Steroid‐Refractory Cryptogenic Organising Pneumonia ( <scp>COP</scp> ) in a Patient With Mannose‐Binding Lectin ( <scp>MBL</scp> ) Deficiency

We report a 67-year-old man with a relapse characterised by fever, respiratory failure and bilateral infiltrates following completion of a short-term empirical methylprednisolone regimen for a similar episode. Serum, upper respiratory tract and bronchoalveolar lavage samples were negative for infection, whereas an extensive antibody panel showed no remarkable findings. The presence of migratory opacities on chest imaging, a mixed cellular lavage pattern and prior steroid responsiveness supported a provisional diagnosis of cryptogenic organising pneumonia. During outpatient follow-up, frequent exacerbations occurred, barring any steroid tapering attempts. An individualised pathophysiological hypothesis is proposed for the recalcitrant course following incidental detection of mannose-binding lectin deficiency. Given the potential role of mannose binding lectin in apoptotic cell clearance and modulation of inflammation, we postulate an impingement on the disease trajectory, which has been previously observed in chronic obstructive pulmonary disease, bronchiectasis and bronchiolitis obliterans post-transplant.

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Journal
Respirology Case Reports
Published
2026-01-29
DOI
https://doi.org/10.1002/rcr2.70494
Primary Topic
Complement system in diseases
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article

Steroid‐Refractory Cryptogenic Organising Pneumonia ( COP ) in a Patient With Mannose‐Binding Lectin ( MBL ) Deficiency

Sotirios I. Sinis, Z. Daniil, Cormac McCarthy, I Dimeas et al.
Respirology Case Reports
Complement system in diseases
article

Steroid‐Refractory Cryptogenic Organising Pneumonia ( COP ) in a Patient With Mannose‐Binding Lectin ( MBL ) Deficiency

Sotirios I. Sinis, Z. Daniil, Cormac McCarthy, I Dimeas, P Kirgou
article en

Abstract

We report a 67-year-old man with a relapse characterised by fever, respiratory failure and bilateral infiltrates following completion of a short-term empirical methylprednisolone regimen for a similar episode. Serum, upper respiratory tract and bronchoalveolar lavage samples were negative for infection, whereas an extensive antibody panel showed no remarkable findings. The presence of migratory opacities on chest imaging, a mixed cellular lavage pattern and prior steroid responsiveness supported a provisional diagnosis of cryptogenic organising pneumonia. During outpatient follow-up, frequent exacerbations occurred, barring any steroid tapering attempts. An individualised pathophysiological hypothesis is proposed for the recalcitrant course following incidental detection of mannose-binding lectin deficiency. Given the potential role of mannose binding lectin in apoptotic cell clearance and modulation of inflammation, we postulate an impingement on the disease trajectory, which has been previously observed in chronic obstructive pulmonary disease, bronchiectasis and bronchiolitis obliterans post-transplant.

Respirology Case ReportsVol. 14(2)
University College Dublin (IE), University of Thessaly (GR), St. Vincent's University Hospital (IE)
Good health and well-being
Openalex Percentile: Top 73%
Complement system in diseases
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Steroid‐Refractory Cryptogenic Organising Pneumonia ( <scp>COP</scp> ) in a Patient With Mannose‐Binding Lectin ( <scp>MBL</scp> ) Deficiency — Sotirios I. Sinis, Z. Daniil, et al. · Respirology Case Reports (2026) | TGRS Research Map | TGRS