Pneumocystis jirovecci pneumonia: from mild disease to a real disaster. A pictorial review of the different radiologic patterns in acute settings

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Pneumocystis jirovecci pneumonia: from mild disease to a real disaster. A pictorial review of the different radiologic patterns in acute settings Poster No.: C-1425 Congress: ECR 2017 Type: Educational Exhibit Authors: A. Fuentealba, N. Rossel, P. Sepulveda, D. Ramirez, J. P. Durán, G. P. Zamboni, M. CASTRO; Santiago/CL Keywords: Trauma, Infection, AIDS, Treatment effects, Diagnostic procedure, Fluoroscopy, CT, Thorax, Emergency DOI: 10.1594/ecr2017/C-1425 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 26

Learning objectives After reviewing this educational exhibit you will be able to: Recognise the radiological patterns in chest x ray and chest CT Learn the typical and atypical presentation of Pneumocystis jirovecci pneumonia (PJP) in Chest CT Propose a differential diagnosis with radiological findings. Know the primary and secondary complications of PJP. Page 2 of 26

Background Introduction: Pneumocystis jirovecci pneumonia (PJP) is an opportunistic fungal infection affecting individuals with T-cell inmmunodeficiency as AIDS, corticosteroids therapy, organ transplantation, chemotherapy and monoclonal antibodies therapy. Prior to the widespread use of antibiotic prophylaxis and antiretroviral therapies (ART), P. jirovecii caused pneumonia in over half of all patients with AIDS in the course of their disease. Nevertheless, PJP remains the most common opportunistic pneumonia in HIV-infected persons, even in developed countries, being the first presenting illness in patients with previously unrecognized HIV infection. Page 3 of 26

Images for this section: Fig. 1: Case 1. 64 yo woman, user of immunomodulation for AR. Presented with cough. Department of Radiology, Clínica INDISA; Santiago, Chile Page 4 of 26

Findings and procedure details 1. Imaging Features: a) Typical Findings On chest radiographs (CR), PJP typically manifests with diffuse or perihilar fine reticular and ill-defined ground-glass opacities (GGO). Untreated, these opacities may progress to diffuse homogeneous opacification in a few days. The CR can be normal at presentation in 6% of symptomatic patients. Page 5 of 26

Fig. 1: Case 1. 64 yo woman, user of immunomodulation for AR. Presented with cough. References: Department of Radiology, Clínica INDISA; Santiago, Chile b) Atypical Findings: Atypical radiographic features are seen in 5-18% of cases of PJP. Isolated segmental or lobar consolidation can be mistaken for bacterial pneumonia. Page 6 of 26

Fig. 2: Case 2. 47 yo male, presenting with acute respiratory failure. Corticosteroid user for asthma. Negative HIV. References: Department of Radiology, Clínica INDISA; Santiago, Chile Focal nodular opacities with or without cavitation can occur, and could be confused with lung cancer, lymphoma or metastases. This nodules may show granulomatous inflammation on histopathologic examination. Fig. 3: Case 3. 59 yo male. Presented to the Emergency Room with a story of 3 week of coughing and fever. HIV status unknown. References: Department of Radiology, Clínica INDISA; Santiago, Chile PJP rarely manifest as miliary nodules, pleural effusion, endobronchial mass, or hilar and mediastinal lymphadenopathy, which may be calcified. Upper lobe disease mimicking tuberculosis can be seen in patients using aerosolized pentamidine (because of undertreatment of the lung apices). Extrapulmonary disease involving the abdominal viscera is occasionally encountered. Up to one-third of patients with PJP develop pneumatoceles, in either the acute or postinfective period. The cysts are round, oval or crescent-shaped, and range in size from 1 cm to 8.5 cm; they are usually multiple but may be Page 7 of 26

solitary.they may occur at any location, but there is a predilection for the upper lobes. Fig. 4: 36 yo male. Corticosteroid user. Presented to the ER with fever and shortness of breath References: Department of Radiology, Clínica INDISA; Santiago, Chile Spontaneous pneumothorax or pneumomediastinum due to cyst or subpleural bleb rupture may complicate the course of infected patients. Page 8 of 26

Fig. 8: Case 7. 51 yo male from Haiti. Presented with fever and acute onset dyspnea. Pneumomediastinum as an extremely rare complication from PJP (without mechanical ventilation or cyst) References: Department of Radiology, Clínica INDISA; Santiago, Chile Page 9 of 26

Fig. 9: Case 7. 51 yo male from Haiti. Continuation. References: Department of Radiology, Clínica INDISA; Santiago, Chiley 3. Findings related to temporal evolution of disease: Organizing pneumonia caused by P. jirovecii in the setting of immune reconstitution inflammatory syndrome has been described. The CT findings of PJP include diffuse GGO, consolidation and thickening of interlobular septa. Page 10 of 26

Fig. 6: Case 6. 38 yo male. Presented to the emergency room with fever. HIV (+) with ART. Chest CT in coronal (a-c) planes showing GGO in peripheral situation and core sparing. References: Department of Radiology, Clínica INDISA; Santiago, Chile Fig. 7: Case 6. Continuation. References: Department of Radiology, Clínica INDISA; Santiago, Chile Page 11 of 26

Early stages: GGO predominate Chronic, repetitive or undertreated infections: linear opacities Fig. 5: Case 5: 27 yo male, presented with 3 weeks of shortness of breath. HIV (+) without treatment. References: Department of Radiology, Clínica INDISA; Santiago, Chile The disease affects both interstitium and airspaces in an unpredictable way and thickening of interlobular septa can be the only CT manifestation of PJP. 4. Diagnostic Methods. Page 12 of 26

CT is usually not necessary in symptomatic patients with classic findings on CR. However, CT can be useful evaluating patients with suspected PJP and a normal CR, or patients who have atypical radiographic findings. In this setting, the CT may show typical ground-glass opacities and can be used to direct invasive diagnostic procedures. Normal CT is good evidence that PJP is not the cause of symptoms. Radiologic pathologic correlation GGO corresponds to alveolar filling by foamy exudates,inter-intra reticular opacities represents intersticial edema/celular infiltration and nodules are granulomatous inflammation. 5. Differential diagnosis The differential diagnosis on CT is influenced by knowledge of HIV status and CD4-count. In this patients the differential includes: Viral pneumonitis (CMV pneumonitis): similar but cysts are not present Mycobacterium tuberculosis: upper zone compromise Angioinvasive aspergillosis Immune reconstitution inflammatory syndrome Other conditions should be considered in non-aids patients: Prominent Cyst Pulmonary Langerhans histiocytosis Lymphangiomyomatosis Honeycomb lung Bronquiectasis (various causes) Reticulonodular Pattern Sarcoidosis Wegener granulomatosis Pneumoconiosis Hypersensitivity pneumonitis Kaposi sarcoma 6. Primary and secondary complications of PJP. Cystic lung disease (Central location) Page 13 of 26

Spontaneous pneumothorax (6-7% bilateral) Disseminated extrapulmonary disease (1%) Punctate / rimlike calcification within enlarged lymph nodes and abdominal viscera Page 14 of 26

Images for this section: Fig. 1: Case 1. 64 yo woman, user of immunomodulation for AR. Presented with cough. Department of Radiology, Clínica INDISA; Santiago, Chile Page 15 of 26

Fig. 2: Case 2. 47 yo male, presenting with acute respiratory failure. Corticosteroid user for asthma. Negative HIV. Department of Radiology, Clínica INDISA; Santiago, Chile Page 16 of 26

Fig. 3: Case 3. 59 yo male. Presented to the Emergency Room with a story of 3 week of coughing and fever. HIV status unknown. Department of Radiology, Clínica INDISA; Santiago, Chile Page 17 of 26

Fig. 4: 36 yo male. Corticosteroid user. Presented to the ER with fever and shortness of breath Department of Radiology, Clínica INDISA; Santiago, Chile Page 18 of 26

Fig. 8: Case 7. 51 yo male from Haiti. Presented with fever and acute onset dyspnea. Pneumomediastinum as an extremely rare complication from PJP (without mechanical ventilation or cyst) Department of Radiology, Clínica INDISA; Santiago, Chile Page 19 of 26

Fig. 9: Case 7. 51 yo male from Haiti. Continuation. Department of Radiology, Clínica INDISA; Santiago, Chiley Page 20 of 26

Fig. 6: Case 6. 38 yo male. Presented to the emergency room with fever. HIV (+) with ART. Chest CT in coronal (a-c) planes showing GGO in peripheral situation and core sparing. Department of Radiology, Clínica INDISA; Santiago, Chile Fig. 7: Case 6. Continuation. Department of Radiology, Clínica INDISA; Santiago, Chile Page 21 of 26

Fig. 5: Case 5: 27 yo male, presented with 3 weeks of shortness of breath. HIV (+) without treatment. Department of Radiology, Clínica INDISA; Santiago, Chile Page 22 of 26

Conclusion PJP can presents in a variety of patients and with all radiological patterns described in literature. Is important to report all the features that can lead to potential complications or the complications that already appear as pneumothorax or pneumomediastinum that can be life threatening. We must suspect them in order to make the diagnosis and start the treatment as soon as possible. Page 23 of 26

Personal information Andrea I Fuentealba Cargill, MD. Radiology Resident. Radiology Department, Clínica INDISA; Santiago, Chile. andrea.fuentealba@gmail.com Natalia Rossel Bustamante, MD. Radiology Resident. Radiology Department, Clínica INDISA; Santiago, Chile. nataliarosselb@gmail.com Paulina Sepúlveda Pinto, MD. Radiology Resident. Radiology Department, Clínica INDISA; Santiago, Chile. paulinasepulvedapinto@gmail.com Diego Ramírez Montes, MD. Radiology Resident. Radiology Department, Clínica INDISA; Santiago, Chile. dhrmontes@gmail.com Juan Pablo Durán Rodríguez, MD. Radiology Resident. Radiology Department, Clínica INDISA; Santiago, Chile. duranmed@gmail.com Gian Paolo Zamboni Torres, MD. Radiology Department, Clínica INDISA; Santiago, Chile. gpzamboni@gmail.com Marcelo Castro Salas, MD. Radiology Department, Clínica INDISA; Santiago, Chile. marcelo.castro@indisa.cl Page 24 of 26

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References 1. Kanne JP, Yandow DR, Meyer CA. Pneumocystis jiroveci Pneumonia: High-Resolution CT Findings in Patients With and Without HIV Infection, American Journal of Roentgenology. 2012;198: W555-W56. Page 26 of 26