Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

Users Online : 42385

AbstractMaterial and MethodsResultsDiscussionConclusionReferencesDOI and Others
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

Dr Mohan Z Mani

"Thank you very much for having published my article in record time.I would like to compliment you and your entire staff for your promptness, courtesy, and willingness to be customer friendly, which is quite unusual.I was given your reference by a colleague in pathology,and was able to directly phone your editorial office for clarifications.I would particularly like to thank the publication managers and the Assistant Editor who were following up my article. I would also like to thank you for adjusting the money I paid initially into payment for my modified article,and refunding the balance.
I wish all success to your journal and look forward to sending you any suitable similar article in future"



Dr Mohan Z Mani,
Professor & Head,
Department of Dermatolgy,
Believers Church Medical College,
Thiruvalla, Kerala
On Sep 2018




Prof. Somashekhar Nimbalkar

"Over the last few years, we have published our research regularly in Journal of Clinical and Diagnostic Research. Having published in more than 20 high impact journals over the last five years including several high impact ones and reviewing articles for even more journals across my fields of interest, we value our published work in JCDR for their high standards in publishing scientific articles. The ease of submission, the rapid reviews in under a month, the high quality of their reviewers and keen attention to the final process of proofs and publication, ensure that there are no mistakes in the final article. We have been asked clarifications on several occasions and have been happy to provide them and it exemplifies the commitment to quality of the team at JCDR."



Prof. Somashekhar Nimbalkar
Head, Department of Pediatrics, Pramukhswami Medical College, Karamsad
Chairman, Research Group, Charutar Arogya Mandal, Karamsad
National Joint Coordinator - Advanced IAP NNF NRP Program
Ex-Member, Governing Body, National Neonatology Forum, New Delhi
Ex-President - National Neonatology Forum Gujarat State Chapter
Department of Pediatrics, Pramukhswami Medical College, Karamsad, Anand, Gujarat.
On Sep 2018




Dr. Kalyani R

"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."



Dr Kalyani R
Professor and Head
Department of Pathology
Sri Devaraj Urs Medical College
Sri Devaraj Urs Academy of Higher Education and Research , Kolar, Karnataka
On Sep 2018




Dr. Saumya Navit

"As a peer-reviewed journal, the Journal of Clinical and Diagnostic Research provides an opportunity to researchers, scientists and budding professionals to explore the developments in the field of medicine and dentistry and their varied specialities, thus extending our view on biological diversities of living species in relation to medicine.
‘Knowledge is treasure of a wise man.’ The free access of this journal provides an immense scope of learning for the both the old and the young in field of medicine and dentistry as well. The multidisciplinary nature of the journal makes it a better platform to absorb all that is being researched and developed. The publication process is systematic and professional. Online submission, publication and peer reviewing makes it a user-friendly journal.
As an experienced dentist and an academician, I proudly recommend this journal to the dental fraternity as a good quality open access platform for rapid communication of their cutting-edge research progress and discovery.
I wish JCDR a great success and I hope that journal will soar higher with the passing time."



Dr Saumya Navit
Professor and Head
Department of Pediatric Dentistry
Saraswati Dental College
Lucknow
On Sep 2018




Dr. Arunava Biswas

"My sincere attachment with JCDR as an author as well as reviewer is a learning experience . Their systematic approach in publication of article in various categories is really praiseworthy.
Their prompt and timely response to review's query and the manner in which they have set the reviewing process helps in extracting the best possible scientific writings for publication.
It's a honour and pride to be a part of the JCDR team. My very best wishes to JCDR and hope it will sparkle up above the sky as a high indexed journal in near future."



Dr. Arunava Biswas
MD, DM (Clinical Pharmacology)
Assistant Professor
Department of Pharmacology
Calcutta National Medical College & Hospital , Kolkata




Dr. C.S. Ramesh Babu
" Journal of Clinical and Diagnostic Research (JCDR) is a multi-specialty medical and dental journal publishing high quality research articles in almost all branches of medicine. The quality of printing of figures and tables is excellent and comparable to any International journal. An added advantage is nominal publication charges and monthly issue of the journal and more chances of an article being accepted for publication. Moreover being a multi-specialty journal an article concerning a particular specialty has a wider reach of readers of other related specialties also. As an author and reviewer for several years I find this Journal most suitable and highly recommend this Journal."
Best regards,
C.S. Ramesh Babu,
Associate Professor of Anatomy,
Muzaffarnagar Medical College,
Muzaffarnagar.
On Aug 2018




Dr. Arundhathi. S
"Journal of Clinical and Diagnostic Research (JCDR) is a reputed peer reviewed journal and is constantly involved in publishing high quality research articles related to medicine. Its been a great pleasure to be associated with this esteemed journal as a reviewer and as an author for a couple of years. The editorial board consists of many dedicated and reputed experts as its members and they are doing an appreciable work in guiding budding researchers. JCDR is doing a commendable job in scientific research by promoting excellent quality research & review articles and case reports & series. The reviewers provide appropriate suggestions that improve the quality of articles. I strongly recommend my fraternity to encourage JCDR by contributing their valuable research work in this widely accepted, user friendly journal. I hope my collaboration with JCDR will continue for a long time".



Dr. Arundhathi. S
MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
On Aug 2018




Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
The journal has a monthly publication and the articles are published quite fast. In time compared to other journals. The on-line first publication is also a great advantage and facility to review one's own articles before going to print. The response to any query and permission if required, is quite fast; this is quite commendable. I have a very good experience about seeking quick permission for quoting a photograph (Fig.) from a JCDR article for my chapter authored in an E book. I never thought it would be so easy. No hassles.
Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".



Dr. Mamta Gupta
Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.


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

Important Notice

Original article / research
Year : 2022 | Month : March | Volume : 16 | Issue : 3 | Page : OC01 - OC04 Full Version

A Retrospective Study on the Profile and Treatment Response of Patients with Mononeuritis Multiplex and Connective Tissue Diseases


Published: March 1, 2022 | DOI: https://doi.org/10.7860/JCDR/2022/52805.16060
Mythili Seetharaman Varadhan

1. Assistant Professor, Department of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.

Correspondence Address :
Dr. Mythili Seetharaman Varadhan,
House no. 50, 2nd Main Road, SVS Nagar, Valasaravakkam,
Chennai-600087, Tamil Nadu, India.
E-mail: mythiliseethas@gmail.com

Abstract

Introduction: Though Mononeuritis Multiplex (MM) can be caused by many pathological conditions, vasculitis is the most important cause.

Aim: To study clinical features of MM in patients with connective tissue diseases, the time of presentation of MM from disease onset, its association with disease activity, and functional outcome after treatment.

Materials and Methods: A retrospective study was conducted at Outpatient Department (OPD) of Madras Medical College (Government Hospital), Chennai, Tamil Nadu, India, between April 2015 to April 2017. The study included medical records of 18 patients with connective tissue disease who had attended the OPD, with sensory and motor symptoms and who were also diagnosed with MM. Paired t-test was used to find associations. Disease activity levels were determined using various measures and modified Rankin Score (mRS) was used to assess the response to treatment. Data analysis was done using Statistical Package for Social Sciences (SPSS) version 28.0.

Results: Of the 18 study subjects 14 (77.8%) were 18-40 years of age, and 10 (55.6%) were females. The mean duration between the time of diagnosis of connective tissue disease and the development of MM was 18.17 months. Ulnar nerve was the most common nerve involved, 11 subjects (61.1%) had ulnar nerve involvement. Axonal neuropathy was present on nerve conduction studies in 17 (94.4%) of the study subjects and sensory symptoms on history were present in 100% of the study subjects. There was a statistically significant difference (p-value <0.001) between the mRS before treatment (3.89) and after treatment (2.78) for a duration of six months.

Conclusion: It was seen that the disease activity indices for connective tissue diseases were quite high at the time of development of MM, and starting treatment would help improve the functional outcome as is evident by the difference in mRS.

Keywords

Cryoglobulinemic vasculitis, Rheumatoid arthritis, Systemic lupus erythematosus

Autoimmune connective tissue disorders are a heterogenous group of diseases that affect connective tissue in various organs resulting from poorly controlled autoimmune responses, complement activation, interferon dysregulation, and associated inflammation (1). Originally, connective tissue diseases represented a group of disorders in which connective tissues, such as the joints, skin, muscle, and blood vessels, were the primary site of inflammation. Because infiltration of immune, inflammatory, and other cells may also cause inflammation and abnormal immune responses in neuron, nearly all connective tissue diseases and related disorders can be complicated by various neuropsychiatric syndromes (2).

Neurological involvement is associated with significant morbidity in patients with rheumatic diseases and may indicate heightened disease activity. The most prominent features of neurological involvement in rheumatic diseases include cerebral ischaemia, polyneuropathy, and psychiatric symptoms (3). The American College of Rheumatology (ACR) case definitions of neuropsychiatric Systemic Lupus Erythematosus (SLE) include seven types of Peripheral Nervous System (PNS) disease: (i) peripheral neuropathy; (ii) cranial neuropathy; (iii) mononeuropathy single or multiplex; (iv) plexopathy; (v) autonomic neuropathy; (vi) acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome); and (vii) myasthenia gravis (4). Mononeuritis Multiplex (MM) is caused by various pathological conditions, although the main cause is vasculitis (5). The MM is a syndrome of the Peripheral Nervous System (PNS) involving progressive multifocal peripheral nerve lesions. Its distribution is typically asymmetric, and the course of the disease varies with the underlying aetiology. The MM is associated with several medical conditions, including vasculitis, immune-mediated diseases, diabetes, infections, neoplasms, infiltrative diseases, and drugs (6). Modified Rankin Scale (mRS) has several major strengths: it covers the entire range of functional outcomes from no symptoms to death, its categories are intuitive and easily grasped by both clinicians and patients. The scale runs from 0-6, running from perfect health without symptoms to death (7).

Mononeuritis multiplex is a condition that affects the PNS. So, the symptoms are related to those of damaged peripheral nerves. It can cause, motor weakness severe pain and loss of sensation in at least two separate areas of the body (8). In vasculitis, MM occurs due to ischemic insult to vasa nervosum leading to wallerian degeneration and there is deposition of endoneurial immune complex. A study was taken up with the prime objectives of identifying the clinical features of MM, mean duration between diagnosis of connective tissue disease and development of MM, to identify the association between MM and disease activity levels and to study the response to treatment with the help of mRS.

Material and Methods

A retrospective study was done on all eligible medical records between April 2015 and April 2017, in the Rheumatology Department of Madras Medical College, (Government Hospital), Chennai, Tamil Nadu, India. Since the study was done on secondary data (medical records), it had no ethical considerations. The retrospective observational study was done on medical records of connective tissue disease patients who had confirmed MM and had details on mRS before treatment and six months after treatment. The total eligible case records were 18 which was the final sample size.

Inclusion criteria: All connective tissue disease patient’s records with MM and all data available were included in the study. In our institute, connective tissue disease patients with motor weakness and sensory symptoms involving contiguous nerve, nerve conduction study and sural nerve biopsy (optional) were performed to diagnose MM.

Exclusion criteria: Subjects with diabetes, Hansen’s disease, Human Immunodeficiency Virus (HIV), malignancy, Hepatitis B, Hepatitis C, Hepatitis A, alcoholics, and those with other form of neuropathies were excluded from the study as their pre-existing conditions would have interfered with the study findings.

Study Procedure

Nerve conduction reports were used to identify axonal neuropathy among the study participants. Sural nerve biopsy reports were used to confirm the presence of vasculitis. Different disease activity markers were used in the study, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus Collaborating Clinics (SLICC) scores were used for Systemic Lupus Erythematosus (SLE). The Birmingham Vasculitis Activity Score (BVAS) levels and Vasculitis Damage Index (VDI) levels were used to measure disease activity of Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis, Cryoglobulinemic vasculitis, Polyarteritis Nodosa (PAN) and Rheumatoid Arthritis. Sjogren’s Syndrome Disease Activity Index (SSDAI) was used as a measure of disease activity for Sjögren’s syndrome. The mRS was used to measure the patient’s disabilities due to neurological deficits and the initial score (out of 6) was compared with the mRS after six months of treatment with either combinations of steroids with cyclophosphamide or steroids with rituximab to measure the efficacy of treatment and functional improvement (9),(10),(11),(12).

Statistical Analysis

Data entry was done on excel and the data was imported and analysed using Statistical Package for Social Sciences (SPSS) version 28.0. Percentages and 95% confidence intervals were calculated for descriptive data and paired t-test was used to find the association between before and after levels of the mRS. The p-value <0.05 was considered as statistically significant.

Results

Among the study participants 2 (11.1%) were below the age of 18 years and majority of the study population (77.8%) were between the ages of 18 and 40 years. Males were more (55.6%) when compared to females (Table/Fig 1). Among the study participants, seven had SLE and four had PAN (Table/Fig 2). Sural nerve biopsy showed probable vasculitis in 8 (44.4%) of the study subjects and vasculitis in 3 (16.7%) of the study subjects and the rest had a normal biopsy report (Table/Fig 2). The mean duration between the time of diagnosis of connective tissue disease and the time of development was 18.17 months with a standard deviation of 14.29 months. Nerve conduction studies revealed that 17 (94.4%) of the study participants had axonal neuropathy. The most common nerve involved was the ulnar nerve (61.1%), and the least commonly involved nerve was the peroneal nerve (22.2%) (Table/Fig 3). The symptoms based on the initial records showed that among the subjects with SLE, it was seen that five out of seven subjects (71.4%) had positive anti-ds-DNA, low complement three levels, musculoskeletal manifestations, and haematological manifestations. All 7 (100%) of the subjects with SLE had mucocutaneous manifestations, whereas lupus nephritis was present in 5 (28.6%) of the patients with SLE, and serositis was present in 1 (14.3%) of the patients with SLE. It was seen that the commonest symptom was weakness which was present in 16 (88.9%) subjects followed by tingling which was present in 13 (72.2%) subjects (Table/Fig 4). The mean SLEDAI and SLICC scores can be seen in (Table/Fig 5). The mean mRS before treatment was 3.89 (out of a total score of 6) and the mean mRS after initiation of treatment with steroids and cyclophosphamide or rituximab was 2.78, the paired t-test value was 6.52 and the difference was statistically significant with p-value <0.001 (Table/Fig 6).

Discussion

The current study was done to study clinical features of MM in patients with connective tissue diseases, the time of presentation of MM from disease onset, its association with disease activity, and functional outcome after treatment. Because of paucity of literature from the Indian setup, comparison with Indian studies could not be done.

In the current study all 100% of the participants had mucocutaneous manifestations and high ESR was seen in 88.9% of the study population, whereas according to a study done in Barcelona Spain on SLE related vasculitis (13) and published in the year 2006, 89% had cutaneous manifestation and 60% had high ESR levels, the minor differences could be because the current study had included other forms of connective tissue diseases along with SLE. Anti-ds-DNA was positive in 95% and hypocomplementemia was present in 83% of the study population which was slightly higher than in the current study where 71.4% of the SLE patients had positive Anti-ds-DNA and 71.4% had low complement three levels and 57.1% had low complement four levels. This difference could be because of difference in population type and sample size.

A systematic review based on 53 articles with extractable data (14) done to predict damage and mortality among SLE patients using SLEDAI and SLICC found that higher mortality was seen with higher SLEDAI and SLICC scores in six studies and two studies had a statistically significant association between SLEDAI and neuropsychiatric complications. The current study revealed high disease activity in all patients with MM, the mean SLEDAI was 24.00 in the current study and in the systematic review mean scores (>15, 19.6, 23) were significantly associated with mortality by three different studies. A study done on the association between autoimmune rheumatological studies with peripheral neuropathies (15) published in 2021 concluded that 5-93% of patients with connective tissue diseases have PNS involvement, the current study showed axonal neuropathy in 94.4% of the subjects. The higher proportion could be because the current study was done on those with coexisting MM along with connective tissue diseases.

The other study also revealed that the most affected nerves are the peroneal and tibial followed by the Ulnar nerve, whereas according to the current study the most frequently involved nerve was the Ulnar nerve, and the least involved nerve was the peroneal nerve. Further research on higher sample size should be done to corroborate the findings of the current study. A study done on 18 churg strauss syndrome patients (16) where the mRS for functional outcomes were compared before and after treatment with steroids, cyclophosphamide and immunoglobulins showed a statistically significant reduction in the score after 12 months of treatment (p-value <0.04). The findings were consistent with that of the current study were the mean mRS reduced from 3.89 to 2.78 after six months of treatment and the p-value was <0.001 suggesting a significant improvement in functional outcomes with treatment. When vasculitis is pinpointed as cause of MM as in our study, there is high disease activity, patients functional improvement as assessed by mRS shows improvement in the quality of life.

Limitation(s)

The low sample size of 18 is the limitation in the study. Higher sample size could not be achieved because of the rarity of the combination of connective tissue diseases and MM, in the absence of other factors like diabetes.

Conclusion

This study showed that the mean duration between diagnosis of connective tissue diseases and development of MM was around 18 months. So, it will be worthwhile to screen connective tissue patients for MM around this time. The study also reported the significant improvement in functional outcomes with treatment, which emphasises the need for initiation of early treatment.

References

1.
Jog NR, James JA. Biomarkers in connective tissue diseases. J Allergy Clin Immunol. 2017;140(6):1473-83. [crossref] [PubMed]
2.
Kasama T, Maeoka A, Oguro N. Clinical features of neuropsychiatric syndromes in systemic lupus erythematosus and other connective tissue diseases. Clin Med Insights Arthritis Musculoskelet Disord. 2016;9:01-08. [crossref] [PubMed]
3.
Sofat N, Malik O, Higgens CS. Neurological involvement in patients with rheumatic disease. QJM - Mon J Assoc Physicians. 2006;99(2):69-79. [crossref] [PubMed]
4.
Hanly JG, Li Q, Su L, Urowitz MB, Gordon C, Bae SC, et al. Peripheral nervous system disease in systemic lupus erythematosus: Results from an international inception cohort study. Arthritis Rheumatol. 2020;72(1):67-77. [crossref] [PubMed]
5.
Tanemoto M, Hisahara S, Hirose B, Ikeda K, Matsushita T, Suzuki S, et al. Severe mononeuritis multiplex due to rheumatoid vasculitis in rheumatoid arthritis in sustained clinical remission for decades. Intern Med. 2020;59(5):705-10. [crossref] [PubMed]
6.
Park M, Lee Y, Choi YC. Henoch-schönlein purpura presenting as mononeuritis multiplex. J Clin Neurol. 2018;14(1):112-14. [crossref] [PubMed]
7.
Broderick JP, Adeoye O, Elm J. The evolution of the Modified Rankin Scale and its use in future stroke trials. Stroke. 2017;48(7):2007-12. [crossref] [PubMed]
8.
Healthline article [Internet]. Avaialble from: https://www.healthline.com/health/mononeuritis.
9.
Mikdashi J, Naved O. Measuring disease activity in adults with systemic lupus erythematosus: The challenges of administrative burden and responsiveness to patient concerns in clinical research. Arthritis Research & Therapy. 2015;17:183-93. [crossref] [PubMed]
10.
Gladman D, Glinzer E, Goldsmith C, Fortin P, Liang M, Sanchez-Guerrero J, et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum. 1996;39(3):363-69. [crossref] [PubMed]
11.
Kermani TA, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, Koening CL, et al. The Birmingham Vasculitis Activity Score as a Measure of Disease Activity in Patients with Giant Cell Arteritis. J Rheumatol. 2016;43(6):1078-84. [crossref] [PubMed]
12.
Suppiah R, Flossmann O, Mukhtyar C, Alberici F, Baslund B, Brown D, et al. Measurement of damage in systemic vasculitis: A comparison of the Vasculitis Damage Index with the Combined Damage Assessment Index. Annals of the Rheumatic Diseases. 2011;70(1):80-85. [crossref] [PubMed]
13.
Ramos-Casals M, Nardi N, Lagrutta M, Brito-Zerón P, Bové A, Delgado G, et al. Vasculitis in systemic lupus erythematosus: Prevalence and clinical characteristics in 670 patients. Medicine (Baltimore). 2006;85(2):95-104. [crossref] [PubMed]
14.
Keeling SO, Vandermeer B, Medina J, Chatterley T, Nevskaya T, Pope J, et al. Measuring disease activity and damage with validated metrics: A systematic review on mortality and damage in systemic lupus erythematosus. J Rheumatol. 2018;45(10):1448-61. [crossref] [PubMed]
15.
Tulba D, Popescu BO, Manole E, Baicu? C. Immune axonal neuropathies associated with systemic autoimmune rheumatic diseases. Front Pharmacol. 2021;12:01-16. [crossref] [PubMed]
16.
Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G. Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome. Ann Rheum Dis. 2004;63(12):1649-54. [crossref] [PubMed]

DOI and Others

DOI: 10.7860/JCDR/2022/52805.16060

Date of Submission: Oct 10, 2021
Date of Peer Review: Nov 15, 2021
Date of Acceptance: Dec 11, 2021
Date of Publishing: Mar 01, 2022

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was Ethics Committee Approval obtained for this study? NA
• Was informed consent obtained from the subjects involved in the study? NA
• For any images presented appropriate consent has been obtained from the subjects. NA

PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Oct 12, 2021
• Manual Googling: Nov 30, 2021
• iThenticate Software: Dec 23, 2021 (12%)

ETYMOLOGY: Author Origin

JCDR is now Monthly and more widely Indexed .
  • 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
  • Google
  • Popline (reproductive health literature)
  • www.omnimedicalsearch.com