Background: Cryptogenic organising pneumonia (COP) is an idiopathic pneumonic
process featured by a high sensitivity to steroids, but also by a fairly high relapse rate. Accordingly,
treatment needs to be protracted more than expected, leading to occurrence of side effects and need
for therapeutic changes in many patients. There is an increasing evidence that long-term macrolides
can effectively control disease in such situations.
Objective: Aim of this work was to ascertain the overall number of published cases of macrolidetreated
COP, treatment regimens, effectiveness, and tolerability.
Methods: Articles published from 1981 to 2015 were systematically searched through PUBMED,
EMBASE, and other databases. All patients with proven diagnosis of COP and treated with longterm
macrolides were included. Any information was extracted from each case and analysed,
including disease presentation, previous treatments, dose and length of macrolide administration,
clinical and radiological response, side effects, and relapses.
Results: Twelve papers were found for a total of 35 patients. Macrolides were administered at low or
full dose and as monotherapy or in association with steroids. No significant difference was detected
between the therapeutic regimens (P>0.05). Overall, long-term macrolide therapy was effective in
86% of cases, albeit after a minimum period of 3-4 months treatment. No side effects or relapses
Conclusion: Certain macrolide antibiotics seem to be a promising therapeutic option in COP due to
their immunomodulatory properties. Long-term macrolides, even as monotherapy and at low doses,
have been shown to be effective in COP patients with mild-to-moderate disease and/or steroidinduced