Nail Fold Capillaroscopy in a Cohort of Egyptian Dermatomyositis/Polymyositis Patients

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Howaida Elsayed Mansour ◽  
Shafica Ibrahim Ibrahim ◽  
Safaa Abdelsalam Aly Hussein ◽  
Gamer Abdelrahman Azrag
Keyword(s):  
Disease Activity ◽  
Rating Scale ◽  
Clinical Laboratory ◽  
Muscle Disease ◽  
Assessment Tools ◽  
Manual Muscle Testing ◽  
Skin Score ◽  
Muscle Testing ◽  
Activity Assessment ◽  
Disease Activity Assessment

ABASTRACT Background Nailfold capillaroscopy (NC) has been demonstrated to be an important tool in the diagnosis and treatment of patients with juvenile dermatomyositis (JDM) and systemic sclerosis. However, NC has been scarcely studied in adult patients with DM. Objectives Is to visualize capillary changes in DM/PM patients, trying to find a specific capillary pattern and its correlation if any- with disease activity in those patients. Methodology This is a cross sectional observational study included 20 patients (DM.Diagnosed on base of clinical, laboratory and histopathological findings. We excluded patients with hypertension or diabetes mellitus. The NFC performed using videocapillaroscope. A semiquantitative rating scale was used to score capillaroscopy changes. Myositis disease activity assessment tools were used to assess disease activity [Manual Muscle Testing (MMT), Myositis Disease Activity Assessment Visual Analogue Scales (MYOACT), Health Assessment Questionnaire (HAQ), physician’s VAS, patient’s VAS, skin scoring, serum muscle enzymes levels] Result The median age of our patients was 36 (S.D.12) Mean disease duration was 24months (S.D.19).The results presented mean value 3,8 (S.D.3,3) for muscle disease activity score,2(S.D.3) for skin score and 3,92 (S.D.3,09) for global disease activity .Enlarged capillaries was found in12 patients (100%), avascular areas in 10 patients (83.3%), capillaries hemorrhage in 8 patients (66,7%), and branched capillaries in 6 patients (50%).Sixteen patient (80%) had scleroderma like pattern. We found that disease activity positively correlate with branched capillaries(p = 0,04), muscle disease activity correlate positively with capillaries density score(p = 0,002) while hemorrhage correlate positively with skin score(p = 0,024). In our study major capillaroscopic abnormalities appeared to be virtually confined to patients with DM while patients with PM had only minor changes. No signifcant correlations between NFC and other demographic, clinical and laboratory parameters were observed. Conclusion NFC may provide helpful information about patients with PM/DM by: (a) contributing to the diagnosis and (b) correlating with disease activity parameters. Additional investigations with larger series of patients and prospective studies may be useful to reinforce our data.

Alterations in activin A–myostatin–follistatin system associate with disease activity in inflammatory myopathies

Rheumatology ◽  
2020 ◽  
Vol 59 (9) ◽  
pp. 2491-2501 ◽  
Author(s):  
Lucia Vernerová ◽  
Veronika Horváthová ◽  
Tereza Kropáčková ◽  
Martina Vokurková ◽  
Martin Klein ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Disease Activity ◽  
Muscle Tissue ◽  
Activin A ◽  
Muscle Disease ◽  
Manual Muscle Testing ◽  
Healthy Controls ◽  
Inflammatory Myopathies ◽  
Muscle Testing ◽  
Tgf Β1

Abstract Objectives The aim of this study was to investigate the systemic and skeletal muscle levels of atrophy-associated myokines in patients with idiopathic inflammatory myopathies (IIM) and their association with clinical characteristics of myositis. Methods A total of 94 IIM patients and 162 healthy controls were recruited. Of those, 20 IIM patients and 28 healthy controls underwent a muscle biopsy. Circulating concentrations of myostatin, follistatin, activin A and TGF-β1 were assessed by ELISA. The expression of myokines and associated genes involved in the myostatin signalling pathway in muscle tissue was determined by real-time PCR. Results We report decreased levels of circulating myostatin (median 1817 vs 2659 pg/ml; P = 0.003) and increased follistatin (1319 vs 1055 pg/ml; P = 0.028) in IIM compared with healthy controls. Activin A levels were also higher in IIM (414 vs 309 pg/ml; P = 0.0005) compared with controls. Myostatin was negatively correlated to muscle disease activity assessed by physician on visual analogue scale (MDA) (r = −0.289, P = 0.015) and positively to manual muscle testing of eight muscles (r = 0.366, P = 0.002). On the other hand, follistatin correlated positively with MDA (r = 0.235, P = 0.047). Gene expression analysis showed higher follistatin (P = 0.003) and myostatin inhibitor follistatin-like 3 protein (FSTL3) (P = 0.008) and lower expression of activin receptor type 1B (ALK4) (P = 0.034), signal transducer SMAD3 (P = 0.023) and atrophy marker atrogin-1 (P = 0.0009) in IIM muscle tissue compared with controls. Conclusion This study shows lower myostatin and higher follistatin levels in circulation and attenuated expression of myostatin pathway signalling components in skeletal muscle of patients with myositis, a newly emerging pattern of the activin A–myostatin–follistatin system in muscle wasting diseases.

Inconsistencies of the Disease Activity Assessment Tools for Psoriatic Arthritis: Challenges to Rheumatologists

2021 ◽  
pp. 105296
Author(s):  
Halise Hande Gezer ◽  
Mehmet Tuncay Duruöz ◽  
Kemal Nas ◽  
Erkan Kılıç ◽  
Betül Sargın ◽  
...  
Keyword(s):  
Psoriatic Arthritis ◽  
Disease Activity ◽  
Assessment Tools ◽  
Activity Assessment ◽  
Disease Activity Assessment

scholarly journals THU0538 IN PSORIATIC ARTHRITIS PATIENTS CONSIDERED IN REMISSION BY THEIR RHEUMATOLOGIST, CAN DISCORDANCE IN DISEASE ACTIVITY ASSESSMENT BETWEEN PATIENT AND RHEUMATOLOGIST BE EXPLAINED BY RESIDUAL INFLAMMATION AS MEASURED BY ULTRASONOGRAPHIC EXAMINATION?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 508-508
Author(s):  
M. Moly ◽  
C. Lukas ◽  
J. Morel ◽  
B. Combe ◽  
G. Mouterde
Keyword(s):  
Psoriatic Arthritis ◽  
Disease Activity ◽  
Demographic Data ◽  
Univariate Analysis ◽  
Power Doppler ◽  
Minimal Disease Activity ◽  
Activity Assessment ◽  
Grey Scale ◽  
Minimal Disease ◽  
Disease Activity Assessment

Background:Psoriatic arthritis (PsA) is a heterogeneous disease and its assessment is sometimes difficult. Perception of disease activity by patient and physician is frequently discordant in patients in clinical remission. Ultrasound (US) is an imaging technique, which can detect inflammation in PsA.Objectives:The aim of our study was to assess whether persistence of disease activity evaluated by the patient, considered in remission by his rheumatologist, was associated with inflammation measured by US.Methods:We performed a transversal monocentric study. PsA patients were included if they met the CASPAR criteria and were considered in remission by their rheumatologist. Demographic data, characteristics of the disease and treatments were collected. Discordance was defined by a difference between patient’s and rheumatologist’s global assessment ≥30/100 on a Visual Analogic Scale. An US examination was performed on 50 joints, 28 tendons and 14 entheses by an independent investigator. Synovial or tendon sheath hypertrophy and PD signal were evaluated on a semi-quantitative scale, B Mode and PD signal abnormalities on entheses were searched, according to the EULAR-OMERACT scoring system. US remission was defined by no power Doppler (PD) signal on joints, tendons and entheses and minimal US activity by maximum one PD signal on the same sites. Univariate and multivariate analyses were performed to evaluate factors associated with US abnormalities.Results:Sixty-two PsA patients were included. 40.3% were women, the mean (SD) age was 55 (14) years, 42% were in US remission and 71% in minimal US activity (Table 1), 19.4% had ≥1 PD synovitis and 88.7% had a B mode synovitis, 95.2% had a B mode abnormality on entheses and 51.6% had ≥1 PD signal on entheses. Thirty nine percent had a discordant disease activity assessment with their rheumatologist. In univariate analysis, discordance was not associated with US remission (OR=1.71 (95%CI 0.61-4.83), p=0.224) or US minimal disease activity (OR=0.99 (95%CI 0.32-3.05), p=0.602). In multivariate analysis, US remission was independently associated with female gender (OR=3.94 (95%CI 1.20-12.9), p=0.024) and younger age (OR=0.95 (95%CI 0.91-0.99), p=0.027). Minimal US activity was associated with history of enthesis lesion (OR=11.26 (95%CI 1.34-94.93), p=0.026) and age (OR=0.95 (95%CI 0.90-1), p=0.044).Table 1.Ultrasound characteristics of the 62 PsA patients.N (%)Ultrasound remission26 (41.9)Ultrasound minimal disease activity44 (71)Patients with ≥1 grey scale synovitis55 (88.7)Patients with ≥1 Power Doppler synovitis12 (19.4)Patients with ≥1 grey scale tenosynovitis15 (24.2)Patients with ≥1 Power Doppler tenosynovitis1 (1.6)Patients with ≥1 grey scale enthesitis lesion (thickness, hypo echogenicity, calcification, enthesophyte, erosion, bursitis)59 (95.2)Patients with ≥1 Power Doppler enthesitis32 (51.6)Conclusion:Our study showed persistent inflammation evaluated by US in PsA patients considered in remission by their rheumatologist. However, prevalence of residual inflammation evaluated by US was not higher in patients with self-assessment of their disease discordant from their rheumatologist.Disclosure of Interests:Marie Moly: None declared, Cédric Lukas: None declared, Jacques Morel: None declared, Bernard Combe Grant/research support from: Novartis, Pfizer, Roche-Chugai, Consultant of: AbbVie; Gilead Sciences, Inc.; Janssen; Eli Lilly and Company; Pfizer; Roche-Chugai; Sanofi, Speakers bureau: Bristol-Myers Squibb; Gilead Sciences, Inc.; Eli Lilly and Company; Merck Sharp & Dohme; Pfizer; Roche-Chugai; UCB, Gael Mouterde: None declared

Intraepithelial Autoimmune Bullous Dermatoses Disease Activity Assessment and Therapy

2021 ◽  
Author(s):  
Carmen M. Montagnon ◽  
Julia S. Lehman ◽  
Dedee F. Murrell ◽  
Michael J. Camilleri ◽  
Stanislav N. Tolkachjov
Keyword(s):  
Disease Activity ◽  
Activity Assessment ◽  
Disease Activity Assessment

THU0356 DISEASE ACTIVITY ASSESSMENT IN SYSTEMIC SCLEROSIS PATIENTS BASED ON THE CURRENTLY AVAILABLE ACTIVITY INDICES AND THE PHYSICIAN GLOBAL ASSESSMENT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 409.1-409
Author(s):  
T. Minier ◽  
V. Lóránd ◽  
Z. Bálint ◽  
D. Komjati ◽  
G. Nagy ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Activity Index ◽  
Activity Assessment ◽  
One Year ◽  
Active Disease ◽  
The Eu ◽  
Disease Activity Assessment ◽  
Activity Indices

Background:Disease activity assessment is crucial in defining the appropriate therapy and to monitor the efficacy of treatment in systemic sclerosis.Objectives:We aimed to test the performance of the ’old’ European Scleroderma Trials and Research Group (EUSTAR) Activity Index (old-AI) (1), the ’new’ EUSTAR activity index (new-AI) (2), and the scleroderma activity index derived from the old-AI (Pecs-AI) (3). We compared the three indices to the disease activity based on the physician’s global assessment (PGA). We also assessed the correlations with the change in modified Rodnan Skin Score (MRSS), FVC and arthritis after one year follow-up.Methods:We evaluated 77 patients (50 diffuse /dcSSc/ and 27 limited cutaneous SSc /lcSSc/ patients) from a single tertiary clinical center. Cohort enrichment was performed to increase the number of patients with early disease and dcSSc. Seventy-two patients were re-evaluated after one year. Nine patients had overlap syndromes: rheumatoid arthritis (n=3), Sjögren syndrome (n=2), polymyositis (n=2), and mixed connective tissue disease (n=2). The overall disease activity was evaluated using both composite indices (old-AI, Pecs-AI, new-AI) and the PGA of disease activity, based on the blinded evaluation of a single physician (LV). In addition to the minimal essential data from the EUSTAR database we also performed detailed assessment of the musculoskeletal involvement evaluating measures of hand function, DAS28 scores, and the Clinical Disease Activity Index (CDAI) (4).Results:Three times more patients with active disease were identified by the new-AI compared to the old-AI at baseline investigation (n=37, 48.7%, vs. n=11, 14.3%). Two patients (18%) with active disease based on the old-AI were missed by the new-AI. Pecs-AI index identified 15 patients (19.5%) with active disease (cut-off >2.75 points). Active disease was equally frequent in dcSSc and lcSSc patients based on old-AI, but was more frequent in dcSSc patients based on the new-AI in the whole cohort, and also after excluding overlap cases.Patients with active disease based on the old-AI had more frequently rheumatoid factor (6/9, vs. 12/45, p=0.047), and DLCO<70% (11/11, vs. 36/65, p<0.01). Active disease based on the new-Al was associated with current cyclophosphamide treatment (9/37, vs.2/39, p=0.023), and diabetes mellitus (7/30, vs. 0/39, p<0.01). The PGA correlated moderately at both baseline and one year follow-up examination with the old-AI (rho: 0.519, and rho: 0.692, respectively, p<0.001), the new-AI (rho: 0.401, and rho: 0.429, respectively, p<0.001), and the Pecs-AI (rho: 0.425, and rho: 0.593, respectively, p<0.001).CDAI correlated significantly with the old-AI (rho: 0.345, and rho: 0.283, respectively, p<0.05) and the Pecs-AI (rho: 0.363, and rho: 0.324, respectively, p<0.05) at both the baseline and one-year follow-up investigations, but showed no consistent correlation to the new-AI or PGA.Conclusion:The two validated disease activity indices indentify different patient groups. Joint involvement is potentially underrepresented in the new EUSTAR activity index. Active disease is also present in lcSSc and should be assessed regularly in these patients.References:[1]Valentini G, et al. Ann Rheum Dis 2003; 62: 901-3.[2]Valentini G, et al. Ann Rheum Dis 2017;76:270–276.[3]Minier T, et al. Rheumatology (Oxford) 2010;49(6):1133-45.[4]Lorand V, et al. Rheumatology (Oxford). 2016;55(10):1849-58.Acknowledgments:This work was supported by the EU Seventh Framework Program [FP7/2007-2013] under Grant Agreement n° 305495 (DeSScipher), by the Hungarian Scientific Research Fund (contract n°: 112939), and the EU under the Grant Agreement n° PEPSYS GINOP-232-15-2016-00050.Disclosure of Interests:Tünde Minier Speakers bureau: Actelion, Abbvie, MSD, Pfizer, Lilly, Roche, Veronika Lóránd: None declared, Zsófia Bálint: None declared, Dalma Komjati: None declared, Gabriella Nagy Speakers bureau: MSD, Antonietta Kovács: None declared, Orsolya Koncz: None declared, Cecília Varjú Consultant of: Boehringer Ingelheim RCV GmbH & Co KG, Speakers bureau: Lilly, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Balazs Nemeth: None declared

Disease activity assessment in rheumatoid arthritis

2020 ◽  
pp. 161-168
Author(s):  
Josef Smolen
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Activity ◽  
Joint Pain ◽  
Joint Stiffness ◽  
Treat To Target ◽  
Therapeutic Decision ◽  
Activity Assessment ◽  
Disease Modifying ◽  
Therapeutic Decision Making ◽  
Disease Activity Assessment

The major clinical hallmarks of rheumatoid arthritis (RA) are articular swelling, joint pain, and morning joint stiffness. Disease activity assessment is pivotal when following patients with RA throughout the course of their disease, and especially when assessing improvement or deterioration upon institution of the necessary therapies. To prevent an adverse outcome, it is essential to diagnose the disease early and to start treatment with disease-modifying antirheumatic drugs (DMARDs) immediately after diagnosis. Adhering to the treat-to-target approach, which is a central strategy irrespective of the type of treatment available and the therapy applied, requires consistency in using validated composite measures of disease activity. Rather than a mere matter of using specific therapies, it is also a matter of using tools for disease activity assessment to guide therapeutic decision-making. This enables offering and achieving the best possible outcomes for RA patients.

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