Otologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases
, Rosa Novoa 3, Ohad Hilly 4 and Sabri El-Saied 1,*
Patrick Dawes
Round 1
Reviewer 1 Report
This is a two case report and literature review of IgG4-related disease of the mastoid. It is not clear if you considered granulomatosis with polyangitis among your differential diagnosis of these cases.
The case reports highlight the difficulty of diagnosis of this condition.
Table 1 summarises the results of a literature review and indicates 12 reported cases of IgG4-related disease of the middle ear and mastoid.
I have found three other cases reported prior to 2021, two presenting with middle ear/mastoid lesions and one with petrous apicitis. In addition, there are two case reports published this year. For completeness your article should include this material as it influences the upto date report of case presentations and may influence your discussion.
This is one of the hazards of this type of article. However, your cases add to the knowledge base and updating your article should make it relevant and inclusive of cases reported. I also think there may be benefit in describing/discussing potential differential diagnoses and how investigations (serology and imaging) may help reach a diagnosis sooner rather than later.
Chowsilpa, et al. Temporal bone involvement of IgG4-related disease: a rare condition misleading to petrous apicitis causing lateral rectus palsy. BMJ Case Rep. 2019; 12(2): e228550.
Wick CC, Zachariah J, Manjila S, Brown WC, Malla P, Katirji B, Cohen M, Megerian CA.Am J Otolaryngol. 2016 Nov-Dec;37(6):567-571. doi: 10.1016/j.amjoto.2016.08.005. Epub 2016 Aug 18.PMID: 27609186 Review.
Wang J, Sun Z, Zhuo S, Wang K.
Sigmoid sinus occlusion infiltrated by inflammatory myofibroblastic tumor from mastoid. Head Neck. 2015 Jan;37(1):E4-7. doi: 10.1002/hed.23704. Epub 2014 Jul 11.PMID: 24753301
Polianskis et al. Immunoglobulin G4-Related Disease Presenting as Temporal Bone Lesion with Facial Nerve Palsy. ORL J Otorhinolaryngol Relat Spec. 2022 Jun 7;1-7. doi: 10.1159/000522612
Hofmeyer L et al. Case Report: Diagnosis of Petrous Apex IgG4-Related Disease by Middle Cranial Fossa Craniotomy and Temporal Bone Biopsy.
Front Neurol . 2022 Jun 9;13:874451. doi: 10.3389/fneur.2022.874451
Author Response
07/08/2022
Point-to-point response to reviewers
Dear reviewers,
On behalf of the co-authors in the presently revised manuscript, we thank you for your important comments. We do believe the revised manuscript is greatly improved due to your remarks. Listed are the changes made as per the ideas you volunteered in the latest communication:
Response to Reviewer 1 Comments
Point 1: I have found three other cases reported prior to 2021, two presenting with middle ear/mastoid lesions and one with petrous apicitis. In addition, there are two case reports published this year. For completeness your article should include this material as it influences the upto date report of case presentations and may influence your discussion. This is one of the hazards of this type of article. However, your cases add to the knowledge base and updating your article should make it relevant and inclusive of cases reported.
Response 1: Comment accepted. All cases mentioned by the reviewer were added to the review. Acordingally, changes in results and discussion sections were made.
Point 2: I also think there may be benefit in describing/discussing potential differential diagnoses and how investigations (serology and imaging) may help reach a diagnosis sooner rather than later.
Response 2: A paragraph about potential diefferntial diagnosis was writen and added to the the discussion section.
Reviewer 2 Report
We read with interest the paper entitled Otologic Manifestations of IgG4-Related Disease in the Mastoid: Literature Review and Report of Two Cases. The aim of this paper is to summarize findings of patients with IgG4-RD involving the mastoid. I would suggest a modification of the title as follows "Otologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases".
Strengths of the study: it is the first review that focus on the otological manifestations of IgG4 disease, focusing in particular on the mastoid area
No major concerns.
Some issues are herein suggested.
- IgG4/IgG ratio of both cases were below 20% (please comment this result in your discussion as you point out that "a comprehensive cut- off value when plasma cell ratio is over 40%"
- Adding the IgG4 serum level to the IgG/IgG4 ratio (made on the biopsy) and to the number of IgG cell per HFP could have been a valuable element in the case report (even though they are not mandatory for the diagnosis of IgG4-RD and up to 30% of patients have normal levels of serum IgG4)
Grammatical tips:
- line 13 of the Introduction: arena should be replaced by “area”
- page 7 line 4: a full stop should be added after sigmoidal sinus.
Article with related topic:
- Isolated Otologic Involvement of IgG4 Related Disease: A Case Report and Review of Literature (2022, USA)
- IgG4-related pachymeningitis and mastoiditis, associated with cerebral venous thrombosis: A case report (2021, Paris)
- IgG4-Related Disease of the Skull and Skull Base A Systematic Review and Report of Two Cases (2021- Washington University School of Medicine)
Author Response
07/08/2022
Point-to-point response to reviewers
Dear reviewer,
On behalf of the co-authors in the presently revised manuscript, we thank you for your important comments. We do believe the revised manuscript is greatly improved due to your remarks. Listed are the changes made as per the ideas you volunteered in the latest communication:
Response to Reviewer 2 Comments
Point 1: I would suggest a modification of the title as follows "Otologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases”
Response 1: Comment accepted. e have changed the title as suggested.
Point 2: IgG4/IgG ratio of both cases were below 20% (please comment this result in your discussion as you point out that "a comprehensive cut- off value when plasma cell ratio is over 40%"
Response 2: We accept the comment. The ratio in our reported cases was added in the relevant paragraph.
Point 3: Adding the IgG4 serum level to the IgG/IgG4 ratio (made on the biopsy) and to the number of IgG cell per HFP could have been a valuable element in the case report (even though they are not mandatory for the diagnosis of IgG4-RD and up to 30% of patients have normal levels of serum IgG4)
Response 3: We accept the comment. We addressed this point in the discussion.
Point 4: line 13 of the Introduction: arena should be replaced by “area”
Respone 4: The word was corecceted.
Point 5: page 7 line 4: a full stop should be added after sigmoidal sinus.
Response 5: The punctuation was corrected.
Round 2
Reviewer 1 Report
thank you for revising the article
I have the follwoing comments:
Page 8 - Para1 line 1 - there are 19 cases not 18 and this error is carried forward, therefore revision of this section and discuasion will be required to acheive consistency.
There were 8 cases of Otitis Media
page 10 Para 1. The abbreviation HPF is introduced in the text. Please ensure tis has been written in full here or earlier in text and abbreviation introduced.
Page 10 para 3 Figure 6c. The figures in text do not match those in the figure. Time to diagnsis >12m is not in agreement
Page 11 para 1. new text Retrospectively, it can... This sentence requires rewriting to make it meaningful.
With these revisions (and others I may have missed) the article should be ready for publication
Author Response
Point 1: Page 8 - Para1 line 1 - there are 19 cases not 18 and this error is carried forward, therefore revision of this section and discuasion will be required to acheive consistency.
Response 1: Comment acceepted. We re-writed and re-calculated relevant numbers, according exact number of cases in the text.
Point 2: There were 8 cases of Otitis Media
Response 2: We agree with the comment. Numbers were corrected
Point 3: page 10 Para 1. The abbreviation HPF is introduced in the text. Please ensure tis has been written in full here or earlier in text and abbreviation introduced.
Response 3: We accept the comment, and introduced its abbereviation earlier.
Point 4: Page 10 para 3 Figure 6c. The figures in text do not match those in the figure. Time to diagnsis >12m is not in agreement
Response 4: Comment accepted. We revised the number in the figure and in the text to match.
Point : Page 11 para 1. new text Retrospectively, it can... This sentence requires rewriting to make it meaningful.
Response 5: The sentence rewrittened from.
