scholarly journals Favourable Functional Outcomes In Idiopathic Inflammatory Myositis- A Single Centre Experience Over 15 Years

2021 ◽  
Author(s):  
Ramya Janardana ◽  
Sangeetha K. N ◽  
Vasudha Bhat ◽  
Divya Balakrishnan ◽  
John Michael Raj ◽  
...  
Keyword(s):  
Clinical Response ◽  
Functional Outcomes ◽  
Damage Index ◽  
Health Assessment ◽  
Complete Clinical Response ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Longitudinal Cohort ◽  
Damage Accrual

Abstract Background: To describe the long term clinical outcome and prognostic factors associated with outcome in a longitudinal cohort of idiopathic inflammatory myositis (IIM). Methods: In this retrospective cohort study, IIM patients were classified as per Bohan and Peter criteria. In those with ≥ 24 months of follow-up; treatment response, functional outcomes [health assessment questionnaire-disability index(HAQ-DI) and modified Rankin score(MRS)], Myositis damage index(MDI) at last follow-up was recorded. Results: The cohort consists of 175 patients, with a mean age of 40.9(+12.6) years, M:F 1:3.3; the IIM subsets were dermatomyositis(DM) 78(44.6%), overlap myositis(OM) 45(25.7%), antisynthetase syndrome(ASS) 11(6.3%), polymyositis(PM) 25(14.3%) and juvenile DM/OM in 15(8.6%) patients. Mortality rate was 13.4% and disease related deaths was 9.1%. Ninety-four patients have followed up for >24 months, the median (IQR) of 65(35,100.7) months. At last follow-up, 13.8% were in treatment free remission, 73.4%, 11.7% had complete clinical response and partial clinical response with treatment respectively. HAQ-DI and MRS were favourable in > 90% of patients. At last follow-up, one-third were off-steroids. Discontinuation of steroids was associated with HAQ-DI of 0 and lower MRS. Complete clinical response on/off medication at last follow-up was associated with HAQ-DI of 0[OR10.9; 95%CI(3.3,160)], better MRS[OR 3.2; 95%CI(1.4,7.3)] and lesser MDI[OR 1.7; 95% CI(1.1,2.7)] at the last follow-up as compared to partial response. Baseline parameters and IIM subsets did not significantly influence outcome.Conclusion: Our longitudinal cohort of IIM had a good outcome in all major myositis subsets. Partial clinical response on treatment is associated with worse functional outcomes and damage accrual.

scholarly journals Management of the Complete Clinical Response

2017 ◽  
Vol 30 (05) ◽  
pp. 387-394 ◽  
Author(s):  
Guilherme São Julião ◽  
Bruna Vailati ◽  
Ivana Castro ◽  
Debora Raffaele ◽  
Angelita Habr-Gama
Keyword(s):  
Rectal Cancer ◽  
Clinical Response ◽  
Radical Surgery ◽  
Local Relapse ◽  
Neoadjuvant Chemoradiation ◽  
Complete Clinical Response ◽  
Watch And Wait ◽  
Long Term Outcomes

AbstractOrgan preservation is considered in the management of selected patients with rectal cancer. Complete clinical response observed after neoadjuvant chemoradiation for rectal cancer is one of these cases. Patients who present complete clinical response are candidates to the watch-and-wait approach, when radical surgery is not immediately performed and is offered only to patients in the event of a local relapse. These patients are included in a strict follow-up, and up of 70% of them will never be operated during the follow-up. This strategy is associated with similar oncological outcomes as patients operated on, and the advantage of avoiding the morbidity associated to the radical operation. In this article we will discuss in detail the best candidates for this approach, the protocol itself, and the long-term outcomes.

scholarly journals A study on demographic pattern of idiopathic inflammatory myopathies in a tertiary care hospital

2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Lactic Dehydrogenase ◽  
Idiopathic Inflammatory Myopathies ◽  
Care Hospital ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes ◽  
Cross Sectional ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.

scholarly journals Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis

2020 ◽  
Author(s):  
Tamara Vojinovic ◽  
Ilaria Cavazzana ◽  
Paolo Ceruti ◽  
Micaela Fredi ◽  
Denise Modina ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Lung Function Test ◽  
High Resolution Computed Tomography ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511

scholarly journals Long-term safety and limited organ damage in patients with systemic lupus erythematosus treated with belimumab: a Phase III study extension

Rheumatology ◽  
2019 ◽  
Vol 59 (2) ◽  
pp. 281-291 ◽  
Author(s):  
Ronald F van Vollenhoven ◽  
Sandra V Navarra ◽  
Roger A Levy ◽  
Mathew Thomas ◽  
Amy Heath ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Damage Index ◽  
Organ Damage ◽  
Index Score ◽  
Phase Iii ◽  
Extension Study ◽  
Laboratory Parameters ◽  
Damage Accrual

AbstractObjectiveThis extension study of the Phase III, randomized, placebo-controlled Belimumab International SLE Study (BLISS)-52 and BLISS-76 studies allowed non-US patients with SLE to continue belimumab treatment, in order to evaluate its long-term safety and tolerability including organ damage accrual.MethodsIn this multicentre, long-term extension study (GlaxoSmithKline Study BEL112234) patients received i.v. belimumab every 4 weeks plus standard therapy. Adverse events (AEs) were assessed monthly and safety-associated laboratory parameters were assessed at regular intervals. Organ damage (SLICC/ACR Damage Index) was assessed every 48 weeks. The study continued until belimumab was commercially available, with a subsequent 8-week follow-up period.ResultsA total of 738 patients entered the extension study and 735/738 (99.6%) received one or more doses of belimumab. Annual incidence of AEs, including serious and severe AEs, remained stable or declined over time. Sixty-nine (9.4%) patients experienced an AE resulting in discontinuation of belimumab or withdrawal from the study. Eleven deaths occurred (and two during post-treatment follow-up), including one (cardiogenic shock) considered possibly related to belimumab. Laboratory parameters generally remained stable. The mean (s.d.) SLICC/ACR Damage Index score was 0.6 (1.02) at baseline (prior to the first dose of belimumab) and remained stable. At study year 8, 57/65 (87.7%) patients had no change in SLICC/ACR Damage Index score from baseline, indicating low organ damage accrual.ConclusionBelimumab displayed a stable safety profile with no new safety signals. There was minimal organ damage progression over 8 years.Trial registrationClinicalTrials.gov, https://clinicaltrials.gov, NCT00424476 (BLISS-52), NCT00410384 (BLISS-76), NCT00732940 (BEL112232), NCT00712933 (BEL112234).

scholarly journals AB0596 PREDICTORS, LONG TERM CLINICAL AND TREATMENT OUTCOMES IN SOUTH ASIAN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOSITIS: A SINGLE CENTER STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1594.2-1595
Author(s):  
A. Nair ◽  
R. Goel ◽  
P. Chebbi ◽  
A. Mathew ◽  
A. Ganapati ◽  
...  
Keyword(s):  
Pericardial Effusion ◽  
Median Duration ◽  
Muscle Power ◽  
Disease Free Survival ◽  
Muscle Enzymes ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Disease Free

Background:Idiopathic inflammatory myositis (IIM) are a heterogeneous group of immune-mediated disorders with varied presentations and multiple organ involvement. Data on long term outcome among South Asian patients with IIM is sparse.Objectives:To study the long term clinical outcome, treatment responses and factors predicting outcome among adult patients with IIMMethods:Patients diagnosed as ‘Idiopathic Inflammatory Myositis’ under the department of Clinical Immunology and Rheumatology at CMC, Vellore, India were screened retrospectively. Patients aged 18 years and above, satisfying Bohan and Peter criteria, having follow up of one year or more with atleast two outpatient or inpatient visits between January 2010 and April 2019 were included in this study. Those patients with connective tissue disease associated myositis were not included. Details on muscle weakness, extramuscular involvement, muscle enzymes and treatment administered were recorded at baseline, 3, 6, 12, 18, 24 months and yearly thereafter. After assessing their cumulative response, categorization of patients into complete and partial responders was done. Complete responders were defined as patients with persistent muscle power of more than 4/5 and/or MMT 8 more than 76/80, complete resolution of skin, articular and lung involvement (if any) as well as muscle enzymes less than twice the upper limit of normal without any documented flares during the entire follow up period. Patients not satisfying the said criterias were grouped as Partial responders. Disease free survival duration was also analyzed.Results:Out of 310 patients of IIM identified, 187 (60.3%) patients satisfied the inclusion criteria. Women were 2.2 times more than men and mean age at symptom onset was 35.7±12.6 years. Dermatomyositis was the predominant myositis subtype seen. All patients were put on steroids with the mean dose being 45.9 ± 18.6 mg/day. At baseline, the key immunosuppressants used were methotrexate in 44.9% and mycophenolate in 37.6% patients. The median follow up duration was 48 (25-80) months. An associated malignancy was diagnosed in 3.2% after a median duration of 24.5 months. Five patients expired after a median duration of 80 months from diagnosis. Normal muscle power was attained in 76.1% patients and 88.6% were vocational by the last follow up visit. Steroids were discontinued in 56.7% patients after a median duration of 24 months (p=0.0002). Discontinuation of the immunosuppressant was feasible in 10.2% patients after a median duration of 44 months. Assessment of the cumulative responses revealed a relapsing and remitting course in 45.9%. Outcome predictors in univariate analysis were Jo-1 status, presence of arthritis, interstitial lung disease and pericardial effusion at baseline. On multivariate analysis, absence of pericardial effusion (p=0.011) and interstitial lung disease (p=0.067) at baseline were found to be predictors of complete response. Disease free survival probability estimated at 5 years and 10 years was 91.6% and 72.4% respectively. Estimating the probability gender wise, males achieved disease free status earlier than females.Conclusion:A favorable clinical and functional outcome was seen in a significant proportion of these patients with IIM on long term follow up. Pericardial effusion and ILD were identified as predictors of poor clinical outcome.References:[1]Taborda AL, Azevedo P, Isenberg DA. Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study. Clin Exp Rheumatol. 2014 Apr; 32(2):188–93.Acknowledgments:NilDisclosure of Interests:None declared

Metabolic syndrome predicts new damage in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort

Lupus ◽  
2022 ◽  
pp. 096120332110614
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reatégui-Sokolova ◽  
Rocío V Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Cox Regression ◽  
Damage Index ◽  
Immunosuppressive Drugs ◽  
Age At Diagnosis ◽  
Survival Models ◽  
Damage Accrual ◽  
Comorbidity Index

Objectives This study aims to determine whether the MetS predicts damage accrual in SLE patients. Methods This longitudinal study was conducted in a cohort of consecutive SLE patients seen since 2012 at one single Peruvian institution. Patients had a baseline visit and then follow-up visits every 6 months. Patients with ≥ 2 visits were included. Evaluations included interview, medical records review, physical examination, and laboratory tests. Damage accrual was ascertained with the SLICC/ACR damage index (SDI) and disease activity with the SLEDAI-2K. Univariable and multivariable Cox-regression survival models were carried out to determine the risk of developing new damage. The multivariable model was adjusted for age at diagnosis; disease duration; socioeconomic status; SLEDAI; baseline SDI; the Charlson Comorbidity Index; daily dose; and time of exposure of prednisone (PDN), antimalarials, and immunosuppressive drugs. Results Two hundred and forty-nine patients were evaluated; 232 of them were women (93.2%). Their mean (SD) age at diagnosis was 35.8 (13.1) years; nearly all patients were Mestizo. Disease duration was 7.4 (6.6) years. The SLEDAI-2K was 5.2 (4.3) and the SDI, 0.9 (1.3). One hundred and eight patients (43.4%) had MetS at baseline. During follow-up, 116 (46.6%) patients accrued at least one new point in the SDI damage index. In multivariable analyses, the presence of MetS was a predictor of the development of new damage (HR: 1.54 (1.05–2.26); p < 0.029). Conclusions The presence of MetS predicts the development of new damage in SLE patients, despite other well-known risk factors for such occurrence.

Damage index for antiphospholipid syndrome during long term follow-up: Correlation between organ damage accrual and quality of life

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 96-102
Author(s):  
Gabriela Medina ◽  
Erik Antonio Cimé Aké ◽  
Olga Vera-Lastra ◽  
Miguel Ángel Saavedra ◽  
María del Pilar Cruz-Domínguez ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Antiphospholipid Syndrome ◽  
Damage Index ◽  
Organ Damage ◽  
Second Phase ◽  
Primary Antiphospholipid Syndrome ◽  
Sf 36 ◽  
Damage Accrual

Background Consequences of organ damage in primary antiphospholipid syndrome (PAPS) are diverse, our aim was to determine organ damage over time and the correlation of organ damage accrual with health-related quality of life (HRQoL) in PAPS. Methods First phase: retrospective cohort applying Damage Index for Antiphospholipid Syndrome (DIAPS) at 1, 5, 10, 20 years, or longer since diagnosis. Second phase: cross-sectional study, assessing HRQoL by the Medical Outcomes Study Short Form 36 (SF-36), and organ damage accrual. Descriptive statistics and Spearman correlation coefficient were used. Results Sixty-seven patients were included, mean follow-up:15 years. Deep vein thrombosis prevailed (71.6%), pulmonary embolism (35.8%) and stroke (32.8%). Organ damage was found in 98.5%, with a cumulative DIAPS value of 3, with greater involvement in the neuropsychiatric and peripheral vascular domains. Regarding HRQoL, deterioration in the physical component summary (PCS) was found in 89.6%. Organ damage accrual correlated inversely and significantly with all the SF-36 domains, mainly with the total score and PCS. Body pain and PCS correlated the most (rho = −0.503, rho = −0.475). Conclusions Organ damage accrual impaired HRQoL in PAPS. Secondary thromboprophylxis through adequate systemic management and control of cardiovascular risk factors are necessary to prevent further impairment.

Functional outcomes after TEM in patients with complete clinical response after neoadjuvant chemoradiotherapy

2016 ◽  
Vol 31 (7) ◽  
pp. 2997-3003 ◽  
Author(s):  
Roberto Ghiselli ◽  
Monica Ortenzi ◽  
Luca Cardinali ◽  
Edlira Skrami ◽  
Rosaria Gesuita ◽  
...  
Keyword(s):  
Clinical Response ◽  
Functional Outcomes ◽  
Neoadjuvant Chemoradiotherapy ◽  
Complete Clinical Response

scholarly journals Outcomes following mycophenolate mofetil versus cyclophosphamide induction treatment for proliferative juvenile-onset lupus nephritis

Lupus ◽  
2019 ◽  
Vol 28 (5) ◽  
pp. 613-620 ◽  
Author(s):  
EMD Smith ◽  
E Al-Abadi ◽  
K Armon ◽  
K Bailey ◽  
C Ciurtin ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Induction Treatment ◽  
Class Iii ◽  
Juvenile Onset ◽  
Dsdna Antibody ◽  
Damage Accrual

Background Juvenile-onset systemic lupus erythematosus (JSLE) is more severe than adult-onset disease, including more lupus nephritis (LN). Despite differences in phenotype/pathogenesis, treatment is based upon adult trials. This study aimed to compare treatment response, damage accrual, time to inactive LN and subsequent flare, in JSLE LN patients treated with mycophenolate mofetil (MMF) versus intravenous cyclophosphamide (IVCYC). Methods UK JSLE Cohort Study participants, ≤16 years at diagnosis, with ≥4 American College of Rheumatology criteria for SLE, with class III or IV LN, were eligible. Mann–Whitney U tests, Fisher's exact test and Chi-squared tests were utilized for statistical analysis. Results Of the patients, 34/51 (67%) received MMF, and 17/51 (33%) received IVCYC. No significant differences were identified at 4–8 and 10–14 months post-renal biopsy and last follow-up, in terms of renal British Isles Lupus Assessment Grade scores, urine albumin/creatinine ratio, serum creatinine, ESR, anti-dsDNA antibody, C3 levels and patient/physician global scores. Standardized Damage Index scores did not differ between groups at 13 months or at last follow-up. Inactive LN was attained 262 (141–390) days after MMF treatment, and 151 (117–305) days following IVCYC ( p = 0.17). Time to renal flare was 451 (157–1266) days for MMF, and 343 (198–635) days for IVCYC ( p = 0.47). Conclusion This is the largest study to date investigating induction treatments for proliferative LN in children, demonstrating comparability of MMF and IVCYC.

Sign in / Sign up

Export Citation Format

Share Document