{"title":"Hereditary and Acquired Angioedema for Rheumatologists in India: Are We Missing the Diagnosis?","authors":"S. Basu, Reva Tyagi, S. Machhua, A. Jindal","doi":"10.1177/09733698241255547","DOIUrl":"https://doi.org/10.1177/09733698241255547","url":null,"abstract":"Bradykinin-mediated angioedema can broadly be categorised into hereditary angioedema (HAE) and acquired angioedema (AAE). Both HAE and AAE are grossly under-recognised in the country largely because of lack of awareness. Type 1 and 2 HAE is caused by pathogenic variants in the SERPING1 gene that codes for C1-inhibitor protein. Deficiency of C1-inhibitor protein leads to recurrent swelling episodes involving hands, feet, eyes, lips, tongue and genitalia. These episodes are typically not associated with itching or urticaria. Involvement of the larynx leads to a potentially life-threatening episode of choking and, in the past, the mortality because of laryngeal edema used to be as high as 30%. AAE is usually associated with lymphoreticular malignancies; predominantly B cell lymphomas and systemic lupus erythematosus. Angioedema often precedes the diagnosis of primary illness. The clinical features of both AAE and HAE are similar. However, AAE should be suspected in patients with onset of disease in older age. Management of HAE is broadly categorised into three types: on-demand therapy and short-term and long-term prophylaxis (LTP). Patients with AAE also need management of the underlying disease with immunosuppressants. This review focuses on clinical manifestations, diagnostic evaluation, and clinical mimics of HAE and AAE from the perspective of a rheumatologist. The review also briefly discusses the management principles of HAE and AAE.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141346792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neutrophils-derived Parameters as Cost-effective Inflammatory, Disease Activity Marker in Systemic Lupus Erythematosus (SLE) and Role in Prediction of Lupus Nephritis","authors":"Shaila Farheen, Seema Sharma, Ram Nawal Rao, Raghavendra Linghaiah, A. Aggarwal","doi":"10.1177/09733698241231467","DOIUrl":"https://doi.org/10.1177/09733698241231467","url":null,"abstract":"Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with various clinical manifestations and approximately 50% of the SLE patients develop lupus nephritis (LN), which increases the risk of renal failure, cardiovascular diseases and overall survival. Evaluation of neutrophil-derived parameters (Neut-X, Neut-Y and Neut-Z) as an inflammatory, disease activity marker and as a predictor of nephritis in SLE patients. In this cross-sectional study, 3 ml K3EDTA blood was taken from 110 SLE patients presented in Department of Clinical Immunology to evaluate neutrophil-derived parameters in Sysmex XT2000i haematology analyser and their correlation with other inflammatory biomarkers like, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) as well as SLE disease activity index-2000 (SLEDAI-2K). 24-hours urine protein levels were also estimated as a marker of renal involvement. Records of renal biopsy were available in 73 SLE patients, who belonged to different morphologic classification of LN. After performing bivariate Spearman correlation analysis (SPSS software, version 26.0), Neut-X (measures cytoplasmic granularity by side scattering) and Neut-Z (vector sum of Neut-X and Neut-Y) showed a significant positive correlation ( r > 0.200, P < .05) with ESR, CRP and SLEDAI-2K while Neut-Y (measures nucleic acid content by sideward fluorescence) showed a significant negative correlation ( r > 0.200, P < .05) with ESR and CRP. Receiver-operating characteristic curve analysis was used to evaluate diagnostic value of 24-hour urine protein and predictive values of neutrophil-derived parameters for renal involvement in SLE patients. Among neutrophil-derived parameters, Neut-X was found to be the best predictor of renal dysfunction with highest AUC of 0.710 (95% CI: 0.604-0.816; cut-off: 1344.50; sensitivity: 84.7%; specificity: 50%) followed by Neut-Z with AUC of 0.707 (95% CI: 0.600-0.813; cut-off: 1403.67; sensitivity: 87.5%; specificity: 50%). Our study shows that neutrophil-derived parameters may be used as novel cost-effective, non-invasive biomarker of disease activity as well as for predicting renal involvement in SLE patients.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140983284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sri Andala, Hizir Sofyan, K. Hasballah, Marthoenis
{"title":"Investigating Factors Associated with Drug Adherence Among Individuals with Hyperuricemia: A Cross-sectional Study","authors":"Sri Andala, Hizir Sofyan, K. Hasballah, Marthoenis","doi":"10.1177/09733698241231347","DOIUrl":"https://doi.org/10.1177/09733698241231347","url":null,"abstract":"The prevalence of hyperuricemia is on the rise in specific populations, and there are difficulties in maintaining medication adherence. This study aims to investigate the factors linked to drug adherence in individuals with hyperuricemia. This was a cross-sectional study, which was conducted in 2023 by recruiting respondents with hyperuricemia. The demographic data collected included age, gender, occupation, body height and weight, education, marital status and ethnicity. Furthermore, drug adherence was determined based on the response to the Morisky Medication Adherence Scale-8. Acceptance and knowledge were measured using questionnaires given to the participants. Multivariate logistic regression analysis was then used to obtain the determinants of drug adherence. Among 524 respondents who participated in the study, 60.3% and 30.15% had moderate and high adherence to serum uric acid-lowering drugs, respectively. The results showed that older age had a strong association with lower adherence ( p < .01), while high disease acceptance significantly predicted higher levels ( p < .01). Knowledge about gout symptoms ( p = .003; OR: 0.28 [95% CI: 0.12-0.65]), causal factors ( p < .001; OR: 2.63 [95% CI: 1.48-4.65]) and uric acid-lowering alternatives ( p < .001; OR: 8.17 [95% CI: 4.11-16.25]) also had a positive correlation with higher levels. Knowledge and acceptance had a significant correlation with drug adherence among individuals with hyperuricemia in Aceh Province, Indonesia.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140691369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Importance of Prompt Diagnosis and Management of Digital Gangrene Associated with Rheumatic Diseases","authors":"K. Chandwar, D. Misra","doi":"10.1177/09733698241237819","DOIUrl":"https://doi.org/10.1177/09733698241237819","url":null,"abstract":"","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140259344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Estimation of Urinary C-terminal Telopeptide of Type II Collagen and its Correlation with Radiological Grading, Pain, and Function in Patients with Primary Osteoarthritis of the Knee","authors":"R. Nongmaithem, Ranjankumar Singh Lisham, Yengkhom Jotin, Kannamthodi Erumbanottil Shahin, Jerang Obit","doi":"10.1177/09733698241229944","DOIUrl":"https://doi.org/10.1177/09733698241229944","url":null,"abstract":"To estimate the level of urinary C-terminal telopeptide of type II collagen (uCTX-II) as a biomarker of cartilage turnover and to determine its correlation with radiological grading, pain, and functions of patients with knee osteoarthritis (OA). A descriptive cross-sectional study was conducted between September 2017 and August 2019 on 102 patients suffering from knee OA. Detailed demographic data were collected. uCTX-II level was measured for each patient. Radiological grading, pain and functional assessments were performed using Kellgren-Lawrence (K-L) grading, visual analogue scale (VAS), and Western Ontario and McMasters Universities Osteoarthritis Index (WOMAC). The mean age of 62.3 (6.4) years and most patients (n = 56, 54.9%) were 61–70 years old. Females constituted 80.4% (n = 82). Among the occupations, housewives were more affected (55.9%, n = 57). Out of 102 patients, 32 (31.4%) had VAS pain 4, 25 (24.5%) had 5 and 45 (44.1%) had 6; 9 (8.8%) had K-L score 1, 51 (50%) had 2, 41 (40.2%) had 3 and 1 (1%) had 4; 35(34.3%) had WOMAC score <30, 59 (57.8%) had 30–40, and 8 (7.8%) had >40; 55 ( 53.9%) had uCTX-II level <50 ng/mmole, 25 (24.5%) had between 50 and 200 ng/mmole and 22 (21.6%) had >200 ng/mmole. There was a positive correlation between uCTX-II and WOMAC ( P = .006) and VAS ( P = .042). The present study findings highlighted that higher levels of uCTX-II were associated with higher levels of pain and greater difficulty with daily activities (measured by WOMAC disability score). This suggests that uCTX-II could be a useful biomarker for monitoring cartilage turnover in patients with knee OA and its relationship with pain and functional abilities.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140397704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Dhanish, Subbiah Kanthanathan, S. Rajeswari, P. Aseer
{"title":"Translation and Validation of Tamil Version of Fatigue Severity Scale (FSS) in Individuals with Rheumatoid Arthritis","authors":"J. Dhanish, Subbiah Kanthanathan, S. Rajeswari, P. Aseer","doi":"10.1177/09733698241232849","DOIUrl":"https://doi.org/10.1177/09733698241232849","url":null,"abstract":"Fatigue has a significant negative impact on individuals with Rheumatoid Arthritis (RA). Fatigue Severity Scale (FSS) is the most widely used tool to assess fatigue severity which is a common problem in RA. Availability of FSS in local vernacular language could add more value and the study aims to construct the Tamil version of the FSS and validate it among patients with RA. The study was conducted in 2 phases. In phase I, the English version of FSS was translated into Tamil by following the Linguistic Guidelines by mapi eprovide. In Phase II – Validation of Tamil version of FSS was carried out in 50 patients of RA by establishing; Test-retest reliability, Internal consistency, Content validity; and Convergent validity in comparison with HAQ-DI and SF-12 The initial testing between Tamil FSS with English version revealed an intraclass correlation coefficient (ICC) (0.821) and α(0.902). The content validity was established by Cohen’ kappa agreement k which is >80% with a significance <0.004, which shows Good substantial agreement between raters. FSS showed strong positive correlation with HAQ-DI (>0.0005) and negative correlation with SF-12 (>0.0005). The scale demonstrated excellent internal consistency ( α = 0.972) and test-retest reliability (ICC = 0.93). The validated Tamil version demonstrated excellent content validity as well as good internal consistency and test-retest reliability, would be a useful instrument for clinical practice and research. It can be recommended for the assessment of Fatigue severity among Tamil-speaking population with RA.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140397883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Book review: Rohini Handa and Aman Sharma, Rheumatology Clinics: Clinical Scenarios","authors":"Anand N. Malaviya","doi":"10.1177/09733698241236128","DOIUrl":"https://doi.org/10.1177/09733698241236128","url":null,"abstract":"Rohini Handa and Aman Sharma, Rheumatology Clinics: Clinical Scenarios. Evangel Publishers, New Delhi, 2023, 473 pp., ₹3495 (Hardbound).","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140282604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rajni Sharma, Archan Sil, G. Anjani, Ruchi Saka, A. Jindal
{"title":"Psychological Assessment and Behavioural Interventions in a Young Girl with Antineutrophil Cytoplasmic Antibody-associated Vasculitis","authors":"Rajni Sharma, Archan Sil, G. Anjani, Ruchi Saka, A. Jindal","doi":"10.1177/09733698241229893","DOIUrl":"https://doi.org/10.1177/09733698241229893","url":null,"abstract":"","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140420984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prevention of Atherosclerotic Cardiovascular Disease in Rheumatoid Arthritis: Persistent Low-grade In-flammation May Be an Impediment Needing Attention","authors":"Anand N. Malaviya, Babita Bondge, Shiv Prasad","doi":"10.1177/09733698241231424","DOIUrl":"https://doi.org/10.1177/09733698241231424","url":null,"abstract":"To determine the status of ‘residual inflammatory risk’ (RIR) and ‘residual cholesterol risk’ (RCR) in patients with Rheumatoid Arthritis (RA) in remission with the aim of estimating their atheroscle-rotic cardiovascular disease (ASCVD) risk. The study included patients with RA in remission during their last two clinic visits on treatment with disease- mod-ifying anti-rheumatic drugs (DMARDs). Using the QRISK-3 calculator, participants were stratified into four risk categories for ASCVD: ‘Very high’, ‘High’, ‘Moderate’, and ‘Low’. Patients were prescribed lipid-lowering drugs targeting low-density lipoprotein cholesterol (LDL-C) levels to <50 mg/dl, <70 mg/dl, <100 mg/dl or <130 mg/dl, respectively. Those with a history of ASCVD before or during the follow-up period was excluded from the study. Multimorbidity were also recorded in all the patients. The study included 130 patients with RA in remission. The results showed that despite being in remission and taking treatment for lipid control, 81 (62%) and 91 (70%) patients still had ‘RIR’ and ‘RCR’, respectively. Hypertension, hypothyroidism, type-2 diabetes mellitus and obesity were the common multimorbidity. Persistent RIR in 62% of patients despite being in remission, could possibly be due to the current ‘liberal’ definition of RA permitting hs-CRP level up to <10 mg/l, which is five-fold higher than the recommended <2 mg/l for ASCVD prevention. Hence it may be necessary to revise the definition of ‘remission’ in RA factoring in the suggested lower level of hs-CRP. Additionally, rheumatologists might need to be more vigilant in lipid management with appropriate patient education regarding RCR.","PeriodicalId":54167,"journal":{"name":"Indian Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140432858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}