Article in PDF
How to Cite
Citation Manager
Readers' Comments (0)
Audio Visual Article Statistics
Link
to PUBMED
Print this Article
Send to a Friend
Advertisers
Access Statistics Resources
Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
Timely publication of journal: Publication of manuscripts and bringing out the issue in time is one of the positive aspects of JCDR and is possible with strong support team in terms of peer reviewers, proof reading, language check, computer operators, etc. This is one of the great reasons for authors to submit their work with JCDR. Another best part of JCDR is "Online first Publications" facilities available for the authors. This facility not only provides the prompt publications of the manuscripts but at the same time also early availability of the manuscripts for the readers.
Indexation and online availability: Indexation transforms the journal in some sense from its local ownership to the worldwide professional community and to the public.JCDR is indexed with Embase & EMbiology, Google Scholar, Index Copernicus, Chemical Abstracts Service, Journal seek Database, Indian Science Abstracts, to name few of them. Manuscriptspublished in JCDR are available on major search engines ie; google, yahoo, msn.
In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
It is well said that "happy beginning is half done" and it fits perfectly with JCDR. It has grown considerably and I feel it has already grown up from its infancy to adolescence, achieving the status of standard online e-journal form Indian continent since its inception in Feb 2007. This had been made possible due to the efforts and the hard work put in it. The way the JCDR is improving with every new volume, with good quality original manuscripts, makes it a quality journal for readers. I must thank and congratulate Dr Hemant Jain, Editor-in-Chief JCDR and his team for their sincere efforts, dedication, and determination for making JCDR a fast growing journal.
Every one of us: authors, reviewers, editors, and publisher are responsible for enhancing the stature of the journal. I wish for a great success for JCDR."
Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011
Dr. Shankar P.R.
"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."
Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011 Anuradha
Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.
Dr. Anuradha
E-mail: anuradha2nittur@gmail.com
On Jan 2020
Case Series
Full Version
Immunoglobulin G4-related Disease: A Series of Four Cases
Correspondence Address :
Dr. Chandni Thomas,
Junior Resident, Department of Pathology, Amala Institute of Medical Sciences, Thrissur-680001, Kerala, India.
E-mail: chandnithomaz@gmail.com
Immunoglobulin G4-related Disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that affects multiple organs, resulting in tumefactive lesions and/or organ dysfunction. This chronic, multiorgan inflammatory process is characterised by the infiltration of IgG4-positive plasma cells and has a variable clinical presentation depending on the organ involved. The present case series discusses four cases (two males and two females) of IgG4-RD involving different sites in patients presented to present Institution. The authors reviewed four cases of IgG4-RD, including clinical details, biochemical, radiological, and histopathological features. The case series includes IgG4-RD masquerading as meningioma, IgG4-related sclerosing cholangitis, IgG4-RD of the thyroid gland, and IgG4-RD of the lacrimal gland. Since IgG4-RD has non specific clinical features, histopathological analysis and immunohistochemistry play a pivotal role in diagnosis. Despite its diagnostic difficulties, earlier recognition is crucial to prevent significant morbidity and extensive fibrosis leading to organ failure.
Immunoglobulin G4-related sclerosing disease, Immunohistochemistry, Multiorgan
Immunoglobulin G4-related Disease (IgG4-RD) is a systemic immune-mediated condition affecting any organ. It encompasses a spectrum of diseases that were considered independent disorders for past decades, such as type 1 Autoimmune Pancreatitis (AIP), retroperitoneal fibrosis, Mikulicz disease, Riedel thyroiditis, and hypertrophic pachymeningitis. It is characterised by tissue inflammation and fibrotic outcomes (1). Based on organ involvement, four clinical phenotypes have been defined: i) Pancreaticobiliary disease; ii) Retroperitoneal fibrosis with or without aortitis; iii) Head and neck limited disease; iv) Mikulicz syndrome with systemic involvement [2-5]. Pancreatic, retroperitoneal, salivary, and lacrimal gland involvement represent the prototypical manifestations, but lesions in the head and neck are less specific, making the differential diagnosis challenging (6),(7). Dense infiltration of IgG4-positive plasma cells is seen in the involved tissues, with or without elevated plasma levels of IgG4 (8). IgG4-RD is a fibroinflammatory condition with a broad variety of clinical spectrum. Hereby, the authors present just four cases, each of which presented with varied clinical symptoms and underwent systematic work-up to reveal the exact diagnosis.
Case 1
A 78-year-old male patient, diabetic and asthmatic, well controlled on bronchodilators and oral hypoglycemic agents, presented with a history of recurring generalised tonic-clonic seizures and headache for the past month. He also reported a fall at his home one month prior, resulting in a wound over his right forehead. Physical examination did not reveal any neurological deficits. Radiological examination showed a space-occupying lesion in the right temporal lobe, measuring about 41.3×40×39 mm, suggestive of meningioma. A Computed Tomography (CT) scan of the brain revealed extra-axial extension of the lesion with underlying bone erosion (Table/Fig 1)a,b. Preoperative work-up showed an elevated Erythrocyte Sedimentation Rate (88 mm in 1 hr, normal: ≤30 mm). Based on radiological and laboratory investigations, the provisional diagnosis was meningioma. The patient underwent right fronto-temporoparietal craniotomy and excision of the mass, following which the specimen was received in the pathology laboratory. Microscopy showed tissue with dense fibrosis.
Some areas exhibited a storiform pattern. Blood vessels of varying calibre were noted, showing obliterated lumen with perivascular concentric fibrosis. Focally nodular lymphoid aggregates were seen. The entire tissue was infiltrated by eosinophils in sheets and, in some areas, by sheets of plasma cells and lymphocytes. Fibrosis was highlighted with Masson’s trichrome stain (Table/Fig 1)c-i. The patient responded well to subsequent treatment with prednisolone 1 mg/kg and steroid-sparing immunosuppressive therapy. At the time of writing this report, the patient was symptomatically better with no focal neurological deficits.
Case 2
A 59-year-old male patient, known to have chronic calcific pancreatitis and under treatment, presented with colicky abdominal pain for the past three days. An ultrasound of the abdomen showed hepatomegaly with ill-defined heterogeneous hypoechoic areas in the right lobe segment V-VIII and segment VII subcapsularly, suspicious of an evolving abscess. Prominent central biliary ducts were also found, suggestive of biliary obstruction. A Ultrasonography (USG)-guided liver biopsy was performed. Microscopy revealed liver tissue with architectural distortion. Hepatocytes showed mild nuclear atypia. Spotty necrosis and portal inflammation with severe interface hepatitis were noted. The inflammatory cells comprised plasma cells, neutrophils, and foam cells. Portal sclerosis was present with marked expansion of the fibrosed portal area (Table/Fig 2)a-d. These findings were suggestive of IgG4-related sclerosing cholangitis. He was treated with systemic steroids and showed symptomatic improvement after a one-month course.
Case 3
A 56-year-old female patient with diabetes, asthma, and dyslipidemia presented with an enlarged thyroid with a nodule in the left lobe for the past six months. Ultrasound scanning suggested a multinodular goiter {Thyroid Imaging Reporting and Data System (TIRADS-IV)}. She underwent total thyroidectomy with level IV lymph node dissection. Grossly, the thyroid revealed a grey-white solid-cystic lesion in the left lobe of the thyroid, towards the upper pole, measuring 3.5×2.7×1.7 cm (Table/Fig 3)a. Microscopic examination of both lobes of the thyroid showed extensive areas of fibrosis with marked infiltration of chronic inflammatory cells composed of sheets of plasma cells and lymphocytes (Table/Fig 3)b. Dense and broad collagenous fibrous bands were noted, separating the thyroid parenchyma into nodules. Atrophic follicles and folliculolysis (Table/Fig 3)c were noted, along with foci of granuloma composed of foreign body giant cells and epithelioid histiocytes around the colloid. Thick-walled blood vessels of varying calibers, some having obliterated lumens with perivascular fibrosis, were also seen. Lymph nodes showed reactive changes only. With these histological features, a diagnosis of Riedel thyroiditis/IgG4-RD was suggested, and IgG4-positive plasma cells were identified using the IgG4 immunohistochemical marker (Table/Fig 3)d.
Case 4
A 53-year-old female patient presented with painless swelling of the left eyelid, which she noticed a year ago and has gradually enlarged to its present size. On examination, the swelling was firm and non tender. She was not on any routine medications. She was evaluated at a peripheral hospital and clinically diagnosed with lymphoma, following which she was referred to hospital. Excision was done, and authors received a firm, nodular mass measuring 2×2×1 cm; the cut section of which was homogenous and grey-white. Microscopy showed markedly fibrosed lacrimal glands with extensive fibrosis and acinar atrophy. Fibrosis surrounded the ductular elements. Prominent plasma cell infiltration was noted, admixed with lymphocytes and eosinophils focally (Table/Fig 4)a-c. The plasma cells were positive for IgG4 immunohistochemical staining (Table/Fig 4)d. With these features, a possibility of IgG4-RD was suggested. She was treated with systemic steroids for a short while and was symptomatically better afterwards.
ImmunoglobulinG4-related Disease (IgG4-RD) was recognised as a distinct entity during the past decade. It is characterised by the infiltration of one or more target organs by IgG4-positive plasma cells and lymphocytes. Autoimmiune Pancreatitis (AIP), which was first described in 1995, was found to be associated with elevated levels of IgG4 in 2001 (9). Later, it was postulated by Kamisawa T et al., that IgG4-RD is a systemic condition affecting many extra-pancreatic organs such as bile ducts, gall bladder, retroperitoneum, kidneys, breasts, prostate, lungs, and skin (10).
IgG4 constitutes less than 5% of the total immunoglobulins in healthy individuals and is associated with both autoimmune and allergic diseases (11). The presence of elevated IgG4 in the serum is a weak diagnostic feature for IgG4-RD, as up to 50% of patients with IgG4-RD have normal levels, as in one of present cases (12). However, the cut-off level for IgG4 of 135 mg/dL has a positive predictive value for IgG4-RD (13).
Human Leukocyte Antigen (HLA) and non HLA genes seem to play important roles in the pathogenesis of IgG4-RD. Studies have reported higher frequencies of HLA serotypes DRB1 0405 and DQB1 0401 in AIP. Single nucleotide polymorphisms (including Cytotoxic T-Lymphocyte Antigen (CTLA4) and tumour necrosis factor-alpha promoter genes) in non HLA genes were also linked to AIP (8). The expression of Th2 cytokines (IL-4, IL-5, IL-13) and regulatory cytokines (IL10, TGF-beta) was found to be elevated in AIP and IgG4-related tubulointerstitial nephritis (14). Increased production of IL-4, IL-5, IL-13 contributes to peripheral eosinophilia and elevated IgE levels. Increased production of IgG4 is caused by IL-10 and can lead to fibrosis by increasing the expression of the fibrotic cytokine, Transforming Growth Factor-beta (TGF-β). Expanded CD4+ effector/memory T cells with a cytolytic phenotype were found in IgG4-RD patients (8).
The diagnosis of IgG4-RD is made with an understanding of clinical, imaging, serological, and pathological details (15). The original 2011 comprehensive diagnostic criteria for IgG4-RD or its revised 2020 version and the consensus statement on IgG4-RD pathology for diagnosis (Table/Fig 5) (17) are widely used in clinical practice nowadays (7),(16). Many problems have arisen with these criteria, including the difficulty in obtaining biopsy samples from some patients (AIP or retroperitoneal fibrosis) and the limited sensitivity and specificity of serum IgG4 concentrations. Such problems have been addressed by organ-specific criteria for IgG4-RD (17). Patients with a possible or probable diagnosis of IgG4-RD could be re-diagnosed by organ-specific criteria, and patients who fulfilled at least one of the organ-specific criteria can be diagnosed with definite IgG4-RD (18).
The assessment ideally starts with a complete history and physical examination. Laboratory investigations and appropriate radiology evaluation will follow, depending on the site involved. Plasma IgG4 level should be checked. Enumeration of circulating plasmablasts is beneficial, especially in cases with normal serum IgG4 levels. Though elevated levels are seen in various inflammatory conditions, significant elevation (>2000 cells/mL) was observed in IgG4-RD (19). Complement levels have shown correlation with the disease activity in cases of renal IgG4-RD (20).
Histopathology is the current “gold standard” for diagnosis. Combined with immunohistochemistry, it is a definitive cornerstone in evaluation. The present first case contributes to the few published cases in the literature of intracranial IgG4-related pseudo-tumours that masquerade as meningioma. Goulam-Houssein S et al., have described supratentorial meningioma-like lesions (9). Authors’ last case adds to the cases of orbital pathology of this disease spectrum and is the most frequent ophthalmic manifestation of the disease.
The IgG4-related disease is a multiorgan chronic inflammatory process with a widely varied clinical picture. Early recognition and therapy are important to prevent serious irreversible tissue damage. Hence, a multidisciplinary approach for case work-up with a high index of suspicion on morphological features is crucial to reveal the diagnosis.
DOI: 10.7860/JCDR/2024/67738.19190
Date of Submission: Sep 28, 2023
Date of Peer Review: Nov 02, 2023
Date of Acceptance: Jan 02, 2024
Date of Publishing: Mar 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? No
• For any images presented appropriate consent has been obtained from the subjects. NA
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Sep 29, 2023
• Manual Googling: Nov 09, 2024
• iThenticate Software: Dec 20, 2023 (16%)
ETYMOLOGY: Author Origin
EMENDATIONS: 7
- Emerging Sources Citation Index (Web of Science, thomsonreuters)
- Index Copernicus ICV 2017: 134.54
- Academic Search Complete Database
- Directory of Open Access Journals (DOAJ)
- Embase
- EBSCOhost
- Google Scholar
- HINARI Access to Research in Health Programme
- Indian Science Abstracts (ISA)
- Journal seek Database
- Popline (reproductive health literature)
- www.omnimedicalsearch.com