A fungus, when last I checked.
AIDS and hematologic malignancies have been the classic risk, there has been increasing cases in rheumatologic and other patients and may be causing more disease than suspected (PubMed).
Part of normal flora of human upper airway. There has been an outbreak with probable human to human transmission in kidney transplant patients who were not on prophylaxis (PubMed). The data does suggest that it is spread by air and nosocomial spread amongst the immunoincompetent. AIDS clinics and transplant clinics have had transmitted from patient to patient (PubMed).
Patients on temozolomide (an oral alkylating agent) for glioblastoma multiforme and refractory anaplastic astrocytoma can get PJP and Cytomegalovirus infections.
Biologics increase the risk for PJP, but also for Listeria, Salmonella, and Legionella and it has been suggested that all patients on biologics should be on TMP/Sulfa to prevent all of the above (PubMed).
Steroids as a risk? Probably. But how much and how long? 50 mg prednisone not 10 mg (PubMed)?
Ibrutinib, an irreversible inhibitor of Bruton tyrosine kinase (PubMed).
Endogenous steroids? Yep. Cushings is a risk and should get prophylaxis.
Newborns are rapidly colonized from the mother and PJP has been suggested as a cause of respiratory distress and SIDS.
Pneumonia, usually bilateral and diffuse, and often indolent, in AIDS with CD4 < 200, any lymphocytic malignancy (Hodgkin's especially on steroids (the 'P' in CHOP)), transplant patients (especially on steroids). Rare cases of disseminated disease in end stage HIV.
What are the risks?
"Estimated incidence rates could be ranked in three categories:
i) high risk (incidence rates > 45 cases per 100,000 patientyear): polyarteritis nodosa, granulomatosis with polyangiitis, polymyositis/dermatopolymyositis, acute leukemia, chronic lymphocytic leukemia, and nonHodgkin lymphoma;
ii) intermediate risk (25-45 cases per 100,000 patientyear): Waldenström macroglobulinemia, multiple myeloma, and central nervous system cancer; and
iii) low risk (< 25 cases per 100,000 patientyear): other solid tumors, inflammatory diseases, and Hodgkin lymphoma (PubMed)."
Although it is popular in my institution to give prophylaxis to almost anyone (like COPD) on steroids, as of 2014 the data isn't there and tmp/sulfa is not benign. Not that anyone pays attention to me.
Here is the pearl about PJP and high dose steroids: patients get symptomatic as the taper begins. Then the WBC start killing the PJP and the type 2 pneumocytes that PJP binds to. BTW, it is the type 2 pneumocytes death that is the source of the elevated LDH.
See it on BAL or so PCR for diagnosis; last century we would do gallium scans, back when we used stone knives and bear skins (PubMed).
The serum (1-3)-beta-D-glucan is probably the best non-invasive way to make the diagnosis (induced sputum looks great in the literature, stink in real life, and who really wants a bronch?), although remember many yeasts and molds make (1-3)-beta-D-glucan. Using a cutoff of 80 pg/will have a sensitivity of 92%, not bad.
Serum LDH level tends to roughly correlate with severity of disease, as does the serum (1-3)-beta-D-glucan level (PubMed). FYI the other two infectious diseases that cause a sky high LDH are miliary TB and disseminated Histoplasmosis.
"The most promising cutoff levels for diagnosis of PcP were determined to be 400 pg/ml of BG and 350 U/l of LDH, which combined with clinical data presented 92.8 % sensitivity, 83.9 % specificity, 92.8 % PPV, 83.9 % NPV (PubMed)."
TMP/Sulfa 4-5 mg/kg q 6 h is best (15 to 20 mg/kg/day (TMP) or 75 to 100 mg/kg/day (SMX) in 3 or 4 divided doses), then, in no particular order, pentamidine OR clindamycin (600-900 mg q 8) AND primaquine OR trimetrexate OR dapsone AND trimethoprim OR atovaquone. Adding (not substituting) caspofungin may help a failing TMP/Sulfa therapy (PubMed), although the clinical data is variable (PubMed).
Usually 21 days course, IV usually while O2 is required, then suppression. For HIV, can stop prophylaxis if CD4 > 200 AND viral load suppressed for a year.
Patients do not start to turn around until day 3-5.
In one series of non-HIV PJP, low dose (<15 mg/kg/d) TMP/sulfa had equal efficacy to standard dose but less side effects (PubMed).
Duration of treatment is at least 21 d.
There is resistance that is mediated by alterations in the dihydropteroate synthase and dihydrofolate reductase; however it may need to be combined with genotype 7 to result in antibiotic failure (CDC).
In AIDS if pO2 < 60, prednisone will prevent progression: 40 bid x 7 d, 40 q d x 7 d then 20 q d x 7 d. How about non AIDS PJP? The data suggests that steroids do little (PubMed), regardless of recent steroid use.
Primary and secondary prophylaxis is the same list above; TMP/Sulfa should be 1 ds po qd and is preferred; in HIV can stop if CD4 > 200 for 3 months (primary prophylaxis) or 6 months (secondary prophylaxis) on HAART and the viral load remains undetectable.
Inhaled pentamidine (300 q 3-4 weeks) can lead to upper lobe cavitary PJP and is the prophylaxis associated with disseminated disease.
Besides HIV with CD4 < 200, do not forget prophylaxis in lymphoma patients on steroids, transplant patients, and some patients on collagen vascular disease, although the data outside of HIV and transplant is problematic (PubMed).
If the serum LDH is normal the patient almost certainly does not have PJP, in most patients the LDH level roughly follows the severity of disease.
The second P in PJP is pneumonia. So please, lets not say 'PJP pneumonia.' OK? Like PIN number. Moronic.
The use of TMP/Sulfa prophylaxis has increased point mutations in the binding site of TMP; how important this resistance is remains uncertain but can be as high as 70% in some series. They is as of yet no way to test for this clinically, but to be considered a reason for breakthrough of PJP while on TMP/Sulfa. These resistant strains can be spread from person to person (PubMed).
Pneumocystis carinii is the species that is found in rats.
Relevant links to my Medscape blog
Last Update: 09/15/18.