Review for 10.1186/1471-230X-11-77 (2011)

Reviewer's report
Title:Unusual Manifestation of Erdheim-Chester Disease
Version: 1Date:21 April 2011
Reviewer: Julien Haroche
Reviewer's report:
Dear Editor,
Thank you very much for the opportunity to review the manuscript entitled
“Unusual manifestations of Erdheim-Chester disease”, by Pan et al.
In this manuscript, the authors report the case of a 69-year-old man with
abdominal symptoms in whom a laparotomy, along with other typical features,
lead to a diagnosis of ECD.
While the diagnosis of biopsy-proven ECD with abdominal involvement is not
questionable, as the patient has several features of ECD and a typical histology, i
think the discussion and the literature review should be completed, particularly
with some of the important data regarding this rare disease published recently.
Minor issues include the following points:
- In the abstract section, please add the PS100 may sometimes be positive
(#20%).
- In the case report section please add appropriate reference regarding MRI
findings just below figure 6 were reference # 1 is not correct: please refer to Dion
et al, Bone involvement in Erdheim-Chester disease: imaging findings including
periostitis and partial epiphyseal involvement. Radiology. 2006;238:632-9.
- It would also be interesting to have a dosage of IgG4 in this case. Was it done ?
- In the beginning of the discussion section I would specify the number of patients
worldwide with less than 400 known ECD cases referring the recent Blood paper
of 2011 (see Arnaud et al. CNS involvement and treatment with interferon-{alpha}
are independent prognostic factors in Erdheim-Chester disease: a multicenter
survival analysis of 53 patients. Blood. 2011 Mar 10;117(10):2778-82).
- It would improve the discussion in the first paragraph to mention and refer the
gastro-involvement of other histiocytoses, such as Langerhans Cell histiocytosis,
which is much well established than in ECD.
- In the fourth’s paragraph i would also refer to more recent data regarding
pulmonary involvement as this patient died of respiratory failure with Arnaud et
al. Arthritis Rheum. 2010 Nov;62(11):3504-12 as well as Brun AL.
Erdheim-Chester disease: CT findings of thoracic involvement. Eur Radiol. 2010
Nov;20(11):2579-87, for the description of the typical pulmonary involvement. It
would also be interesting for the iconography of this case to show a picture of the
pulmonary infiltration just after figure 1.
- Again, in the second page of the discussion section, the percentages of lung
involvement should also be implemented by more recent data referring for
incense to Arnaud et al. Arthritis Rheum. 2010 Nov;62(11):3504-12.
- The last paragraph section of the discussion section should also be enriched
and partly re-written with more “up to date” data regarding treatment with
interferon alpha with the papers of Braiteh et al. (Successful treatment of
Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with
interferon-alpha, Blood. 2005 Nov 1;106(9):2992-4), Haroche et al. (Variability in
the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of
involvement: results in eight patients. Arthritis Rheum. 2006;54:3330-6) and also
see Arnaud et al. CNS involvement and treatment with interferon-{alpha} are
independent prognostic factors in Erdheim-Chester disease: a multicenter
survival analysis of 53 patients. Blood. 2011 Mar 10;117(10):2778-82). The last
sentence of the discussion should therefore be slightly modified due to recent
advances in treating ECD.
-
Level of interest: An article of importance in its field
Quality of written English: Acceptable
Statistical review: No, the manuscript does not need to be seen by a
statistician.
Declaration of competing interests:
I declare that i have no competing interests