pneumocystis species that is found in rats^[14]) is still in common usage. As a result, Pneumocystis pneumonia (PCP) is also known as Pneumocystis jiroveci[i] pneumonia and (incorrectly) as Pneumocystis carinii pneumonia.^[15]^[16]^[17]

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 stood for PneumoCystis (jirovecii) Pneumonia.^{[citation needed]}

References

^ ^a ^b Aliouat-Denis, C-M., et al. (2008). "Pneumocystis species, co-evolution and pathogenic power". Infection, Genetics and Evolution 8 (5): 708–726. doi:10.1016/j.meegid.2008.05.001. PMID 18565802.
^ 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.
^ "Supplementary Information: Microscopic appearance of Pneumocystis jiroveci from bronchial washings". Retrieved 5 June 2009.
^ Medrano FJ, Montes-Cano M, Conde M, et al. (February 2005). "Pneumocystis jirovecii in general population". Emerging Infect. Dis. 11 (2): 245–50. doi:10.3201/eid1102.040487. PMID 15752442.
^ Ryan KJ; Ray CG (editors) (2004). Sherris Medical Microbiology (4th ed.). McGraw Hill. ISBN 0-8385-8529-9.
^ 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.
^ ^a ^b Morris A, Lundgren JD, Masur H, et al. (October 2004). "Current epidemiology of Pneumocystis pneumonia". Emerging Infect. Dis. 10 (10): 1713–20. doi:10.3201/eid1010.030985. PMID 15504255.
^ 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. doi:10.1086/649868. PMID 20047487.
^ 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. doi:10.1128/JCM.02016-07. PMC 2268360. PMID 18216217.
^ Morris A, Beard CB, Huang L (January 2002). "Update on the epidemiology and transmission of Pneumocystis carinii". Microbes Infect. 4 (1): 95–103. doi:10.1016/S1286-4579(01)01514-3. PMID 11825780.
^ 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. doi:10.1086/513198. PMID 17407029.
^ 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.
^ 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. doi:10.1056/NEJM198112103052402. PMID 6975437.
^ 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.
^ 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.
^ Cushion MT (1998). "Taxonomy, genetic organization, and life cycle of Pneumocystis carinii". Semin. Respir. Infect 13 (4): 304–312. PMID 9872627.
^ Cushion MT (2004). "Pneumocystis: unraveling the cloak of obscurity". Trends Microbiol 12 (5): 243–9. doi:10.1016/j.tim.2004.03.005. PMID 15120144.

UpToDate Contents

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1. 非HIV感染患者におけるニューモシスチス肺炎の疫学、臨床症状、および診断 epidemiology clinical manifestations and diagnosis of pneumocystis pneumonia in non hiv infected patients
2. 非HIV感染患者におけるニューモシスチス肺炎の治療および予防 treatment and prevention of pneumocystis pneumonia in non hiv infected patients
3. HIV感染患者におけるニューモシスチスによる肺感染症の臨床症状および診断 clinical presentation and diagnosis of pneumocystis pulmonary infection in hiv infected patients
4. HIV感染患者におけるニューモシスチス感染の治療および予防 treatment and prevention of pneumocystis infection in hiv infected patients
5. 難治性肺炎 nonresolving pneumonia

English Journal

Incidence of AIDS-Defining Opportunistic Infections in a Multicohort Analysis of HIV-infected Persons in the United States and Canada, 2000-2010.

Buchacz K1, Lau B2, Jing Y2, Bosch R3, Abraham AG2, Gill MJ4, Silverberg MJ5, Goedert JJ6, Sterling TR7, Althoff KN2, Martin JN8, Burkholder G9, Gandhi N10, Samji H11, Patel P1, Rachlis A12, Thorne JE2, Napravnik S13, Henry K14, Mayor A15, Gebo K2, Gange SJ2, Moore RD2, Brooks JT1; North American AIDS Cohort Collaboration on Research and Design (NA-ACCORD) of IeDEA.
The Journal of infectious diseases.J Infect Dis.2016 Sep 15;214(6):862-72. doi: 10.1093/infdis/jiw085. Epub 2016 Apr 18.
BACKGROUND: There are few recent data on the rates of AIDS-defining opportunistic infections (OIs) among human immunodeficiency virus (HIV)-infected patients in care in the United States and Canada.METHODS: We studied HIV-infected participants in 16 cohorts in the North American AIDS Cohort Collabor
PMID 27559122

ECIL guidelines for treatment of Pneumocystis jirovecii pneumonia in non-HIV-infected haematology patients.

Maschmeyer G1, Helweg-Larsen J2, Pagano L3, Robin C4, Cordonnier C5, Schellongowski P6; 6th European Conference on Infections in Leukemia (ECIL-6), a joint venture of The European Group for Blood and Marrow Transplantation (EBMT), The European Organization for Research and Treatment of Cancer (EORTC), the International Immunocompromised Host Society (ICHS) and The European LeukemiaNet (ELN).
The Journal of antimicrobial chemotherapy.J Antimicrob Chemother.2016 Sep;71(9):2405-13. doi: 10.1093/jac/dkw158. Epub 2016 May 12.
The initiation of systemic antimicrobial treatment of Pneumocystis jirovecii pneumonia (PCP) is triggered by clinical signs and symptoms, typical radiological and occasionally laboratory findings in patients at risk of this infection. Diagnostic proof by bronchoalveolar lavage should not delay the s
PMID 27550993

ECIL guidelines for preventing Pneumocystis jirovecii pneumonia in patients with haematological malignancies and stem cell transplant recipients.

Maertens J1, Cesaro S2, Maschmeyer G3, Einsele H4, Donnelly JP5, Alanio A6, Hauser PM7, Lagrou K8, Melchers WJ9, Helweg-Larsen J10, Matos O11, Bretagne S6, Cordonnier C12; 5th European Conference on Infections in Leukaemia (ECIL-5), a joint venture of the European Group for Blood and Marrow Transplantation (EBMT), the European Organisation for Research and Treatment of Cancer (EORTC), the Immunocompromised Host Society (ICHS) and the European LeukemiaNet (ELN).
The Journal of antimicrobial chemotherapy.J Antimicrob Chemother.2016 Sep;71(9):2397-404. doi: 10.1093/jac/dkw157. Epub 2016 May 12.
The 5th European Conference on Infections in Leukaemia (ECIL-5) meeting aimed to establish evidence-based recommendations for the prophylaxis of Pneumocystis jirovecii pneumonia (PCP) in non-HIV-infected patients with an underlying haematological condition, including allogeneic HSCT recipients. Reco
PMID 27550992

Japanese Journal

日和見感染症(細菌性肺炎,結核,ニューモシスチス肺炎) (第5土曜特集くすりの副作用のすべて) -- (病態からみたくすりの副作用)

大河内康実,徳田均
医学のあゆみ 251(9), 819-825, 2014-11-29
NAID 40020277093

転移性腎癌に対しテムシロリムスによる治療を施行し重症肺炎を来した1例

川原一朗,大坪智志,長谷川周二
西日本泌尿器科 = The Nishinihon journal of urology 76(10), 365-369, 2014-10
NAID 40020233712

症例びまん性すりガラス陰影から鑑別を要したAIDS関連肺カポジ肉腫の1例

塩野文子,平間崇,三尾友彦 [他]
日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 3(5), 675-679, 2014-09-10
NAID 40020210597

「ニューモシスチス肺炎」

Pneumocystis jirovecii pneumonia
	Classification and external resources
	Pneumocystis jirovecii cysts from bronchoalveolar lavage, stained with Toluidin blue O stain
ICD-10	B20.6
ICD-9	136.3
DiseasesDB	10160
MedlinePlus	000671
eMedicine	med/1850
MeSH	D011020

　　[★]

英: pneumocystis pneumonia, pneumocystis jirovecii pneumonia pneumocystis jirovecn pneumonia
同: カリニ肺炎 carinii　pneumonia、Pneumocystis肺炎; ニューモシスチス・カリニ肺炎 pneumocystis carinii　pneumonia PCP、ニューモシスチス・カリニ肺炎、ニューモシステイスカリニ肺炎
関: ニューモシスチス症 pneumocystosis

病原体

Pneumocystis jiroveci

病因

CD4+リンパ球が200/μ以下の患者に発生する

IMD.1083

剖検例で悪性リンパ腫の1/4、成人T細胞白血病の1/10、全身性エリテマトーデスの1/20 に認められる。
AIDSに高率に合併。

リスク因子

非HIV

別の易感染性の原因を有するグルココルチコイド投与患者
他の免疫抑制薬

拒絶反応を抑制する薬剤(免疫抑制薬)
プリン類似体
インフリキシマブ

細胞性免疫不全
悪性腫瘍(特に造血系悪性腫瘍)
造血幹細胞移植(HCT、特にallogeneic HCT)
固形臓器の臓器移植
拒絶反応に対する治療
炎症状態に対する治療(例えば、多発血管炎を伴う肉芽腫症など(ウェゲナー肉芽腫症)
重症の低栄養(特に低蛋白)
一次性免疫不全(特に重症複合免疫不全)
未熟児

症状

乾性咳嗽、発熱、呼吸困難

検査

末梢血液検査：リンパ球数減少。
胸部X線検査：肺門から両肺野にかけて微細な網状から索状影を認め、徐々にすりガラス様陰影となる。
呼吸機能検査：PaO2 が基準値の 1/2～1/3 に低下。PaCO2 は上昇することもある。
(USMLE:first aid step1 2006 p.147)

diagnosed by lung biopsy or lavage
identified by methenamine silver stain of lung tissue

胸部X線写真

両側性に肺門から末梢に拡がる間質性陰影

治療

通常の抗菌薬は無効
スルファメトキサゾール・トリメトプリム合剤（ST合剤）
イセチオン酸ペンタミジン：ST合剤にアレルギーを有するか、抵抗性の場合に用いられる

予後

無治療の場合、ほぼ死亡

HIV患者よりも非HIV患者の方が致死率が高い。

国試

101A013

「Pneumocystis pneumonia」

　　[★] ニューモシスチス肺炎

「Pneumocystis」

　　[★] ニューモシスチス属

[Aliouat-Denis_2008-1] Aliouat-Denis, C-M., et al. (2008). "Pneumocystis species, co-evolution and pathogenic power". Infection, Genetics and Evolution 8 (5): 708–726. doi:10.1016/j.meegid.2008.05.001. PMID 18565802.

[2] 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.

[3] "Supplementary Information: Microscopic appearance of Pneumocystis jiroveci from bronchial washings". Retrieved 5 June 2009.

[Medrano_2005-4] Medrano FJ, Montes-Cano M, Conde M, et al. (February 2005). "Pneumocystis jirovecii in general population". Emerging Infect. Dis. 11 (2): 245–50. doi:10.3201/eid1102.040487. PMID 15752442.

[Sherris-5] Ryan KJ; Ray CG (editors) (2004). Sherris Medical Microbiology (4th ed.). McGraw Hill. ISBN 0-8385-8529-9.

[pmid19517677-6] 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.

[Morris_2004-7] Morris A, Lundgren JD, Masur H, et al. (October 2004). "Current epidemiology of Pneumocystis pneumonia". Emerging Infect. Dis. 10 (10): 1713–20. doi:10.3201/eid1010.030985. PMID 15504255.

[8] 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. doi:10.1086/649868. PMID 20047487.

[9] 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. doi:10.1128/JCM.02016-07. PMC 2268360. PMID 18216217.

[10] Morris A, Beard CB, Huang L (January 2002). "Update on the epidemiology and transmission of Pneumocystis carinii". Microbes Infect. 4 (1): 95–103. doi:10.1016/S1286-4579(01)01514-3. PMID 11825780.

[11] 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. doi:10.1086/513198. PMID 17407029.

[12] 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.

[13] 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. doi:10.1056/NEJM198112103052402. PMID 6975437.

[14] 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.

[Cushion_1998-15] 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.

[Cushion_1998b-16] Cushion MT (1998). "Taxonomy, genetic organization, and life cycle of Pneumocystis carinii". Semin. Respir. Infect 13 (4): 304–312. PMID 9872627.

[Cushion_2004-17] Cushion MT (2004). "Pneumocystis: unraveling the cloak of obscurity". Trends Microbiol 12 (5): 243–9. doi:10.1016/j.tim.2004.03.005. PMID 15120144.

リンク元	「ニューモシスチス肺炎」
関連記事	「Pneumocystis pneumonia」「Pneumocystis」

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案内

pneumocystis pneumonia