From the Guidelines
The primary treatment for cryptogenic organizing pneumonia (COP) is oral corticosteroids, which have been used in reported case series to induce remission in non-remitting or progressive cases 1.
Treatment Approach
The use of oral corticosteroids is based on the observation that the prognosis of COP is good, particularly when consolidation is the primary pattern on CT, and many untreated cases experience spontaneous remission 1.
- The typical dose and duration of corticosteroid therapy are not well-established, but a common approach is to start with a dose of 0.5-1 mg/kg/day (usually 40-60 mg daily) for 1-3 months, followed by a slow taper over 6-12 months total.
- For patients who cannot tolerate or respond poorly to corticosteroids, alternative immunosuppressive agents may be considered, although there is limited evidence to support their use in COP.
- Macrolide antibiotics like erythromycin have been associated with improved outcomes in some respiratory conditions, but their role in COP is not well-established, and they may be considered as steroid-sparing agents due to their anti-inflammatory properties 1.
Monitoring and Follow-up
- Treatment duration should be individualized based on clinical and radiological response, with regular monitoring for side effects of corticosteroids, including hyperglycemia, hypertension, and osteoporosis.
- Prophylaxis against Pneumocystis jirovecii pneumonia with trimethoprim-sulfamethoxazole should be considered for patients on prolonged high-dose steroids.
- Organizing pneumonia responds well to treatment in most cases, but relapses can occur, particularly during steroid tapering, necessitating careful follow-up with pulmonary function tests and imaging.
From the Research
Treatment Options for Cryptogenic Organizing Pneumonia (COP)
- Corticosteroids are the standard therapy for COP, with most patients responding well to treatment 2, 3
- Adjunctive drugs such as cyclosporine and macrolide may be considered in refractory cases 2
- Clarithromycin has been shown to be an effective alternative treatment for COP, particularly in patients with a high risk of adverse events from corticosteroids 4
- Immunoglobulins may be considered in cases of steroid-resistant COP, especially in patients with immunoglobulin deficiency 5
- Rituximab therapy has been shown to be effective in steroid-resistant COP, allowing for reduction or discontinuation of steroid treatment 6
Treatment Response and Outcomes
- Resolution of symptoms is more frequent in patients with cryptogenic OP than those with secondary OP 3
- Relapse is infrequent in both cryptogenic and secondary OP 3
- Five-year survival is higher in patients with cryptogenic OP (73%) than in secondary OP (44%) 3
- Patients with asymptomatic focal OP have an excellent prognosis with no treatment required 3