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Case Reports: Findings that shed new light on the possible pathogenesis of a disease
Relapse of idiopathic acute fibrinous and organising pneumonia 12 years after successful treatment with corticosteroids
  1. Emma McNally1,
  2. Eddie Moloney1,
  3. Siobhan Nicholson2 and
  4. Vincent Young3
  1. 1Department of Respiratory Medicine, Tallaght University Hospital, Dublin, Ireland
  2. 2Department of Pathology, St James Hospital, Dublin, Ireland
  3. 3Department of Cardiothoracic Surgery, St James Hospital, Dublin, Ireland
  1. Correspondence to Dr Emma McNally; emcnall{at}tcd.ie

Abstract

Acute fibrinous and organising pneumonia (AFOP) is a rare form of interstitial lung disease. It is a pathological diagnosis sharing similarities to organising pneumonia, diffuse alveolar damage and eosinophilic pneumonia, however, is histologically distinct, characterised by intra-alveolar fibrin deposition (‘fibrin balls’) and associated organising pneumonia. AFOP was first described in 2002, only 150 cases have been reported since. While it has been described in association with infection, autoimmune disorders, connective tissue diseases, drugs, environmental exposures and organ transplant, it can also be idiopathic in nature. AFOP follows an acute course with potential rapid fulminant respiratory failure, or a subacute trajectory with a more favourable prognosis. Corticosteroids are commonly prescribed to induce remission. While cases of relapse of AFOP during weaning or cessation of steroids have been described, there are no published cases of remote relapse of AFOP. We describe a case of idiopathic AFOP, which recurred after 12 years of good health.

  • Pneumonia (respiratory medicine)
  • Respiratory system
  • Pathology
  • Radiology

https://doi.org/10.1136/bcr-2022-250424

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    Footnotes

    • Contributors EMcNally prepared this report for publication including consenting the patient and conducting a review of the existing literature, along with preparing the manuscript. SN was involved in this patient’s care by reporting on the histology and preparing the slides for publication in this report. VY was involved in this patient’s care by performing the surgical lung biopsy. EMoloney was the overall treating respiratory consultant who continues to look after this patient and who was involved in the editing and preparation of this manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.