Organising pneumonia
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
rapidly progressive OP
mechanical ventilation + intravenous corticosteroid followed by oral corticosteroid
Rapidly progressive OP begins with a flu-like illness and progresses to shortness of breath and respiratory failure within a few days. The patient is admitted to the ICU and supported with mechanical ventilation.
Intravenous methylprednisolone is given for 3 days, followed by oral prednisolone at a dose determined by the patient's weight. This dose is subsequently tapered over weeks to months, and patients should be discharged on oral corticosteroids.
Primary options
methylprednisolone: 250 mg intravenously every 6 hours for 3 days
and
prednisolone: 2 mg/kg/day orally initially following methylprednisolone administration, slowly taper over weeks to months
cyclophosphamide
Treatment recommended for ALL patients in selected patient group
It is the author's experience that this type of OP is generally treated the same as acute interstitial pneumonia but requires high doses of corticosteroids and cyclophosphamide.
Primary options
cyclophosphamide: consult specialist for guidance on dose
treatment of underlying causes/removal of causative factor
Treatment recommended for ALL patients in selected patient group
Drug-related OP is reversible with drug cessation.
Toxin-exposure OP can be treated by avoidance of contact with the toxin.
Post-radiation OP occurs in all regions of the lungs and will resolve without treatment.
In post-infectious OP the infection either resolves on its own (some viral pneumonias) or is treated with antibiotics or antimalarials.
pulmonary rehabilitation
Treatment recommended for ALL patients in selected patient group
This is an important part of managing the mid-to-late phase of OP (after the initial few days of treatment, with the patient ambulatory and with improving symptoms and radiographic findings). It introduces an exercise programme for improving muscle conditioning, muscle oxygen efficiency, and sense of well-being. Patients also receive guidance for an ongoing exercise programme at home or at a commercial exercise facility.
cryptogenic OP
oral corticosteroid
Prednisolone remains the treatment of choice for OP.
For most patients, 6 months of treatment is effective. In others, treatment may take 12 months.
About 5% of patients require intermittent doses for 3 to 5 years and this does not appear to affect mortality or morbidity.[57]Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7. https://www.atsjournals.org/doi/10.1164/ajrccm.162.2.9909015 http://www.ncbi.nlm.nih.gov/pubmed/10934089?tool=bestpractice.com
Primary options
prednisolone: 40-60 mg orally once daily for 4 weeks, followed by 30-40 mg once daily for 4 weeks, followed by 20 mg once daily for 4 weeks, followed by 10 mg once daily for 6 weeks, followed by 5 mg once daily for 6 weeks
pulmonary rehabilitation
Treatment recommended for ALL patients in selected patient group
This is an important part of managing the mid-to-late phase of OP (after the initial few days of treatment, with the patient ambulatory and with improving symptoms and radiographic findings). It introduces an exercise programme for improving muscle conditioning, muscle oxygen efficiency, and sense of well-being. Patients also receive guidance for an ongoing exercise programme at home or at a commercial exercise facility.
macrolide antibiotic
Additional treatment recommended for SOME patients in selected patient group
Mild disease may respond to macrolide antibiotics.[58]Stover DE, Mangino D. Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia? Chest. 2005 Nov;128(5):3611-7. https://www.sciencedirect.com/science/article/pii/S0012369215529383?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/16304320?tool=bestpractice.com However, present studies are all observational and macrolides in cryptogenic OP need to be investigated in clinical trials. Erythromycin was used successfully in 6 Japanese women, with 1 responding at 2 months and 5 responding at 3 months.[59]Ichikawa Y, Ninomiya H, Katsuki M, et al. Low-dose/long-term erythromycin for treatment of bronchiolitis obliterans organizing pneumonia (BOOP). Kurume Med J. 1993;40(2):65-7. https://www.jstage.jst.go.jp/article/kurumemedj1954/40/2/40_2_65/_article http://www.ncbi.nlm.nih.gov/pubmed/8231065?tool=bestpractice.com Clarithromycin was also used successfully.[58]Stover DE, Mangino D. Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia? Chest. 2005 Nov;128(5):3611-7. https://www.sciencedirect.com/science/article/pii/S0012369215529383?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/16304320?tool=bestpractice.com Azithromycin may also be effective. Macrolides may be useful for prevention of recurrence.
Primary options
erythromycin: consult specialist for guidance on dose
OR
azithromycin: consult specialist for guidance on dose
OR
clarithromycin: consult specialist for guidance on dose
corticosteroid-sparing agent
Additional treatment recommended for SOME patients in selected patient group
If prednisolone is not effective or its dose cannot be weaned below 40 mg/day, cyclophosphamide, azathioprine, and ciclosporin have been used with variable success as corticosteroid-sparing agents (with ongoing lower corticosteroid dose).[60]Purcell IF, Bourke SJ, Marshall SM. Cyclophosphamide in severe steroid-resistant bronchiolitis obliterans organizing pneumonia. Respir Med. 1997 Mar;91(3):175-7. https://www.sciencedirect.com/science/article/pii/S095461119790055X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/9135858?tool=bestpractice.com
In these situations, it is important to confirm that the primary process is OP (no honeycombing by high-resolution computed tomography scan), as many patients with seemingly corticosteroid-resistant OP do not have primary OP but have an underlying fibrosing process, such as usual interstitial pneumonia or non-specific interstitial pneumonia, not responsive to corticosteroid therapy. Here, OP is a secondary inflammatory lesion, responsive to corticosteroid therapy.
Primary options
cyclophosphamide: consult specialist for guidance on dose
OR
azathioprine: consult specialist for guidance on dose
OR
ciclosporin: consult specialist for guidance on dose
secondary OP
treatment of underlying cause/removal of causative factor
Drug-related OP is reversible with drug cessation.
Toxin-exposure OP can be treated by avoidance of contact with the toxin.
Post-radiation OP occurs in all regions of the lungs and will resolve without treatment.
In post-infectious OP the infection either resolves on its own (some viral pneumonias) or is treated with antibiotics or antimalarials.
pulmonary rehabilitation
Treatment recommended for ALL patients in selected patient group
This is an important part of managing the mid-to-late phase of OP (after the initial few days of treatment, with the patient ambulatory and with improving symptoms and radiographic findings). It introduces an exercise programme for improving muscle conditioning, muscle oxygen efficiency, and sense of well-being. Patients also receive guidance for an ongoing exercise programme at home or at a commercial exercise facility.
oral corticosteroid
Additional treatment recommended for SOME patients in selected patient group
The addition of corticosteroids should form a part of treatment for toxin-exposure OP as well as severe cases of post-infectious OP and rapidly progressive OP. In drug-related and post-radiation OP not responsive to the removal of the causative agent, the addition of oral corticosteroids should also be considered. When OP is associated with rheumatological or connective tissue disorders, it is often responsive to corticosteroid therapy.
For most patients, 6 months of treatment is effective. In others, treatment may take 12 months. About 5% of patients require intermittent doses for 3 to 5 years and this does not appear to affect mortality or morbidity.[57]Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7. https://www.atsjournals.org/doi/10.1164/ajrccm.162.2.9909015 http://www.ncbi.nlm.nih.gov/pubmed/10934089?tool=bestpractice.com
Primary options
prednisolone: 40-60 mg orally once daily for 4 weeks, followed by 30-40 mg once daily for 4 weeks, followed by 20 mg once daily for 4 weeks, followed by 10 mg once daily for 6 weeks, followed by 5 mg once daily for 6 weeks
corticosteroid-sparing agent
Additional treatment recommended for SOME patients in selected patient group
If prednisolone is not effective or its dose cannot be weaned below 40 mg/day, cyclophosphamide, azathioprine, and ciclosporin have been used with variable success as corticosteroid-sparing agents (with ongoing lower corticosteroid dose).[60]Purcell IF, Bourke SJ, Marshall SM. Cyclophosphamide in severe steroid-resistant bronchiolitis obliterans organizing pneumonia. Respir Med. 1997 Mar;91(3):175-7. https://www.sciencedirect.com/science/article/pii/S095461119790055X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/9135858?tool=bestpractice.com
In these situations, it is important to confirm that the primary process is OP (no honeycombing by high-resolution computed tomography scan) as many patients with seemingly corticosteroid-resistant OP do not have primary OP but have an underlying fibrosing process, such as usual interstitial pneumonia or non-specific interstitial pneumonia, not responsive to corticosteroid therapy. Here, OP is a secondary inflammatory lesion, responsive to corticosteroid therapy.
Primary options
cyclophosphamide: consult specialist for guidance on dose
OR
azathioprine: consult specialist for guidance on dose
OR
ciclosporin: consult specialist for guidance on dose
recurrent OP, rapidly progressive
intravenous corticosteroid followed by oral corticosteroid
OP may recur in up to one third of patients. The symptoms will be the same as the initial episode, and the radiograph usually has the same pattern, although new lung regions may become involved.
If recurrent OP is rapidly progressing, the patient should be admitted to the ICU and supported with mechanical ventilation.
Intravenous methylprednisolone is given for 3 days, followed by oral prednisolone at a dose determined by the patient's weight. This dose is subsequently tapered over weeks to months, and patients should be discharged on oral corticosteroid.
If an OP recurrence has been established with recurrent symptoms, recurrence radiographic findings, and deteriorating diffusing capacity, following intravenous methylprednisolone, oral prednisolone can be reinstituted at 20 mg/day higher than the dose at the time of recurrence. This new dose can be given for 3 months, then tapered.[57]Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7. https://www.atsjournals.org/doi/10.1164/ajrccm.162.2.9909015 http://www.ncbi.nlm.nih.gov/pubmed/10934089?tool=bestpractice.com
In those cases with a known cause, the underlying cause should be treated or causative factor removed.
A second and third recurrence can be treated in the same way.
Primary options
methylprednisolone: 250 mg intravenously every 6 hours for 3 days, followed by oral prednisolone
and
prednisolone: 2 mg/kg/day orally initially following methylprednisolone administration, slowly taper over weeks to months.
pulmonary rehabilitation
Treatment recommended for ALL patients in selected patient group
This is an important part of managing the mid-to-late phase of OP (after the initial few days of treatment, with the patient ambulatory and with improving symptoms and radiographic findings). It introduces an exercise programme for improving muscle conditioning, muscle oxygen efficiency, and sense of well-being. Patients also receive guidance for an ongoing exercise programme at home or at a commercial exercise facility.
lung transplantation
Additional treatment recommended for SOME patients in selected patient group
Very rarely, lung transplantation may be necessary for patients who do not respond to treatment or who have an unusual or hybrid form of OP.
recurrent OP, not rapidly progressive
oral corticosteroid
OP may recur in up to one third of patients. The symptoms will be the same as the initial episode, and the radiograph usually has the same pattern, although new lung regions may become involved.
If an OP recurrence has been established with recurrent symptoms, recurrence radiographic findings, and deteriorating diffusing capacity, prednisolone can be reinstituted at 20 mg/day higher than the dose at the time of recurrence. This new dose can be given for 3 months, then tapered.[57]Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7. https://www.atsjournals.org/doi/10.1164/ajrccm.162.2.9909015 http://www.ncbi.nlm.nih.gov/pubmed/10934089?tool=bestpractice.com
In those cases with a known cause, the underlying cause should be treated or causative factor removed.
A second and third recurrence can be treated in the same way.
Primary options
prednisolone: 20 mg/day higher than dose at time of recurrence orally once daily for 12 weeks, then gradually taper according to response
pulmonary rehabilitation
Treatment recommended for ALL patients in selected patient group
This is an important part of managing the mid-to-late phase of OP (after the initial few days of treatment, with the patient ambulatory and with improving symptoms and radiographic findings). It introduces an exercise programme for improving muscle conditioning, muscle oxygen efficiency, and sense of well-being. Patients also receive guidance for an ongoing exercise programme at home or at a commercial exercise facility.
lung transplantation
Additional treatment recommended for SOME patients in selected patient group
Very rarely, lung transplantation may be necessary for patients who do not respond to treatment or who have an unusual or hybrid form of OP.
Choose a patient group to see our recommendations
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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