Acute Fibrinous And Organizing Pneumonia Research Articles

Acute fibrinosis and organized pneumonia

Acute fibrinous and organizing pneumonia (AFOP), first described histologically by Beasley et al.1 in 2002. AFOP occurs in a wide age range (38-78 age) and in a non-sexist spectrum of patients. Although idiopathic cases have been reported, case series in which the underlying etiology is known. The histologically specific pattern is the presence of organized intra-alveolar fibrin and is the essential parameter for diagnosis. There is no significant difference in the radiological pattern except for the halo finding in the comparison of AFOP and COP. In patients presenting with an acute and more fulminant picture, the clinic presents with rapidly worsening respiratory failure. The main complaints were fever, cough and chest pain respectively. Since AFOP is a diagnosis of exclusion, most patients are diagnosed with pneumonia that does not respond to treatment or has delayed resolution during follow up. Although the clinical presentations of the fulminant and subacute forms of AFOP are different, a clear distinction cannot be made for treatment due to the high mortality of the fulminant form. The prognosis is poor in acute fulminant cases.

Acute fibrinous and organizing pneumonia (AFOP): A case series.

Acute fibrinous and organizing pneumonia (AFOP): A case series.

Everolimus-induced acute fibrinous and organizing pneumonia (AFOP).

Everolimus-induced acute fibrinous and organizing pneumonia (AFOP).

Acute Fibrinous and Organizing Pneumonia

Acute fibrinous and organizing pneumonia (AFOP) is a very rare form of acute or subacute lung injury, which is characterized by patches of fibrin deposits within the alveoli. There are no typical clinical features, and hence, it is often misdiagnosed as pneumonia, tuberculosis, or acute lung injury. A definitive diagnosis is obtained from a lung biopsy. AFOP responds to corticosteroids. Here, we present a case of AFOP and discuss the clinical manifestations, radiological findings, and management.

(1217) Acute Fibrinous and Organizing Pneumonia (AFOP) in Lung Transplant Recipient: A Case Report of Successful Treatment with Infliximab® and Tocilizumab®

(1217) Acute Fibrinous and Organizing Pneumonia (AFOP) in Lung Transplant Recipient: A Case Report of Successful Treatment with Infliximab® and Tocilizumab®

ACUTE FIBRINOUS AND ORGANIZING PNEUMONIA (AFOP) PRESENTING AS DIFFUSE CAVITARY LESIONS IN THE SETTING OF EPSTEIN BARR VIRUS (EBV)-INDUCED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH)

ACUTE FIBRINOUS AND ORGANIZING PNEUMONIA (AFOP) PRESENTING AS DIFFUSE CAVITARY LESIONS IN THE SETTING OF EPSTEIN BARR VIRUS (EBV)-INDUCED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH)

Acute fibrinous and organizing pneumonia following the COVID-19 mRNA-1273 vaccine

A 57-year-old man developed respiratory symptoms 72 hours after receiving his third dose of the COVID-19 mRNA-1273 vaccine and underwent computed tomography (CT) chest imaging. This showed new diffuse subpleural and peribronchovascular nodular opacities with reverse halo sign. Laboratory investigations demonstrated an eosinophil count of 0.8 x 10E9/L and unremarkable connective tissue disease screen. Bronchoscopy with lavage and transbronchial biopsies demonstrated lymphocytic alveolitis without evidence of infection and histopathology consistent with acute fibrinous and organizing pneumonia (AFOP). He was treated with corticosteroids resulting in resolution of symptoms and CT findings. We present radiographic and histopathologic findings of AFOP following COVID-19 mRNA-1273 vaccination.

Acute Fibrinous and Organizing Pneumonia with Excellent Response to Steroid Therapy

Acute Fibrinous and Organizing Pneumonia (AFOP) is a diffuse infiltrative pulmonary disease characterized by the presence of intra alveolar fibrin and organized pneumonia. It’s a rare and relatively unknown "entity" with only a few cases described. Herein, we describe the association of acute fibrinous and organizing pneumonia with excellent response to steroid therapy. We suggest that, such an association has been very rarely reported. A32 year's old Sudanese female was presented to us in ELmek Nimer university hospital complaining of fever, cough and shortness of breath for 10 days and received a full course of antibiotics without any improvement, her condition deteriorated. The initial chest radiography showed well defined inhomogeneous pacification in the upper lobe of the left lung with no air bronchogram, Computed Tomography of the chest showed a rather well defined Lt Upper lobe mass lesion closely related to the arch of the aorta. Ultrasound guided biopsy of the lung showed a histological diagnosis of acute fibrinous and organizing pneumonia. After establishing the diagnosis of fibrinous pneumonia the patient received oral steroid (prednisone 1mg/kg) for 10 days then tapering. The patient showed rapid improvement and excellent response and discharged in good condition. Chest radiography after one month showed complete resolution of the radiological finding in her previous imaging. We recommend that AFOP should be considered in the differentials of a suspected pulmonary infection unresponsive to optimal antibiotic "therapy". AFOP may present as pulmonary mass on chest X-ray and diagnosed made with biopsy and histological examination. We conclude that AFOP may be treated with "steroids" therapy.

Characteristics of AMR Predicts Prognosis and Phenotype of CLAD

<h3>Purpose</h3> Antibody mediated rejection (AMR) has been linked to development of chronic lung allograft dysfunction (CLAD) either bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS). It has never been assessed if AMR characteristics could predict evolution towards a phenotype of CLAD and post-treatment outcomes. <h3>Methods</h3> Data from patients treated for AMR in our center with antibody depleting therapy (ADT) (plasma exchange, high dose corticosteroids, rituximab and intravenous immunoglobulin) were reviewed. We subsequently distinguished patients presenting with late acute graft failure (LAGF) or not. LAGF was defined as acute allograft failure associated with radiological or histopathological signs compatible with acute fibrinous and organizing pneumonia (AFOP), diffuse alveolar damage (DAD) or organizing pneumonia (OP). Evolution towards CLAD and survival was investigated using Fisher's exact test and Kaplan-Meier survival curve with log-rank test. Predictive factors of death after treatment were analyzed using multiple stepwise logistic regression. <h3>Results</h3> Forty eight patients were treated with ADT for AMR. At baseline, 67% presented with CLAD (38% BOS, 44% RAS, 18% mixed). 71% presented with a clinical picture of LAGF while 29% did not. After ADT, for patients who survived long enough for reevaluation (n=42), there was a majority of patients who evolved towards a CLAD phenotype BOS in the group without LAGF (BOS n=9, RAS n=4, no CLAD n=1) and towards CLAD phenotype RAS in the LAGF group (BOS n=6, RAS n=21, no CLAD n=1) (P = 0.0148). Kaplan-Meier survival curves displayed lower survival for patients with AMR and LAGF versus patients with AMR without LAGF (P=0.0335) (figure 1). Multivariate analysis showed that LAGF (P=0.0388) and acute respiratory failure (P=0.0040) were independent predictive factors for death. <h3>Conclusion</h3> AMR may present in two distinct phenotypes: one with LAGF which evolves towards RAS with poor prognosis and a second without LAGF which evolves towards BOS with better outcomes after ADT.

SARS-Cov-2 pneumonia phenotyping on imaging exams of patients submitted to minimally invasive autopsy.

BackgroundCorrelation between pathology and imaging of the new SARS-Cov-2 disease (COVID-19) is scarce. This study aimed to characterize SARS-Cov-2 pneumonia on imaging of patients submitted to minimally invasive autopsy (MIA).MethodsThis unicentric retrospective observational study included 46 consecutive patients with confirmed COVID-19 who underwent MIA. All clinical chest images were reviewed and classified for the presence and grade of viral pneumonia, as well as disease evolution. On CT, phenotypes were described as consistent with mild, moderate, or severe viral pneumonia, with or without radiological signs of organizing pneumonia (OP). In severe pneumonia, CT could also be classified as diffuse progressive OP or radiological diffuse alveolar damage (DAD). Specific features on CT were noted, including fibroproliferative signs that could indicate potential or initial fibrosis.ResultsMIA showed a heterogeneous panel of alterations, with a high prevalence of OP and acute fibrinous and organizing pneumonia (AFOP). Also, signs of interstitial fibrosis corresponded to the most prevalent pathological feature. Initial chest radiography (CXR) findings were mainly consistent with moderate or severe viral pneumonia. Most patients showed stability or improvement (reduction of opacities) on imaging. CTs were performed on 15 patients. Consolidations were found in most patients, frequently showing features consistent with an OP phenotype. Fibroproliferative changes were also prevalent on CT.ConclusionsIn this study, SARS-Cov-2 pneumonia showed heterogeneous radiological and pathological patterns. Signs of organization and potential or initial fibrosis were prevalent on both imaging and pathology. Imaging phenotyping may help to predict post-infection fibrosing interstitial pneumonitis in COVID-19.

IS ACUTE FIBRINOUS AND ORGANIZING PNEUMONIA AN EMERGING CAUSE OF DIFFUSE ALVEOLAR HEMORRHAGE?

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a rare and poorly understood form of interstitial lung disease that can present either acutely or insidiously. Among the acute manifestations of AFOP, diffuse alveolar hemorrhage (DAH) has only been described once before in the literature. CASE PRESENTATION: A 25 year old male with a history of end stage renal disease secondary to bilateral posterior urethral valves (on hemodialysis) and cutaneous neutrophilic dermatosis (Sweet syndrome) presented with the abrupt onset of severe dyspnea. Given concern for flash pulmonary edema on initial chest radiograph (Figure 1), he was placed on a nitroglycerine drip and underwent emergent hemodialysis with ultrafiltration. He subsequently developed rapidly progressive acute hypoxic respiratory failure with worsening pulmonary infiltrates (Figure 2) requiring emergent intubation and mechanical ventilation. Copious bloody secretions were noted during intubation, and the patient required multiple blood transfusions following admission. Despite mechanical ventilation with paralysis and proning, the patient developed severe adult respiratory distress syndrome with a P:F ratio nadir of 74. Chest CT revealed diffuse severe consolidative lung injury of unclear etiology (Figure 3). Bronchoscopy with serial aliquots on bronchoalveolar lavage confirmed the presence of diffuse alveolar hemorrhage. The patient’s condition improved rapidly with initiation of systemic glucocorticoids, and he was subsequently extubated on hospital day 5. Shortly after discharge home, the patient was readmitted with recurrent severe acute hypoxic respiratory failure in the setting of tapering his glucocorticoids, but he was successfully extubated within 24 hours following reinitiation of high-dose systemic glucocorticoids. Bronchoscopically-guided cryobiopsy during this admission revealed a diagnosis of AFOP, and the patient was ultimately discharged home with plans for a prolonged course of steroids and possible transition to steroid-sparing agents. DISCUSSION: Further investigations are needed to determine whether AFOP represents a distinct clinical entity, or rather the sequelae of concomitant inflammatory or autoimmune processes such as those present in this patient. Regardless of the etiology, data from case reports suggest that AFOP can be highly responsive to systemic glucocorticoids and other forms of immunosuppression. Delay in diagnosis, however, remains a significant barrier to the treatment of AFOP and portends poor prognosis in the acute form of this illness. CONCLUSIONS: The addition of this case to the literature suggests that DAH may represent an emerging manifestation of AFOP. Prompt recognition of this rare and heterogeneous disease process is imperative, as increasing evidence suggests favorable outcomes with early initiation of systemic glucocorticoids. REFERENCE #1: Crowley N, Sessler C, Miller K, Robila V, Debesa, O. Relapsing acute fibrinous and organizing pneumonia complicated by diffuse alveolar hemorrhage. Chest. 2018;154(4)(suppl):409A-410A. doi: 10.1016/j.chest.2018.08.375. REFERENCE #2: Lee JH, Yum HK, Jamous F, Santos C, Campisi A, Surani S, Lococo F, Goo JM, Yoon SH. Diagnostic procedures and clinico-radiological findings of acute fibrinous and organizing pneumonia: a systematic review and pooled analysis. Eur Radiol. 2021 Mar 31. doi: 10.1007/s00330-021-07868-z. Epub ahead of print. REFERENCE #3: Santos C, Oliveira RC, Serra P, Baptista JP, Sousa E, Casanova P, Pimentel J, Carvalho L. Pathophysiology of acute fibrinous and organizing pneumonia - Clinical and morphological spectra. Pathophysiology. 2019 Sep-Dec;26(3-4):213-217. doi: 10.1016/j.pathophys.2019.04.001. DISCLOSURES: No relevant relationships by Andrew Davis, source=Web Response No relevant relationships by Nathan Sandbo, source=Web Response

ACUTE FIBRINOUS ORGANIZING PNEUMONIA: A NEW ASSOCIATION

TOPIC: Lung Pathology TYPE: Fellow Case Reports INTRODUCTION: Acute Fibrinous and Organizing Pneumonia (AFOP) is a rare histological pattern of acute lung injury of unclear etiology. It can present with severe hypoxemia and characteristic bilateral basilar infiltrates meeting criteria for acute respiratory distress syndrome(ARDS). We present the case of a 48-year-old female with ARDS who was diagnosed with AFOP after an open lung biopsy. CASE PRESENTATION: A 48-year-old female with a past history of schizophrenia, hypertension, type 2 diabetes, osteoarthritis and monoclonal gammopathy of undetermined significance (MGUS) presented with cough and progressive shortness of breath, worsening acutely over three days. Upon arrival, the patient was hypoxic with oxygen saturation of 47%, on room air so she was emergently intubated. Initial chest X-ray revealed bilateral mid to lower zone infiltrates concerning for severe pneumonia (Picture 1). Chest CT-Angiogram ruled out pulmonary embolism. She was admitted to our intensive care unit and started on empiric broad spectrum antibiotics (Vancomycin/Piperacillin-Tazobactam/Levaquin) and ventilated using a lung-protective strategy for ARDS. Over the next 15 days, she remained ventilator dependent with high FiO2/PEEP requirements and eventually had a tracheostomy. Microbiological studies including bronchoscopic samples remained negative. An open lung biopsy was performed which showed lymphocyte and plasma cell infiltration and intra-alveolar fibrin balls with an absence of neutrophil/eosinophil infiltration and hyaline membrane formation typical of other well-recognized patterns of acute lung injury, leading to a diagnosis of AFOP (Picture 2). She was started on high dose steroids (methyprednisolone 60mg IV q8h) with prompt resolution of infiltrates and improvement in her oxygenation. DISCUSSION: (AFOP) is a rare histologic pattern of acute lung injury with multiple clinical associations but unclear etiology. Two clinical patterns have been described: i) an acute fulminant condition with severe hypoxemia and ii) a subacute respiratory illness. Diagnosis requires biopsy revealing intra-alveolar fibrin balls amidst an organizing pneumonia. AFOP has been described in patients with HIV infection, decitabine exposure, amiodarone toxicity, post hematopoietic stem cell transplantation, connective tissue diseases etc. Patients have been treated with corticosteroids, mycophenolate mofetil, cyclophosphamide, and cyclosporine with variable response. [1] CONCLUSIONS: In this case, monoclonal gammopathy of unknown significance (MGUS) represents a new association of AFOP and thus adds to our understanding of the clinical and pathological characteristics of this disease. REFERENCE #1: 1. Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report. Valim V, Rocha RH, Couto RB, Paixão TS, Serrano EV. Case Rep Rheumatol. 2012;2012:549298. doi: 10.1155/2012/549298. Epub 2012 Apr 4. DISCLOSURES: No relevant relationships by Sukhmani Boparai, source=Web Response No relevant relationships by Prangthip Charoenpong, source=Web Response No relevant relationships by Deon Ford, source=Web Response

Diagnostic procedures and clinico-radiological findings of acute fibrinous and organizing pneumonia: a systematic review and pooled analysis

To evaluate the clinico-radiological findings of acute fibrinous and organizing pneumonia (AFOP) in the literature according to whether a surgical or non-surgical biopsy was performed, as well as to identify prognostic predictors. We searched the Embase and OVID-MEDLINE databases to identify studies that presented CT findings of AFOP and had extractable individual patient data. We compared the clinical and CT findings of the patients depending on whether a surgical or non-surgical biopsy was performed and identified survival predictors using a multivariate logistic regression analysis. Eighty-one patients (surgical biopsy, n = 52; non-surgical biopsy, n = 29) from 63 studies were included. The surgical biopsy group frequently experienced an acute fulminant presentation (p = .011) and dyspnea (p = .001) and less frequently had a fever (p = .006) than the non-surgical biopsy group. The surgical biopsy group had a worse prognosis than the non-surgical biopsy group in terms of mechanical ventilation and mortality (both, p = .023). For survival analysis, the patients with the predominant CT finding of patchy or mass-like air-space consolidation survived more frequently (p < .001) than those with other CT findings. For prognostic predictors, subacute indolent presentation (p = .001) and patchy or mass-like air-space consolidation on CT images (p = .002) were independently associated with good survival. Approximately one-third of alleged AFOP cases in the literature were diagnosed via non-surgical biopsy, but those cases had different symptomatic presentations and prognosis from surgically proven AFOP. Subacute indolent presentation and patchy or mass-like air-space consolidation at the presentation on CT images indicated a good prognosis in patients with AFOP. • Acute fibrinous and organizing pneumonia (AFOP) cases diagnosed via non-surgical biopsy had different symptomatic presentations and prognosis from surgically proven AFOP. • Subacute indolent presentation and patchy or mass-like air-space consolidation on CT images indicated a good prognosis in patients with acute fibrinous and organizing pneumonia.

Analysis of clinical characteristics of 21 cases of acute fibrinous and organizing pneumonia

Objective: To summarize the clinical features of 21 cases of acute fibrinous and organizing pneumonia (AFOP) confirmed by pathology, thereby improving clinicians' understanding of this disease and avoiding misdiagnosis in clinical practice. Methods: Twenty-one patients diagnosed pathologically with AFOP from January 2016 to April 2019 were analyzed retrospectively. The clinical symptoms, laboratory examination results, imaging features, treatments and outcomes were analyzed comprehensively. Results: There were 10 males and 11 females, with an average age of (58±10) years. All the cases presented subacute disease onset. The main symptoms were cough, expectoration and fever. The results from laboratory examination showed that the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were significantly higher than the normal levels. The total number of leukocytes, the percentage of neutrophils, and procalcitonin were also higher than the normal levels. Among these patients, 4 cases showed positive sputum bacteriology. Nine patients were found to have probable etiological factors (infections in 4, tumors in 4 cases, and connective tissue disease in 1 case). Twelve patients had no confirmed etiological factors. As to radiological findings, the patterns were multiple patchy infiltrates(16/21), solitary mass (3/21) and multiple nodules in both lungs (2/21). Most lesions were subpleural in distribution (15/21), with air bronchogram sign (11/21), pleural effusion (9/21), and cavity (4/21). Three patients received anti-infective therapy only. The infiltration in lung disappeared within 2 months in one patient, but the lesion still existed in one case after three years of follow-up. However, one patient were lost during the follow-up. Eighteen patients were treated with oral glucocorticoids, and about 50% of the patients showed significant improvement in symptoms and imaging findings within one month. The average follow-up time was (22±10) months, and there was no death. Conclusions: The clinical and imaging findings of AFOP are nonspecific. The exact mechanism of its pathogenesis is not clear. Infection and tumor may be related to the pathogenesis of AFOP. AFOP with subacute onset has a good response to glucocorticoid treatment with a better prognosis.

Late-onset "acute fibrinous and organising pneumonia" impairs long-term lung allograft function and survival.

Acute fibrinous and organising pneumonia (AFOP) after lung transplantation is associated with a rapid decline in pulmonary function. However, the relation with chronic lung allograft dysfunction (CLAD) remains unclear. We investigated the association between detection of AFOP in lung allograft biopsies with clinically important endpoints.We reviewed lung allograft biopsies from 468 patients who underwent lung transplantation at the University Hospitals Leuven (2011-2017). AFOP was categorised as early new-onset (≤90 days post-transplant) or late new-onset (>90 days post-transplant); and associated with CLAD-free survival, graft survival, donor-specific antibodies, airway and blood eosinophilia.Early and late AFOP was detected in 24 (5%) and 30 (6%) patients, respectively. CLAD-free survival was significantly lower in patients with late AFOP (median survival 2.42 years; p<0.0001) compared with patients with early or without AFOP and specifically associated with development of restrictive allograft syndrome (OR 28.57, 95% CI 11.34-67.88; p<0.0001). Similarly, graft survival was significantly lower in patients with late AFOP (median survival 4.39 years; p<0.0001) compared with patients with early AFOP or without AFOP. Late AFOP was furthermore associated with detection of circulating donor-specific antibodies (OR 4.75, 95% CI 2.17-10.60; p=0.0004) compared with patients with early or without AFOP, and elevated airway and blood eosinophilia (p=0.043 and p=0.045, respectively) compared with early AFOP patients.Late new-onset AFOP is associated with a worse prognosis and high risk of CLAD development, specifically restrictive allograft syndrome. Our findings indicate that late new-onset AFOP might play a role in the early pathogenesis of restrictive allograft syndrome.

Different Chronic Allograft Pathology Lesions in Two Orthotopic Lung Transplant Rat Strain Combinations

Chronic lung allograft dysfunction (CLAD) remains the major obstacle to long-term survival after lung transplantation. Two different phenotypes have recently been described. About 80% of patients have primarily obstructive lung disease associated with obliterative bronchiolitis, airway fibrosis with intraluminal narrowing (OB-CLAD) while a smaller subset has lung restriction with hypoxemia and parenchymal fibrosis (R-CLAD). Preclinical modeling is essential to better analyze the pathophysiological processes leading to CLAD development. Lung allografts from a large cohort of two different rat strain combinations orthotopically transplanted in the last decade were re-evaluated. From 2007 to 2017 127 rats were transplanted in our centre: 55 Lewis (Lew; RT-1l) into Fisher 344 (F344; RT-1v) (Group 1, G1) and 72 Brown-Norway (BN; RT1n) into Lew (Group 2, G2). Chronic rejection was detected in 39 cases, 18 G1 and 21 G2 rats, respectively, at a mean time of 61 and 17 days, respectively. Several pathological lesions (airway, pleural and vascular fibrosis, airway obliteration, endothelialitis, lymphoid aggregates, epithelial hyperplasia and flattening) were graded following a recent proposed grading system for animal model CLAD (Martinu et al, 2019, AJT). An end stage lung remodelling was detected in 33% of G1 and 48% of G2 models. A vanishing obliterative bronchiolitis (end stage OB-CLAD) was observed in 33% and 20% of G1 and G2 rats, respectively, while signs of fibrosis/fibroelastosis (end stage R-CLAD) were detected in 67% and 80% of G1 and G2 rats, respectively. Acute fibrinous and organizing pneumonia (AFOP) was present in 37.5% of G2 strains and never observed in G1 animals. Interestingly, G2 rats showed higher endothelialitis, vascular fibrosis and lower lymphoid aggregates than G1 strain (p=0.005, p=0.01, p=0.03, respectively). A combination score of highly suggestive R-CLAD lesions (parenchymal, pleural, vascular fibrosis and endothelialitis) was more frequently detected in G2 rats (p=0.03). CLAD associated lesions in lung rat allografts mimic those observed in humans. The earlier R-CLAD developed in G2 may be related to a higher degree of mismatch in this strain combination. These animal models could allow future mechanistic studies to better understand CLAD pathogenesis.

ACUTE FIBRINOUS AND ORGANIZING PNEUMONIA: A RARE CAUSE OF INTERSTITIAL LUNG DISEASE

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a rare cause of interstitial lung disease that is commonly misdiagnosed in patients presenting with diffuse alveolar opacities. Here we present a case of a 60-year-old previously healthy man who presented with fevers, shortness of breath and cough and was eventually diagnosed with steroid-responsive AFOP. CASE PRESENTATION: A 60-year-old otherwise healthy man presented to the hospital with shortness of breath, dry cough and recurrent fevers as high as 40C of two weeks duration. Physical exam was significant for a respiratory rate of 22, oxygen saturation on room air of 94% and bibasilar crackles. Lab work-up revealed elevated white blood cell count at 12K, erythrocyte sedimentation rate and C-reactive protein were highly elevated at >120mm and 190 mg/L, respectively. Respiratory viral panel and atypical respiratory bacterial culture were negative. Chest x-ray showed left basilar and right lower lobe opacities. After 4 days of broad-spectrum antibiotics, the patient failed to improve with paroxysmal high fevers and hypoxemia, requiring 3-5L of oxygen with nasal cannula. A high-resolution CT of the chest (Figure 1) showed worsening multi-focal parenchymal consolidation in the lower lobes and new patchy involvement in the upper lobes. Antibiotics were discontinued after bronchoscopy & bronchoalveolar lavage showed the absence of infectious organisms. Work-up for collagen vascular disease was negative. Biopsy of the lung parenchyma was done and showed prominent intra-alveolar fibrin, scattered inflammatory cells, and patchy intraluminal connective tissue consistent with AFOP. The patient was started on high dose oral prednisone of 80mg daily with a plan for a prolonged taper over 8 weeks. Over the next few days, his symptoms, oxygen requirements and chest X-ray findings all improved. The patient was discharged home with no oxygen supplementation. DISCUSSION: One of the rare histologic interstitial pneumonias, AFOP was first reported in 2002 (1). It is defined as a subgroup of idiopathic interstitial lung disease with acute lung injury characterized by intra-alveolar fibrin deposition and organization of loose connective tissue (2). Patients with AFOP tend to present with pulmonary as well as extra-pulmonary symptoms. Cough, both productive and non-productive, has been found to be the most common symptom, followed by dyspnea and fever. While AFOP can present as an idiopathic disease process, it has been found to be associated with other disease processes as well including autoimmune, infectious and connective tissue diseases.Treatment consists of systemic glucocorticoids and immunosuppression (3). CONCLUSIONS: AFOP is a rare histopathological diagnosis that requires a high degree of clinical suspicion for diagnosis. Increasing awareness of this entity might lead to earlier diagnosis and more prompt initiation of appropriate therapy. Reference #1: Beasley MB, Franks TJ, Galvin JR, et al. 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. Reference #2: Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, et al. 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(6):733–48. Reference #3: Bhatti S, Hakeem A, Torrealba J, et al. Severe acute fibrinous and organizing pneumonia (AFOP) causing ventilatory failure: successful treatment with mycophenolate mofetil and corticosteroids. Respir Med 2009;103:1764. DISCLOSURES: No relevant relationships by Shameek Gayen, source=Web Response No relevant relationships by Xiang Liu, source=Web Response No relevant relationships by Mahmoud Madi, source=Web Response

DECITABINE-INDUCED ARDS: AN UNCOMMON CLINICAL ENTITY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Infiltrative lung diseases as a result of anti-neoplastic agent toxicity are being increasingly recognized as a cause of lung disease in patients with hematological malignancies. Decitabine is a hypomethylating agent which undergoes phosphorylation and is incorporated into cell DNA causing cell death within the S-phase of the cell cycle. Pulmonary side-effects of Decitabine vary from upper respiratory tract infection to non-cardiogenic pulmonary edema (NCPE), Adult Respiratory Distress Syndrome (ARDS) and Acute fibrinous and Organizing pneumonia (AFOP). We present a case of decitabine induced ARDS. CASE PRESENTATION: A 53-year-old male with past medical history of myelodysplastic syndrome, chronic monomyelocytic leukemia type 1 and pure red cell aplasia on treatment with decitabine who was awaiting a bone marrow transplant presented with acute hypoxic respiratory failure. Patients initial CT scan was concerning for multifocal pneumonia and patient had escalating oxygen requirements requiring admission to the ICU. Due to patient’s reluctance to be intubated, he was being managed with bilevel positive airway pressure ventilation (BiPAP). Patient's exhaustive infectious work up including sputum and blood cultures remained negative. He was maintained on broad spectrum antibiotics including cefepime, vancomycin, trimethoprim-sulfamethoxazole, and voriconazole without any improvement. Patient progressed to ARDS and an elective intubation for bronchoscopy was pursued. The results of the BAL specimens including fungal/bacterial/viral cultures & opportunistic infection serologies were negative. Due to lack of improvement and persistent fevers patient was initiated on high dose steroids IV methylprednisolone 500 mg per day due to growing concern for infiltrative lung disease secondary to use of decitabine. Patient started improving and all antibiotics were subsequently discontinued. Patient improved significantly and was able to be extubated. Due to his wishes to not be intubated again he was transitioned to comfort measures and eventually passed away. DISCUSSION: Decitabine has been associated with myriad of pulmonary complications, including severe ones such as ARDS, AFOP. In phase III clinical trials of decitabine there was a 6% incidence of increased pulmonary complications as compared to placebo. Pulmonary edema not related to heart failure is described as NCPE. Pathophysiology involves fluid retention and increased capillary leak caused by cytokine release. Literature regarding pulmonary side effects of decitabine is limited to case-reports. Similar to this presentation, in those case reports as well, patient failed initial trial of antibiotics and subsequently responded to high dose steroids. CONCLUSIONS: ARDS is a rare but known complication of decitabine that should be considered when dealing a patient who has worsening respiratory failure while on therapy with this anti-neoplastic agent. Reference #1: Vahid B, Marik PE. Infiltrative lung diseases: complications of novel antineoplastic agents in patients with hematological malignancies. Can Respir J. 2008;15(4):211-6. Reference #2: Oskuei, Assad & Alvarez, Miguel. (2014). Acute Fibrinous and Organizing Pneumonia: A Severe Side Effect of Decitabine. CHEST Journal. 146. 306A. 10.1378/chest.1988926. DISCLOSURES: No relevant relationships by Jennifer Kanaan, source=Web Response No relevant relationships by Gaurav Manek, source=Web Response No relevant relationships by Rudra Ramanathan, source=Web Response No relevant relationships by Nihar Shah, source=Web Response No relevant relationships by Toishi Sharma, source=Web Response

The Prognostic Value of Fibrin and Organizing Pneumonia in Transbronchial Biopsies from Lung Transplant Patients

Purpose Acute fibrinous and organizing pneumonia (AFOP) after lung transplantation has recently gained attention as it may result in a rapid decline in respiratory function and death; or chronic lung allograft dysfunction (CLAD) in patients surviving the acute onset. We investigated the effect of fibrin deposition and organizing pneumonia (OP) in transbronchial biopsies (TBB) from lung transplant patients on graft survival and CLAD-free survival. Methods We reviewed all pathology reports of TBB obtained from 468 patients who underwent lung transplantation in the University Hospital Leuven between 2011 and 2017 (follow-up until 9/2018). The presence of intra-alveolar fibrin deposition or OP was assessed and defined as early ( 3 months). CLAD was defined as a persistent FEV1 decline ( 10% decline in total lung capacity and the presence of persistent radiological opacities. Results The presence of early and late fibrin deposition or OP was detected in 23 (5%) and 28 (6%) patients, respectively. CLAD was diagnosed in 107 (22.9%) of 468 patients (median time to CLAD = 2.90y, IQR [1.41-4.78]), in 4 of 23 patients (17.4%) with early fibrin or OP (1 RAS +), and in 16 of 28 patients (57.1%) with late fibrin or OP (12 RAS +). CLAD-free survival was significantly lower in patients with late fibrin or OP (p 7.68y and >7.64y, respectively). The presence of late fibrin or OP significantly increased the risk of later RAS (OR: 13.31; CI [3.871 - 40.2], p 7.81y and >7.77y, respectively). Patients with late fibrin or OP mostly died from CLAD (n=10, 9 RAS +), humoral rejection (n=2), sepsis (n=1), or tuberculosis (n=1). Conclusion The pathologist should actively search for fibrin and OP in TBB, as it is associated with worse prognosis and a high risk of subsequent CLAD development, specifically RAS. This indicates that the presence of late fibrin and OP in TBB could be an early pathologic manifestation of later RAS development.

Acute fibrinous and organizing pneumonia as initial presentation of primary Sjögren’s syndrome: a case report and literature review

Acute fibrinous and organizing pneumonia (AFOP) is a new histopathological pattern of acute lung injury first described by Beasley et al. in 2002. Hallmarks of pathological findings are characterized by the presence of intra-alveolar fibrin in the form of fibrin "balls" within the alveolar spaces and organizing pneumonia with a patchy distribution. Patients with AFOP may have an acute or subacute clinical presentation. Although the pathogenesis of AFOP is not fully elucidated, it may be associated with autoimmune diseases. Reported herein is a patient diagnosed of acute AFOP associated with primary Sjögren's syndrome. The patient's condition promptly improved after treatment with corticosteroid.