IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES A Woman with Progressive Dyspnea and Multiple Lung Consolidative Lesions Filippo Lococo1, Giulio Rossi2, Angelina Filice3, Napoleone Prandini4, Cristian Rapicetta1, and Massimiliano Paci1 1 Unit of Thoracic Surgery, 2Division of Pathology, and 3Department of Nuclear Medicine, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS)-Arcispedale Santa Maria Nuova, Reggio Emilia, Italy; 4Department of Nuclear Medicine, Modena University Hospital, Modena, Italy
Figure 1. (A and B) Radiological findings: multiple bilateral consolidative lung lesions, some with pseudonodular appearance, were detected by a computed tomography scan. (C and D) On an 18F-fludeoxyglucose–positron emission tomography/computed tomography scan, the lung lesions (yellow arrows) showed a moderate up to intense uptake (standard uptake value max = 2.9) of the radiotracer.
A 65-year-old woman presented with nonproductive cough and slowly progressive dyspnea. Chest X-ray and high-resolution computed tomography imaging revealed multiple bilateral consolidative lung lesions, some with a pseudonodular appearance (Figures 1A and 1B). A PET/computed tomography scan (Figures 1C and 1D) showed moderate uptake of the radiotracer at the level of the lung lesions (standard uptake value max = 2.9). Differential diagnosis consisted of pulmonary involvement of inflammatory or connective tissue disease and neoplastic lung dissemination of an unknown tumor. The lung pathology acquired from a surgical biopsy revealed (Figure 2) hyperplastic pneumocytes with intraalveolar deposit of fibrin (black arrows, Figure 2) and a background of organizing pneumonia (OP) with patchy distribution.
Author Contributions: Conception and design: F.L., G.R., and M.P.; analysis and interpretation: A.F. and G.R.; drafting the manuscript for important intellectual content: C.R. and N.P. Am J Respir Crit Care Med Vol 190, Iss 6, pp e22–e23, Sep 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201310-1759IM Internet address: www.atsjournals.org
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 6 | September 15 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. Open lung biopsy showed an acute lung injury with hyperplastic pneumocytes, intraalveolar deposit of fibrin (black arrows), and organizing pneumonia.
A diagnosis of acute fibrinous and organizing pneumonia (AFOP) was made. AFOP is a very rare descriptive pathologic pattern (about 30 cases reported in the literature [1, 2]), indicating a peculiar morphologic form of acute lung injury in the great majority of cases, but also reported in less dramatic cases simulating OP. In addition, AFOP represents a histologic pattern, recently recognized by the American Thoracic Society and the European Respiratory Society (3), that can be observed in different conditions, such as hypersensitivity pneumonia, infections, pulmonary drug toxicity, hematological malignancies, and connective diseases (4). As in our case, some cases of AFOP are idiopathic, because a meticulous clinical work-up failed to demonstrate any associated risk factors. Distinguishing AFOP from OP can be challenging and requires a careful histologic examination demonstrating the presence of intraalveolar fibrin deposition akin to OP with hyperplastic pneumocytes. Indeed, AFOP might be considered a variant of OP. Since fibrin-like material may be observed as a procedural artifact associated with transbronchial biopsy, a reliable diagnosis of either OP or AFOP may require thoracoscopic or open lung biopsy. A biopsy is likely not needed solely to distinguish AFOP from cryptogenic organizing pneumonia. In the present case, oral administration of corticosteroids was started with complete clinical and radiological remission in about 6 weeks. n Author disclosures are available with the text of this article at www.atsjournals.org. Acknowledgment: The authors thank Dr. Cavazza Alberto and Dr. Graziano Paolo (pathologists who are experts on interstitial lung diseases) for their precious contribution (“confirmatory opinion”).
References 1. Lopez-Cuenca ´ S, Morales-Garc´ıa S, Martı´n-Hita A, Frutos-Vivar F, Fernandez-Segoviano ´ P, Esteban A. Severe acute respiratory failure secondary to acute fibrinous and organizing pneumonia requiring mechanical ventilation: a case report and literature review. Respir Care 2012;57:1337–1341. 2. Beasley MB, Franks TJ, Galvin JR, Gochuico B, Travis WD. Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med 2002;126:1064–1070.
3. Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, et al.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188: 733–748. 4. Hariri LP, Unizony S, Stone J, Mino-Kenudson M, Sharma A, Matsubara O, Mark EJ. Acute fibrinous and organizing pneumonia in systemic lupus erythematosus: a case report and review of the literature. Pathol Int 2010;60:755–759.
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Figure 1. (A and B) Radiological findings: multiple bilateral consolidative lung lesions, some with pseudonodular appearance, were detected by a computed tomography scan. (C and D) On an 18F-fludeoxyglucose–positron emission tomography/computed tomography scan, the lung lesions (yellow arrows) showed a moderate up to intense uptake (standard uptake value max = 2.9) of the radiotracer.
A 65-year-old woman presented with nonproductive cough and slowly progressive dyspnea. Chest X-ray and high-resolution computed tomography imaging revealed multiple bilateral consolidative lung lesions, some with a pseudonodular appearance (Figures 1A and 1B). A PET/computed tomography scan (Figures 1C and 1D) showed moderate uptake of the radiotracer at the level of the lung lesions (standard uptake value max = 2.9). Differential diagnosis consisted of pulmonary involvement of inflammatory or connective tissue disease and neoplastic lung dissemination of an unknown tumor. The lung pathology acquired from a surgical biopsy revealed (Figure 2) hyperplastic pneumocytes with intraalveolar deposit of fibrin (black arrows, Figure 2) and a background of organizing pneumonia (OP) with patchy distribution.
Author Contributions: Conception and design: F.L., G.R., and M.P.; analysis and interpretation: A.F. and G.R.; drafting the manuscript for important intellectual content: C.R. and N.P. Am J Respir Crit Care Med Vol 190, Iss 6, pp e22–e23, Sep 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201310-1759IM Internet address: www.atsjournals.org
e22
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 6 | September 15 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. Open lung biopsy showed an acute lung injury with hyperplastic pneumocytes, intraalveolar deposit of fibrin (black arrows), and organizing pneumonia.
A diagnosis of acute fibrinous and organizing pneumonia (AFOP) was made. AFOP is a very rare descriptive pathologic pattern (about 30 cases reported in the literature [1, 2]), indicating a peculiar morphologic form of acute lung injury in the great majority of cases, but also reported in less dramatic cases simulating OP. In addition, AFOP represents a histologic pattern, recently recognized by the American Thoracic Society and the European Respiratory Society (3), that can be observed in different conditions, such as hypersensitivity pneumonia, infections, pulmonary drug toxicity, hematological malignancies, and connective diseases (4). As in our case, some cases of AFOP are idiopathic, because a meticulous clinical work-up failed to demonstrate any associated risk factors. Distinguishing AFOP from OP can be challenging and requires a careful histologic examination demonstrating the presence of intraalveolar fibrin deposition akin to OP with hyperplastic pneumocytes. Indeed, AFOP might be considered a variant of OP. Since fibrin-like material may be observed as a procedural artifact associated with transbronchial biopsy, a reliable diagnosis of either OP or AFOP may require thoracoscopic or open lung biopsy. A biopsy is likely not needed solely to distinguish AFOP from cryptogenic organizing pneumonia. In the present case, oral administration of corticosteroids was started with complete clinical and radiological remission in about 6 weeks. n Author disclosures are available with the text of this article at www.atsjournals.org. Acknowledgment: The authors thank Dr. Cavazza Alberto and Dr. Graziano Paolo (pathologists who are experts on interstitial lung diseases) for their precious contribution (“confirmatory opinion”).
References 1. Lopez-Cuenca ´ S, Morales-Garc´ıa S, Martı´n-Hita A, Frutos-Vivar F, Fernandez-Segoviano ´ P, Esteban A. Severe acute respiratory failure secondary to acute fibrinous and organizing pneumonia requiring mechanical ventilation: a case report and literature review. Respir Care 2012;57:1337–1341. 2. Beasley MB, Franks TJ, Galvin JR, Gochuico B, Travis WD. Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med 2002;126:1064–1070.
3. Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, et al.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188: 733–748. 4. Hariri LP, Unizony S, Stone J, Mino-Kenudson M, Sharma A, Matsubara O, Mark EJ. Acute fibrinous and organizing pneumonia in systemic lupus erythematosus: a case report and review of the literature. Pathol Int 2010;60:755–759.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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