PCP: Clinical Presentation Dr. Immaculate Kariuki Consultant Paediatrician Nairobi, Kenya

Intended Learning Objectives To understand the onset and presentation of the primary infection with Pneumocystis jirovecii To understand the presentation of Pneumocystis pneumonia (PCP) To review existing and commonly used childhood pneumonia algorithms that can be used for PCP

Primary Infection Primary infection is common and usually asymptomatic in immunocompetent infants Self limiting mild upper respiratory symptoms may be seen Reports of association with Sudden Unexplained Infants Death Syndrome (SIDS) RARE: Extra pulmonary manifestation involving reticuloendothelial system, auditory system or eyes

Near-universal prevalence of Pneumocystis and associated increase in mucus in the lungs of infants with Sudden Unexpected Death AUTOPSY RESULTS NUMBER PNEUMOCYSTIS DNA FOUND N ( %) Unexplained death 85 71 (83.5%) Unexplained death with mild autopsy findings 28 24 (85.7%) Explained death 15 10 (66.7%) Total 128 105 (82%) Study in Santiago, Chile.128 infants with mean age 101 days. Results Pneumocystis DNA was detected by nPCR in 105 of the 128 infants (82.0%) and Pneumocystis organisms were visualized by IF in 99 (94.3%) of the DNA-positive infants. The infection was commonest at 3–4 months with 40 of 41 (97.6%) infants of that age testing positive. MUC5AC was significantly increased in Pneumocystis-positive tissue specimens (P= .013). Death was unexplained in 113 (88.3%) infants; Pneumocystis was detected in 95 (84.0%) of them vs 10 of 15 (66.7%) with explained death (P= .28). Vargas et al Clin infect Dis 2013; 56(2):171-9.

Clinical presentation Most common presentation is pneumonia TACHYPNOEA Dry cough Low grade fever Hypoxia presenting as progressive shortness of breath Usually hypoxaemia is very marked and out of proportion with physical findings.

Clinical presentation in younger children Non specific Poor feeding and malaise Progressive respiratory distress Cyanosis or apnea

Look for general danger signs Ask if child has cough or difficulty breathing YES Ask about duration of symptoms Listen for stridor Look for lower chest wall indrawing Count respiratory rate for 1 minute NO Classify using cough & difficulty breathing charts Adapted from WHO all cause-pneumonia algorithm. Algorithm is deliberately sensitive to improve access to life saving antibiotics but is poorly specific. Ask about next main symptoms

Classification of all-cause pneumonia Cyanosed or O2 saturations <90%? Unable to drink? Reduced level of consciousness? Grunting? Y Very Severe Pneumonia Admit, IV/IM antibiotics Y Lower chest wall indrawing? Severe Pneumonia Out patient care oral Antibiotics RR ≥ 50 aged 2 –11 months RR ≥ 40 aged 1 – 4 yrs? Y Pneumonia Y None of the above? No Pneumonia Syndromic classification of pneumonia severity in the Emergency Treatment and Triage ( ETAT +) guidelines Cyanosis, SPO2 <90%, unable to drink, AVPU<A, Grunting – These are danger signs and signs of severe respiratory distress. Newer guidelines have combined the severe pneumonia and pneumonia into 1 group and advocate for outpatient oral antibiotics for both groups. http://www.who.int/maternal_child_adolescent/documents/9241546875/en/

iv) Absence of vomiting i2 i) Age < 6 months ii) Pa O2 < 92% iii) RR > 60/ minute iv) Absence of vomiting Features associated with PCP Fatti et al. Int J Infect Dis. 2006:10 (4):282-285

Post test probability using four symptoms Number of indicators Post test probability of PCP If pretest probability =10% If pretest probability=20% If pretest probability =49% Any 3 36 (18-58) 56 (33-76) 83 ( 66-92) Any 4 80 (33-97) 90 (52-96) 97 (81-99) Fatti et al. Int J Infect Dis. 2006:10 (4):282-285

Presentation in AIDS vs non AIDS Average duration of symptoms Weeks Days Respiratory rate ++ +++ Degree of hypoxaemia Mortality Lower Higher Pyrgas et al Paediatrics Review 2009 (10) : 192- 198

Summary PCP commonly presents as a hypoxic pneumonia in the context of immunosuppression indistinct from pneumonia due to other causes Widely available pneumonia algorithms may be used to make a clinical diagnosis of PCP Non HIV PCP presents more acutely and with marked hypoxia as compared to HIV PCP

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