Editorial, March 2017

Dear Readers,

The first Issue of the PAP Newsletter has been sent to to 64 medical liaisons and has received a very nice open rate. This success has motivated the Editorial Team to continue and expand this important initiative by involving PAP experts worldwide.

We will open the second issue of our newsletter with a Letter written by Prof Ishii from the Kyorin University, Tokyo, illustrating the state of the art on PAP in Japan, a country with a longstanding tradition of research and networking for this rare disease. Prof Ishii has published milestone papers in the history of PAP, especially about the secondary form, from epidemiology to radiological characterization and treatment [1-3].

In his letter, Ishii provides interesting data on epidemiology not only for autoimmune and secondary PAP, but also for the unclassifiable cases, which represent a challenging subgroup of patients from the therapeutic point of view.

At the last ERS congress in London (September 2016) there was an entire abstracts session with a broad spectrum of topics dedicated to PAP, especially to whole lung lavage (WLL), currently the gold standard treatment for PAP.  Several abstracts were dedicated to the assessment of its efficacy, including biomarkers.  Besides that, the preliminary results of the Italian combination trial (WLL plus inhalational GM-CSF/sagramostin) were presented during this session, addressing the important finding that that combination therapy seems to be more effective than WLL alone in controlling disease progression.

The last article of this issue has been dedicated to the ongoing IMPALA Trial with molgramostim, the first randomized, double-blind, placebo-controlled, parallel group, international clinical trial for autoimmune PAP.  By illustrating the study design, the inclusion/exclusion criteria and the main outcomes, we hope to wake the interest of physicians dealing with PAP patients and to increase the awareness of this important trial in the history of PAP. If it is true that we all are committed to get the approval of the first drug for autoimmune PAP as soon as possible, there is still the need to increase the referral of PAP patients to the centres which are performing the trial, in order to reach the recruitment completion.

We hope that this new issue will be appreciated by the readers. Moreover, we are interested in receiving and publishing case reports on particularly difficult PAP cases, and letters with updates about ongoing initiatives on PAP worldwide.

We thank you for your interest and dedication to PAP!

Francesco Bonella
on behalf of the Editorial Team


  1. Ishii H, Seymour JF, Tazawa R, Inoue Y, Uchida N, Nishida A, Kogure Y, Saraya T, Tomii K, Takada T, Itoh Y, Hojo M, Ichiwata T, Goto H, Nakata K. Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan. BMC Pulm Med 2014: 14: 37.
  2. Ishii H, Tazawa R, Kaneko C, Saraya T, Inoue Y, Hamano E, Kogure Y, Tomii K, Terada M, Takada T, Hojo M, Nishida A, Ichiwata T, Trapnell BC, Goto H, Nakata K. Clinical features of secondary pulmonary alveolar proteinosis: pre-mortem cases in Japan. Eur Respir J 2011: 37(2): 465-468.
  3. Ishii H, Trapnell BC, Tazawa R, Inoue Y, Akira M, Kogure Y, Tomii K, Takada T, Hojo M, Ichiwata T, Goto H, Nakata K. Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis. Chest 2009: 136(5): 1348-1355.
Editor in chief

Dr. med. Francesco Bonella
Senior Clinical Researcher
Interstitial and Rare Lung Disease Unit
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