Severe Acute Respiratory Failure Secondary to Acute Fibrinous and Organizing Pneumonia Requiring Mechanical Ventilation: A Case Report and Literature Review

López-Cuenca, Sonia; Morales-García, Silvia; Martín-Hita, Ana; Frutos‐Vivar, Fernando; Fernández-Segoviano, Pilar; Esteban, Andrés

doi:10.4187/respcare.01452

Cited by 25 publications

(32 citation statements)

References 13 publications

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“…Patients present with progressive dyspnea, cough, hemoptysis, fever, fatigue, or acute respiratory distress syndrome. Patients can present with an acute, rapidly progressing form associated with poor outcomes, or more commonly, a subacute form, with a gradual onset of symptoms and a more favorable prognosis [1][2][3]. Poor outcomes are associated with patients requiring mechanical ventilation [1].…”

Section: Discussionmentioning

confidence: 99%

Life after acute fibrinous and organizing pneumonia: a case report of a patient 30 months after diagnosis and review of the literature

Kuza

Matheos

Kathman

et al. 2016

Journal of Critical Care

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Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic interstitial pneumonia pattern recently described in the literature with fewer than 120 cases published. AFOP is often difficult to diagnose and may be mistaken for other pulmonary disorders such as interstitial pneumonias or pneumonitides. Patients often present with vague symptoms of cough, dyspnea, hemoptysis, fatigue, and occasionally respiratory failure. Radiological findings show diffuse patchy opacities and ground glass appearance of the lungs. On histologic examination, intra-alveolar fibrin balls are observed. We discuss a case of a man who presented with hemoptysis and dyspnea and whose open lung biopsy revealed AFOP. We will describe the presentation, diagnosis, and post-discharge course, and review the current literature. There are only 4 cases which have reported the patients' course of disease after 1 year, the longest being 2 years. To our knowledge, this is the only case of AFOP in the literature that describes the course of a patient more than 2 years after the diagnosis of AFOP, and is the most comprehensive review of the current literature.

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Section: Discussionmentioning

confidence: 99%

Life after acute fibrinous and organizing pneumonia: a case report of a patient 30 months after diagnosis and review of the literature

Kuza

Matheos

Kathman

et al. 2016

Journal of Critical Care

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“…However, in some cases, focal or diffuse organizing pneumonia predominates, in keeping with the outcome dichotomy described above. Traction bronchiectasis and fibrosis can also develop . AFOP manifesting as a solitary mass has been reported .…”

Section: Afopmentioning

confidence: 99%

“…Corticosteroid therapy and other immunosuppressive regimens have been tried in AFOP including cyclophosphamide, mycophenolate mofetil and azathioprine with variable responses …”

Section: Afopmentioning

confidence: 99%

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

et al. 2015

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In the 2013 reclassification of the idiopathic interstitial pneumonias (IIPs), two rare IIPs (idiopathic lymphoid interstitial pneumonia (LIP), idiopathic pleuroparenchymal fibroelastosis (IPPFE)) and two rare histologic patterns (acute fibrinous and organizing pneumonia (AFOP), bronchiolocentric pattern of interstitial pneumonia (BPIP)) are described. All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. LIP is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin-walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. IPPFE is defined histologically by coexisting upper lobe pleural and intraalveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. AFOP is a rare histologic pattern lying within the spectrum of acute/subacute lung injury, characterized by organizing pneumonia and intra-alveolar fibrin deposition without hyaline membranes. BPIP is characterized histologically by fibrosis and/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, AFOP and BPIP are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features.

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“…The lung biopsies showed extensive damage, with a prevalent, temporally homogeneous, and well-recognisable OP pattern, but also an interstitial component, with fibroblast proliferation and inflammatory cells causing alveolar wall thickening. No evidence of acute fibrinous OP, which usually has a severe presentation [9], was observed.…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Fibrosing Organising Pneumonia Causing Respiratory Failure with Mycophenolic Acid

Paul

Lin-Shaw²,

Joseph³

et al. 2016

Respiration

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Organising pneumonia (OP) is usually promptly responsive to corticosteroid treatment. We describe a series of 3 cases of severe, progressive, biopsy-proven fibrosing OP causing respiratory failure. All cases presented with peribronchial and subpleural consolidations, had a fibro-inflammatory infiltrative component in the alveolar septa, and only had a partial and unsatisfactory response to corticosteroids. However, they responded to mycophenolic acid (MPA) treatment with resolution of respiratory failure as well as clinical and functional improvement. MPA as an additional treatment option for aggressive forms of fibrosing OP and interstitial lung disease needs to be further explored.

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Severe Acute Respiratory Failure Secondary to Acute Fibrinous and Organizing Pneumonia Requiring Mechanical Ventilation: A Case Report and Literature Review

Cited by 25 publications

References 13 publications

Life after acute fibrinous and organizing pneumonia: a case report of a patient 30 months after diagnosis and review of the literature

Life after acute fibrinous and organizing pneumonia: a case report of a patient 30 months after diagnosis and review of the literature

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

Successful Treatment of Fibrosing Organising Pneumonia Causing Respiratory Failure with Mycophenolic Acid

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