CASE CONFERENCES The Expert Clinician Series Editors: Jess Mandel, M.D. and Peter Clardy, M.D.
A 25-Year-Old Man with Fatigue, Dyspnea, and Pulmonary Cysts Aaron M. Mulhall and Francis X. McCormack Division of Pulmonary, Critical Care, and Sleep Medicine. University of Cincinnati Medical Center, Cincinnati, Ohio
In Brief
arterial (A-a) oxygenation gradient was 35 mm Hg. A chest radiograph disclosed bilateral parenchymal abnormalities with multiple irregular and ring-like shadows (Figure 1). Computed tomographic imaging of the chest showed diffuse ground-glass opacities and parenchymal cystic changes. These cystic changes were distributed predominantly around the hila of the lungs and spared the lower lobes (Figures 2A–2C).
Case Vignette
Laboratory testing was notable for a normocytic anemia with a hemoglobin level of 11.0 g/dl (reference range, 13.2–17.1 g/dl). Other laboratory data, including the remainder of a complete blood count, metabolic panel, and liver function test results, were all normal. Arterial blood gas measured at the time of admission while the patient breathed ambient air showed a pH of 7.45, PCO2 of 35 mm Hg, PO2 of 71 mm Hg, and base excess of 0.6 mmol/L. The calculated alveolar–
A 25-year-old white male presented with a 12-month history of generalized weakness and a 15-pound unintentional weight loss. Over the previous 6 months, he had also experienced worsening fatigue, dyspnea on exertion, and a cough productive of clear sputum. He denied fever, chills, night sweats, wheezing, or stridor. He had a past history of depression. Family history was negative for respiratory diseases. He reported high-risk sexual exposures 4 years previously. He denied tobacco, alcohol, or illicit drug use. On physical examination, the patient appeared comfortable and was cachectic. Blood pressure was 117/72, heart rate was 118 beats/min, and respiratory rate was 18 breaths/min. Oxygen saturation was 94% while the patient breathed ambient air. Auscultation of the lungs revealed diffusely diminished breath sounds and scattered rhonchi throughout.
Figure 1. Chest X-ray reveals extensive bilateral parenchymal abnormality with irregular, ring-like shadows and fluid-filled cystic spaces.
A 25-year-old man presented generalized weakness, weight loss, dyspnea on exertion, and productive cough of several months duration. Initial chest imaging revealed multifocal lung cysts. This prompted consideration of a differential diagnosis for cystic lung disease.
(Received in original form July 29, 2014; accepted in final form December 4, 2014 ) Correspondence and requests for reprints should be addressed to Aaron M. Mulhall, M.D., Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati Medical Center. 231 Albert Sabin Way, MSB Room 605, Mail Location 0564, Cincinnati, OH 45267. E-mail: [email protected] Ann Am Thorac Soc Vol 12, No 1, pp 120–123, Jan 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201407-339CC Internet address: www.atsjournals.org
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CASE CONFERENCES
Figures 2. (A–C) Chest computed tomography without contrast reveals diffuse ground-glass opacities, and cystic structures in a perihilar distribution, sparing the lower lobes.
Questions 1. What is the likely diagnosis? 2. What is the most appropriate diagnostic test? 3. What is the appropriate treatment for this disease?
Clinical Reasoning Cystic lung changes can be focal, multifocal, or diffuse (involving all lobes of both lungs) (1). The differential diagnosis for multifocal cystic lung lesions is extensive, and includes infectious and noninfectious causes (Table 1). Our patient had no prior chest imaging Table 1. Differential diagnosis for pulmonary cysts Differential Diagnosis Infectious Pulmonary mycobacteriosis Coccidioidomycosis Pneumocystis jirovecii (pneumatoceles) Staphylococcus aureus (pneumatoceles) Hydatid cysts (Echinococcus granulosus or E. multilocularis) Congenital cystic lesions Bronchogenic cysts Pulmonary sequestration Congenital cystic adenomatoid malformations Centrilobular emphysema Cystic bronchiectasis (can mimic pulmonary cysts) Langerhans’s histiocytosis Desquamative interstitial pneumonia Lymphocytic interstitial pneumonia Hypersensitivity pneumonitis Lymphangioleiomyomatosis Birt-Hogg-Dube´ syndrome Pulmonary metastatic disease Light-chain deposition disease Traumatic cysts Intravenous drug use
Case Conferences: The Expert Clinician
performed at this hospital or any local hospital, so it was unclear whether the cystic changes were temporally related to his symptoms or pre-existent. The patient’s age and the rate of progression of his respiratory and constitutional symptoms led us to begin our diagnostic evaluation by considering acquired immunomodulatory diseases and chest infections. Evaluation for cystic bronchiectasis included quantitation of Ig levels, rheumatoid factor, and Sj¨ogren’s syndrome (SS) antibodies (SS-A and SS-B), all of which were within normal limits. Rapid human immunodeficiency virus (HIV) antibody testing obtained during admission infectious evaluation was positive. HIV infection was confirmed by Western blot ELISA. CD4 lymphocyte count was 19 cells/ml. Given the severity of his immunosuppression, the lung cysts were thought to be infectious in nature, and a definitive diagnosis was needed to direct the proper treatment. Bronchoscopy with bronchoalveolar lavage (BAL) of the right middle lobe was performed. A quantitative cell count performed on the BAL fluid showed macrophage predominance. Grocott-Gomori’s methenamine silver stain of the fluid was positive for Pneumocystis jirovecii. Bacterial, fungal, and acid-fast bacilli (AFB) cultures were all negative. Cytomegalovirus and Epstein-Barr virus PCR were also negative. Treatment for P. jirovecii pneumonia (PCP) was initiated with 320 mg trimethoprim (TMP)/1,600 mg sulfamethoxazole (SMX) three times a day for 21 days and 40 mg prednisone (initiated because of an A-a gradient of 35 mm Hg) tapered over 21 days.
Discussion PCP, although rare, is one of the most common opportunistic infections in
patients with HIV (2). It still remains the most common cause of respiratory failure in the era of highly active antiretroviral therapy, with a mortality rate of about 10% (3, 4). The typical PCP presentation is subacute, and includes fever, fatigue, nonproductive cough, and dyspnea on exertion. Typical chest imaging findings are bilateral, diffuse, often perihilar, reticular interstitial opacifications that can progress to focal consolidations. Rarely, nodular opacities, cavitation, and cysts can be seen, especially in immunocompromised patients. Pneumatoceles occur in 10–34% in HIV-infected subjects with PCP, but are only rarely a presenting manifestation of HIV/acquired immunodeficiency syndrome (AIDS) (5, 6). The identification of cystic lesions in an HIV-positive patient is highly suggestive of PCP. Pneumatoceles associated with PCP are almost exclusively seen in patients with AIDS. These cysts are often multifocal, upper lobe predominant, and are 1–5 cm in diameter (5, 6). There have been multiple proposed mechanisms for the development of pneumatoceles in PCP, including small airways obstruction with distal cyst formation, alveolar tissue destruction due to macrophage elastase release, and direct tissue destruction by PCP (7–9). The natural history of cystic lesions is thought to be variable. In a study by Chow and colleagues (5) that evaluated 100 patients with PCP, 34 patients had pulmonary cysts on radiographic imaging; 53% of these patients had parenchymal opacities on initial imaging that later developed into cystic lesions. The remainder had both cystic changes and opacities on initial radiographic imaging. It is thought that these cystic lesions develop after the parenchymal opacities and the associated inflammation, and tissue destruction, seen with PCP. PCP is associated with an increased incidence of spontaneous pneumothorax that has been reported to be as high as 35%. This is thought to be due to the rupturing of subpleural cysts (5, 9). The finding of a spontaneous pneumothorax in a patient with HIV is practically diagnostic of PCP. Spontaneous pneumothorax in a patient with PCP is a therapeutic dilemma, as chest tube drainage often results in persistent air 121
CASE CONFERENCES leaks that often require pleurodesis. Spontaneous pneumothorax in a patient with PCP has a significantly higher mortality when compared with patients with PCP alone (5, 9). We attribute our patient’s 6-month history of worsening dyspnea to his PCP infection. Other infectious etiologies were excluded by testing BAL fluid with bacterial culture, Legionella culture, fungal culture, AFB culture, cytomegalovirus PCR, Epstein-Barr PCR, and respiratory viral panel PCR. The clinical presentation of PCP can be subtle, often with a slow progression of dyspnea, cough, and fatigue over the course of days, weeks, or months (10). The diagnosis of PCP is generally made by direct microscopic visualization of characteristic cysts and trophic forms on appropriately stained samples of expectorated or induced sputum or BAL fluid. Typically, methenamine silver, toluidine blue, or periodic acid Schiff stains are used. Sensitivities of samples obtained by BAL or induced sputum for PCP range from 89 to 98% and 74 to 83%, respectively (11). Newer diagnostic methods include PCR and b-D-glucan assays, and are also being used to diagnose PCP. Qualitative PCR of sputum or BAL to identify P. jirovecii has reported sensitivities up to 95% and specificities greater than 90%. Quantitative PCR has the advantage of differentiating true infection from colonization of P. jirovecii; however, this specialized technique is not routinely available for clinical use (12). b-D-glucan is a component of the cell wall of a number of fungi, including P. jirovecii and various Candida and
Aspergillus species. b-D-glucan testing serves as a highly sensitive screening test for detecting PCP, but lacks sensitivity. A pooled meta-analysis of 12 studies using b-D-glucan to diagnose PCP reported a sensitivity of 96% and specificity of 84% (13). TMP/SMX has proven to be the first-line treatment for PCP in HIVpositive patients (14). It is often dosed as 320 mg TMP/1,600 mg SMX three times per day for 21 days. After initial treatment, the patient should be started on PCP prophylaxis with TMP/SMX. A number of studies have shown that treatment with TMP/SMX results in complete or partial resolution of cystic lesions, as well as improvement in pulmonary function. These studies have reported a time to radiographic improvement/ resolution ranging from 5 days up to 1 year (5). Corticosteroids can also be given, in addition to antibiotics, for PCP in HIV-positive patients. The indication for adding corticosteroids in this setting is severity of hypoxemia (PaO2 on room air 35 mm Hg) (15). The dose of corticosteroids is usually given as 40 mg prednisone twice daily for 5 days, then 40 mg daily on Days 6–11, and then 20 mg daily through Day 21 (14). Our case demonstrates the wide spectrum of chest imaging findings in patients with PCP, and emphasizes the importance of considering PCP and a new diagnosis of HIV in the differential diagnosis of multifocal cystic lung disease.
References 1 Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc 2003;78:744–752. 2 Powell K, Davis JL, Morris AM, Chi A, Bensley MR, Huang L. Survival for patients with HIV admitted to the ICU continues to improve in the current era of combination antiretroviral therapy. Chest 2009;135:11–17. 3 Austin JH, Muller ¨ NL, Friedman PJ, Hansell DM, Naidich DP, Remy-Jardin M, Webb WR, Zerhouni EA. Glossary of terms for CT of the lungs: recommendations of the Nomenclature Committee of the Fleischner Society. Radiology 1996;200:327–331. 4 Naidich DP. High-resolution computed tomography of cystic lung disease. Semin Roentgenol 1991;26:151–174. 5 Chow C, Templeton PA, White CS. Lung cysts associated with Pneumocystis carinii pneumonia: radiographic characteristics, natural history, and complications. AJR Am J Roentgenol 1993;161: 527–531.
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Answers 1. What is the likely diagnosis? PCP with pneumatoceles. 2. What is the most appropriate diagnostic test? Bronchoscopy with BAL. 3. What is the appropriate treatment for this disease? TMP/SMX and prednisone for hypoxemia (PaO2 on room air < 70 mm Hg or the A-a gradient > 35 mm Hg).
Follow-Up The patient was discharged on TMP/SMX and a prednisone taper. He returned to see an infectious disease specialist on one occasion. Thereafter, the patient moved out of state and was lost to follow-up at our institution.
Insights d
d
d
The differential diagnosis for multifocal cystic lung lesions includes both infectious and noninfectious causes (Table 1). Pulmonary cysts in a patient with a new diagnosis of HIV is highly suggestive of PCP. Pulmonary cysts occur in 10–34% of HIV-positive patients with PCP, but are only rarely a presenting manifestation of HIV/AIDS. n
Author disclosures are available with the text of this article at www.atsjournals.org.
6 Sandhu JS, Goodman PC. Pulmonary cysts associated with Pneumocystis carinii pneumonia in patients with AIDS. Radiology 1989;173:33–35. 7 Eng RH, Bishburg E, Smith SM. Evidence for destruction of lung tissues during Pneumocystis carinii infection. Arch Intern Med 1987;147: 746–749. 8 Feurestein IM, Archer A, Pluda JM, Francis PS, Falloon J, Masur H, Pass HI, Travis WD. Thin-walled cavities, cysts, and pneumothorax in Pneumocystis carinii pneumonia: further observations with histopathologic correlation. Radiology 1990;174:697–702. 9 Kuhlman JE. Pneumocystic infections: the radiologist’s perspective. Radiology 1996;198:623–635. 10 Wilkin A, Feinberg J. Pneumocystis carinii pneumonia: a clinical review. Am Fam Physician 1999;60:1699–1708, 1713–1714. 11 Huang L, Morris L, Limper AH, Beck JM; ATS Pneumocystis Workshop Participants. An official ATS workshop summary: recent advances and future directions in
AnnalsATS Volume 12 Number 1 | January 2015
CASE CONFERENCES Pneumocystis pneumonia (PCP). Proc Am Thorac Soc 2006;3: 655–664. 12 Muhlethaler ¨ K, Bogli-Stuber ¨ K, Wasmer S, von Garnier C, Dumont P, Rauch A, Muhlemann ¨ K, Garzoni C. Quantitative PCR to diagnose Pneumocystis pneumonia in immunocompromised non-HIV patients. Eur Respir J 2012;39:971–978. 13 Onishi A, Sugiyama D, Kogata Y, Saegusa J, Sugimoto T, Kawano S, Morinobu A, Nishimura K, Kumagai S. Diagnostic accuracy of serum 1,3-b-D-glucan for Pneumocystis jiroveci pneumonia, invasive candidiasis, and invasive aspergillosis: systematic review and meta-analysis. J Clin Microbiol 2012; 50:7–15.
Case Conferences: The Expert Clinician
14 Limper AH, Knox KS, Sarosi GA, Ampel NM, Bennett JE, Catanzaro A, Davies SF, Dismukes WE, Hage CA, Marr KA, et al.; American Thoracic Society Fungal Working Group. An official American Thoracic Society statement: treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med 2011;183:96–128. 15 The National Institutes of Health–University of California Expert Panel for Corticosteroids as Adjunctive Therapy for Pneumocystis Pneumonia. Consensus statement on the use of corticosteroids as adjunctive therapy for Pneumocystis pneumonia in the acquired immunodeficiency syndrome. N Engl J Med 1990;323: 1500–1504.
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A 25-Year-Old Man with Fatigue, Dyspnea, and Pulmonary Cysts Aaron M. Mulhall and Francis X. McCormack Division of Pulmonary, Critical Care, and Sleep Medicine. University of Cincinnati Medical Center, Cincinnati, Ohio
In Brief
arterial (A-a) oxygenation gradient was 35 mm Hg. A chest radiograph disclosed bilateral parenchymal abnormalities with multiple irregular and ring-like shadows (Figure 1). Computed tomographic imaging of the chest showed diffuse ground-glass opacities and parenchymal cystic changes. These cystic changes were distributed predominantly around the hila of the lungs and spared the lower lobes (Figures 2A–2C).
Case Vignette
Laboratory testing was notable for a normocytic anemia with a hemoglobin level of 11.0 g/dl (reference range, 13.2–17.1 g/dl). Other laboratory data, including the remainder of a complete blood count, metabolic panel, and liver function test results, were all normal. Arterial blood gas measured at the time of admission while the patient breathed ambient air showed a pH of 7.45, PCO2 of 35 mm Hg, PO2 of 71 mm Hg, and base excess of 0.6 mmol/L. The calculated alveolar–
A 25-year-old white male presented with a 12-month history of generalized weakness and a 15-pound unintentional weight loss. Over the previous 6 months, he had also experienced worsening fatigue, dyspnea on exertion, and a cough productive of clear sputum. He denied fever, chills, night sweats, wheezing, or stridor. He had a past history of depression. Family history was negative for respiratory diseases. He reported high-risk sexual exposures 4 years previously. He denied tobacco, alcohol, or illicit drug use. On physical examination, the patient appeared comfortable and was cachectic. Blood pressure was 117/72, heart rate was 118 beats/min, and respiratory rate was 18 breaths/min. Oxygen saturation was 94% while the patient breathed ambient air. Auscultation of the lungs revealed diffusely diminished breath sounds and scattered rhonchi throughout.
Figure 1. Chest X-ray reveals extensive bilateral parenchymal abnormality with irregular, ring-like shadows and fluid-filled cystic spaces.
A 25-year-old man presented generalized weakness, weight loss, dyspnea on exertion, and productive cough of several months duration. Initial chest imaging revealed multifocal lung cysts. This prompted consideration of a differential diagnosis for cystic lung disease.
(Received in original form July 29, 2014; accepted in final form December 4, 2014 ) Correspondence and requests for reprints should be addressed to Aaron M. Mulhall, M.D., Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati Medical Center. 231 Albert Sabin Way, MSB Room 605, Mail Location 0564, Cincinnati, OH 45267. E-mail: [email protected] Ann Am Thorac Soc Vol 12, No 1, pp 120–123, Jan 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201407-339CC Internet address: www.atsjournals.org
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AnnalsATS Volume 12 Number 1 | January 2015
CASE CONFERENCES
Figures 2. (A–C) Chest computed tomography without contrast reveals diffuse ground-glass opacities, and cystic structures in a perihilar distribution, sparing the lower lobes.
Questions 1. What is the likely diagnosis? 2. What is the most appropriate diagnostic test? 3. What is the appropriate treatment for this disease?
Clinical Reasoning Cystic lung changes can be focal, multifocal, or diffuse (involving all lobes of both lungs) (1). The differential diagnosis for multifocal cystic lung lesions is extensive, and includes infectious and noninfectious causes (Table 1). Our patient had no prior chest imaging Table 1. Differential diagnosis for pulmonary cysts Differential Diagnosis Infectious Pulmonary mycobacteriosis Coccidioidomycosis Pneumocystis jirovecii (pneumatoceles) Staphylococcus aureus (pneumatoceles) Hydatid cysts (Echinococcus granulosus or E. multilocularis) Congenital cystic lesions Bronchogenic cysts Pulmonary sequestration Congenital cystic adenomatoid malformations Centrilobular emphysema Cystic bronchiectasis (can mimic pulmonary cysts) Langerhans’s histiocytosis Desquamative interstitial pneumonia Lymphocytic interstitial pneumonia Hypersensitivity pneumonitis Lymphangioleiomyomatosis Birt-Hogg-Dube´ syndrome Pulmonary metastatic disease Light-chain deposition disease Traumatic cysts Intravenous drug use
Case Conferences: The Expert Clinician
performed at this hospital or any local hospital, so it was unclear whether the cystic changes were temporally related to his symptoms or pre-existent. The patient’s age and the rate of progression of his respiratory and constitutional symptoms led us to begin our diagnostic evaluation by considering acquired immunomodulatory diseases and chest infections. Evaluation for cystic bronchiectasis included quantitation of Ig levels, rheumatoid factor, and Sj¨ogren’s syndrome (SS) antibodies (SS-A and SS-B), all of which were within normal limits. Rapid human immunodeficiency virus (HIV) antibody testing obtained during admission infectious evaluation was positive. HIV infection was confirmed by Western blot ELISA. CD4 lymphocyte count was 19 cells/ml. Given the severity of his immunosuppression, the lung cysts were thought to be infectious in nature, and a definitive diagnosis was needed to direct the proper treatment. Bronchoscopy with bronchoalveolar lavage (BAL) of the right middle lobe was performed. A quantitative cell count performed on the BAL fluid showed macrophage predominance. Grocott-Gomori’s methenamine silver stain of the fluid was positive for Pneumocystis jirovecii. Bacterial, fungal, and acid-fast bacilli (AFB) cultures were all negative. Cytomegalovirus and Epstein-Barr virus PCR were also negative. Treatment for P. jirovecii pneumonia (PCP) was initiated with 320 mg trimethoprim (TMP)/1,600 mg sulfamethoxazole (SMX) three times a day for 21 days and 40 mg prednisone (initiated because of an A-a gradient of 35 mm Hg) tapered over 21 days.
Discussion PCP, although rare, is one of the most common opportunistic infections in
patients with HIV (2). It still remains the most common cause of respiratory failure in the era of highly active antiretroviral therapy, with a mortality rate of about 10% (3, 4). The typical PCP presentation is subacute, and includes fever, fatigue, nonproductive cough, and dyspnea on exertion. Typical chest imaging findings are bilateral, diffuse, often perihilar, reticular interstitial opacifications that can progress to focal consolidations. Rarely, nodular opacities, cavitation, and cysts can be seen, especially in immunocompromised patients. Pneumatoceles occur in 10–34% in HIV-infected subjects with PCP, but are only rarely a presenting manifestation of HIV/acquired immunodeficiency syndrome (AIDS) (5, 6). The identification of cystic lesions in an HIV-positive patient is highly suggestive of PCP. Pneumatoceles associated with PCP are almost exclusively seen in patients with AIDS. These cysts are often multifocal, upper lobe predominant, and are 1–5 cm in diameter (5, 6). There have been multiple proposed mechanisms for the development of pneumatoceles in PCP, including small airways obstruction with distal cyst formation, alveolar tissue destruction due to macrophage elastase release, and direct tissue destruction by PCP (7–9). The natural history of cystic lesions is thought to be variable. In a study by Chow and colleagues (5) that evaluated 100 patients with PCP, 34 patients had pulmonary cysts on radiographic imaging; 53% of these patients had parenchymal opacities on initial imaging that later developed into cystic lesions. The remainder had both cystic changes and opacities on initial radiographic imaging. It is thought that these cystic lesions develop after the parenchymal opacities and the associated inflammation, and tissue destruction, seen with PCP. PCP is associated with an increased incidence of spontaneous pneumothorax that has been reported to be as high as 35%. This is thought to be due to the rupturing of subpleural cysts (5, 9). The finding of a spontaneous pneumothorax in a patient with HIV is practically diagnostic of PCP. Spontaneous pneumothorax in a patient with PCP is a therapeutic dilemma, as chest tube drainage often results in persistent air 121
CASE CONFERENCES leaks that often require pleurodesis. Spontaneous pneumothorax in a patient with PCP has a significantly higher mortality when compared with patients with PCP alone (5, 9). We attribute our patient’s 6-month history of worsening dyspnea to his PCP infection. Other infectious etiologies were excluded by testing BAL fluid with bacterial culture, Legionella culture, fungal culture, AFB culture, cytomegalovirus PCR, Epstein-Barr PCR, and respiratory viral panel PCR. The clinical presentation of PCP can be subtle, often with a slow progression of dyspnea, cough, and fatigue over the course of days, weeks, or months (10). The diagnosis of PCP is generally made by direct microscopic visualization of characteristic cysts and trophic forms on appropriately stained samples of expectorated or induced sputum or BAL fluid. Typically, methenamine silver, toluidine blue, or periodic acid Schiff stains are used. Sensitivities of samples obtained by BAL or induced sputum for PCP range from 89 to 98% and 74 to 83%, respectively (11). Newer diagnostic methods include PCR and b-D-glucan assays, and are also being used to diagnose PCP. Qualitative PCR of sputum or BAL to identify P. jirovecii has reported sensitivities up to 95% and specificities greater than 90%. Quantitative PCR has the advantage of differentiating true infection from colonization of P. jirovecii; however, this specialized technique is not routinely available for clinical use (12). b-D-glucan is a component of the cell wall of a number of fungi, including P. jirovecii and various Candida and
Aspergillus species. b-D-glucan testing serves as a highly sensitive screening test for detecting PCP, but lacks sensitivity. A pooled meta-analysis of 12 studies using b-D-glucan to diagnose PCP reported a sensitivity of 96% and specificity of 84% (13). TMP/SMX has proven to be the first-line treatment for PCP in HIVpositive patients (14). It is often dosed as 320 mg TMP/1,600 mg SMX three times per day for 21 days. After initial treatment, the patient should be started on PCP prophylaxis with TMP/SMX. A number of studies have shown that treatment with TMP/SMX results in complete or partial resolution of cystic lesions, as well as improvement in pulmonary function. These studies have reported a time to radiographic improvement/ resolution ranging from 5 days up to 1 year (5). Corticosteroids can also be given, in addition to antibiotics, for PCP in HIV-positive patients. The indication for adding corticosteroids in this setting is severity of hypoxemia (PaO2 on room air 35 mm Hg) (15). The dose of corticosteroids is usually given as 40 mg prednisone twice daily for 5 days, then 40 mg daily on Days 6–11, and then 20 mg daily through Day 21 (14). Our case demonstrates the wide spectrum of chest imaging findings in patients with PCP, and emphasizes the importance of considering PCP and a new diagnosis of HIV in the differential diagnosis of multifocal cystic lung disease.
References 1 Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc 2003;78:744–752. 2 Powell K, Davis JL, Morris AM, Chi A, Bensley MR, Huang L. Survival for patients with HIV admitted to the ICU continues to improve in the current era of combination antiretroviral therapy. Chest 2009;135:11–17. 3 Austin JH, Muller ¨ NL, Friedman PJ, Hansell DM, Naidich DP, Remy-Jardin M, Webb WR, Zerhouni EA. Glossary of terms for CT of the lungs: recommendations of the Nomenclature Committee of the Fleischner Society. Radiology 1996;200:327–331. 4 Naidich DP. High-resolution computed tomography of cystic lung disease. Semin Roentgenol 1991;26:151–174. 5 Chow C, Templeton PA, White CS. Lung cysts associated with Pneumocystis carinii pneumonia: radiographic characteristics, natural history, and complications. AJR Am J Roentgenol 1993;161: 527–531.
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Answers 1. What is the likely diagnosis? PCP with pneumatoceles. 2. What is the most appropriate diagnostic test? Bronchoscopy with BAL. 3. What is the appropriate treatment for this disease? TMP/SMX and prednisone for hypoxemia (PaO2 on room air < 70 mm Hg or the A-a gradient > 35 mm Hg).
Follow-Up The patient was discharged on TMP/SMX and a prednisone taper. He returned to see an infectious disease specialist on one occasion. Thereafter, the patient moved out of state and was lost to follow-up at our institution.
Insights d
d
d
The differential diagnosis for multifocal cystic lung lesions includes both infectious and noninfectious causes (Table 1). Pulmonary cysts in a patient with a new diagnosis of HIV is highly suggestive of PCP. Pulmonary cysts occur in 10–34% of HIV-positive patients with PCP, but are only rarely a presenting manifestation of HIV/AIDS. n
Author disclosures are available with the text of this article at www.atsjournals.org.
6 Sandhu JS, Goodman PC. Pulmonary cysts associated with Pneumocystis carinii pneumonia in patients with AIDS. Radiology 1989;173:33–35. 7 Eng RH, Bishburg E, Smith SM. Evidence for destruction of lung tissues during Pneumocystis carinii infection. Arch Intern Med 1987;147: 746–749. 8 Feurestein IM, Archer A, Pluda JM, Francis PS, Falloon J, Masur H, Pass HI, Travis WD. Thin-walled cavities, cysts, and pneumothorax in Pneumocystis carinii pneumonia: further observations with histopathologic correlation. Radiology 1990;174:697–702. 9 Kuhlman JE. Pneumocystic infections: the radiologist’s perspective. Radiology 1996;198:623–635. 10 Wilkin A, Feinberg J. Pneumocystis carinii pneumonia: a clinical review. Am Fam Physician 1999;60:1699–1708, 1713–1714. 11 Huang L, Morris L, Limper AH, Beck JM; ATS Pneumocystis Workshop Participants. An official ATS workshop summary: recent advances and future directions in
AnnalsATS Volume 12 Number 1 | January 2015
CASE CONFERENCES Pneumocystis pneumonia (PCP). Proc Am Thorac Soc 2006;3: 655–664. 12 Muhlethaler ¨ K, Bogli-Stuber ¨ K, Wasmer S, von Garnier C, Dumont P, Rauch A, Muhlemann ¨ K, Garzoni C. Quantitative PCR to diagnose Pneumocystis pneumonia in immunocompromised non-HIV patients. Eur Respir J 2012;39:971–978. 13 Onishi A, Sugiyama D, Kogata Y, Saegusa J, Sugimoto T, Kawano S, Morinobu A, Nishimura K, Kumagai S. Diagnostic accuracy of serum 1,3-b-D-glucan for Pneumocystis jiroveci pneumonia, invasive candidiasis, and invasive aspergillosis: systematic review and meta-analysis. J Clin Microbiol 2012; 50:7–15.
Case Conferences: The Expert Clinician
14 Limper AH, Knox KS, Sarosi GA, Ampel NM, Bennett JE, Catanzaro A, Davies SF, Dismukes WE, Hage CA, Marr KA, et al.; American Thoracic Society Fungal Working Group. An official American Thoracic Society statement: treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med 2011;183:96–128. 15 The National Institutes of Health–University of California Expert Panel for Corticosteroids as Adjunctive Therapy for Pneumocystis Pneumonia. Consensus statement on the use of corticosteroids as adjunctive therapy for Pneumocystis pneumonia in the acquired immunodeficiency syndrome. N Engl J Med 1990;323: 1500–1504.
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