Reviewer's report 
 Idiopathic portal hypertension complicating systemic sclerosis: Case report Title: 
 1  4 March 2005 Version: Date: 
 Radha K Dhiman Reviewer: 
Reviewer's report: 
General 
The authors have reported an association between progressive systemic sclerosis and idiopathic 
portal hypertention, which could be a coincidental or a causal association. The manuscript is well 
written, however some points need consideration for inclusion in â €œDiscussionâ €. 
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Major Compulsory Revisions (that the author must respond to before a decision on publication can 
be reached) IPH is commonly seen in India and Japan. Authors should include references of major studies from Japan and India in patients with IPH (Dhiman RK, Chawla Y, Vasishta RK et al. Non-cirrhotic portal 
fibrosis (Idiopathic portal hypertension): experience with 151 patients and a review of the literature. 
J. Gastroenterol. Hepatol. 2001; 17: 6â €“16 and Okuda K, Kono K, Ohnishi K et al. Clinical study of 
eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 1984; 86: 600â €“10.) The largest study from India also observed female 
preponderance in Indian patients (Dhiman et al). 
Authors should also discuss whether the association between these two disorders is causal or 
merely a coincidental. In Japan, autoimmune disorders are frequently associated with IPH; in a 
questionnaire-based study about 12% of cases with IPH were found to be associated with one or 
more autoimmune disease and one quarter of them had hyperglobulinemia. The reported autoimmune diseases were systemic lupus erythematosus, progressive systemic sclerosis, chronic thyroiditis, mixed connective tissue disease, Sjogren syndrome, autoimmune hemolytic anemia, etc. 
(Saito K, Nakanuma Y, Takegoshi K et al. Non-specific immunological abnormalities and association 
of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire. 
Hepatogastroenterology 1993; 40: 163â €“6.) An immunological basis for IPH pathogenesis has also 
been suggested (Terada T, Nakanuma Y, Hoso M, Obata H. Expression of HLA-DR antigen on 
hepatic vascular endothelial cells in idiopathic portal hypertension. Clin. Exp. Immunol. 1991; 84: 
303â €“7). Therefore, a causal relationship between these two disorders is a possibility. 
------------------------------------------------------------------------------- 
Minor Essential Revisions (such as missing labels on figures, or the wrong use of a term, which the 
author can be trusted to correct) 
------------------------------------------------------------------------------- 
Discretionary Revisions (which the author can choose to ignore) 
 Accept after minor essential revisions What next?: 
 An article of importance in its field Level of interest: 
 Acceptable Quality of written English: 
 No Statistical review: 
Declaration of competing interests: 
'I declare that I have no competing interests' 
Reviewer's report 
 Idiopathic portal hypertension complicating systemic sclerosis: Case report Title: 
 1  4 March 2005 Version: Date: 
 Radha K Dhiman Reviewer: 
Reviewer's report: 
General 
The authors have reported an association between progressive systemic sclerosis and idiopathic 
portal hypertention, which could be a coincidental or a causal association. The manuscript is well 
written, however some points need consideration for inclusion in â €œDiscussionâ €. 
------------------------------------------------------------------------------- 
Major Compulsory Revisions (that the author must respond to before a decision on publication can 
be reached) IPH is commonly seen in India and Japan. Authors should include references of major studies from Japan and India in patients with IPH (Dhiman RK, Chawla Y, Vasishta RK et al. Non-cirrhotic portal 
fibrosis (Idiopathic portal hypertension): experience with 151 patients and a review of the literature. 
J. Gastroenterol. Hepatol. 2001; 17: 6â €“16 and Okuda K, Kono K, Ohnishi K et al. Clinical study of 
eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 1984; 86: 600â €“10.) The largest study from India also observed female 
preponderance in Indian patients (Dhiman et al). 
Authors should also discuss whether the association between these two disorders is causal or 
merely a coincidental. In Japan, autoimmune disorders are frequently associated with IPH; in a 
questionnaire-based study about 12% of cases with IPH were found to be associated with one or 
more autoimmune disease and one quarter of them had hyperglobulinemia. The reported autoimmune diseases were systemic lupus erythematosus, progressive systemic sclerosis, chronic thyroiditis, mixed connective tissue disease, Sjogren syndrome, autoimmune hemolytic anemia, etc. 
(Saito K, Nakanuma Y, Takegoshi K et al. Non-specific immunological abnormalities and association 
of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire. 
Hepatogastroenterology 1993; 40: 163â €“6.) An immunological basis for IPH pathogenesis has also 
been suggested (Terada T, Nakanuma Y, Hoso M, Obata H. Expression of HLA-DR antigen on 
hepatic vascular endothelial cells in idiopathic portal hypertension. Clin. Exp. Immunol. 1991; 84: 
303â €“7). Therefore, a causal relationship between these two disorders is a possibility. 
------------------------------------------------------------------------------- 
Minor Essential Revisions (such as missing labels on figures, or the wrong use of a term, which the 
author can be trusted to correct) 
------------------------------------------------------------------------------- 
Discretionary Revisions (which the author can choose to ignore) 
 Accept after minor essential revisions What next?: 
 An article of importance in its field Level of interest: 
 Acceptable Quality of written English: 
 No Statistical review: 
Declaration of competing interests: 
'I declare that I have no competing interests' 
Please review this paper.
user
Reviewer's report 
 Idiopathic portal hypertension complicating systemic sclerosis: Case report Title: 
 1  4 March 2005 Version: Date: 
 Radha K Dhiman Reviewer: 
Reviewer's report: 
General 
The authors have reported an association between progressive systemic sclerosis and idiopathic 
portal hypertention, which could be a coincidental or a causal association. The manuscript is well 
written, however some points need consideration for inclusion in â €œDiscussionâ €. 
------------------------------------------------------------------------------- 
Major Compulsory Revisions (that the author must respond to before a decision on publication can 
be reached) IPH is commonly seen in India and Japan. Authors should include references of major studies from Japan and India in patients with IPH (Dhiman RK, Chawla Y, Vasishta RK et al. Non-cirrhotic portal 
fibrosis (Idiopathic portal hypertension): experience with 151 patients and a review of the literature. 
J. Gastroenterol. Hepatol. 2001; 17: 6â €“16 and Okuda K, Kono K, Ohnishi K et al. Clinical study of 
eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 1984; 86: 600â €“10.) The largest study from India also observed female 
preponderance in Indian patients (Dhiman et al). 
Authors should also discuss whether the association between these two disorders is causal or 
merely a coincidental. In Japan, autoimmune disorders are frequently associated with IPH; in a 
questionnaire-based study about 12% of cases with IPH were found to be associated with one or 
more autoimmune disease and one quarter of them had hyperglobulinemia. The reported autoimmune diseases were systemic lupus erythematosus, progressive systemic sclerosis, chronic thyroiditis, mixed connective tissue disease, Sjogren syndrome, autoimmune hemolytic anemia, etc. 
(Saito K, Nakanuma Y, Takegoshi K et al. Non-specific immunological abnormalities and association 
of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire. 
Hepatogastroenterology 1993; 40: 163â €“6.) An immunological basis for IPH pathogenesis has also 
been suggested (Terada T, Nakanuma Y, Hoso M, Obata H. Expression of HLA-DR antigen on 
hepatic vascular endothelial cells in idiopathic portal hypertension. Clin. Exp. Immunol. 1991; 84: 
303â €“7). Therefore, a causal relationship between these two disorders is a possibility. 
------------------------------------------------------------------------------- 
Minor Essential Revisions (such as missing labels on figures, or the wrong use of a term, which the 
author can be trusted to correct) 
------------------------------------------------------------------------------- 
Discretionary Revisions (which the author can choose to ignore) 
 Accept after minor essential revisions What next?: 
 An article of importance in its field Level of interest: 
 Acceptable Quality of written English: 
 No Statistical review: 
Declaration of competing interests: 
'I declare that I have no competing interests' 
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