P015 Imaging findings in cervical spine in patients with Juvenile Idiopathic Arthritis in Tunisia

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Hiba Bettaieb ◽  
Hanene Ferjani ◽  
Kaouther Maatallah ◽  
Dorra Ben Nessib ◽  
Wafa Triki ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Cervical Spine ◽  
Disease Activity ◽  
Activity Index ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Joint Damage ◽  
Odontoid Process ◽  
P Value ◽  
Limited Range Of Motion

Abstract Background Juvenile Idiopathic Arthritis (JIA) is a chronic disease characterized by prolonged synovial inflammation that may cause structural joint damage. However, little is known about cervical spine involvement in JIA. The main objective of this study is to describe radiological findings of the cervical spine in patients with JIA. Methods We conduct a retrospective monocentric study. All JIA patients were included (ILAR criteria). Sociodemographic, JIA subtype, and clinical characteristics were collected. Disease activity at JIA diagnosis was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis-related enthesitis form. Cervical spine radiographs including anteroposterior and lateral with flexion views were analyzed. A p-value < 0.05 was considered significant. Results We included 25 patients (16 girls and 9 boys) diagnosed with JIA with a mean age at disease onset of 9.9 ± 3.9 [3–16]. The median disease duration was 36 months (IQR 25–75%; 30–84). The JIA subtypes were in decreasing order of frequency: Enthesitis-related Arthritis (n = 9), Oligoarticular (n = 6), Polyarticular RF- (n = 4), Polyarticular RF + (n = 2), Systemic (n = 2), Psoriatic Arthritis (n = 1), and Undifferentiated (n = 1). Median ESR and CRP were 17 mm/h [2–98] and 15.4 mg/l [0–56] respectively. The Median BASDAI score was 2.8 [1–6.3]. Median JADAS10 score was 5.3 [0–20]. Four patients (16%) were on long-term corticosteroid therapy. Five patients (20 %) have a cervical spine involvement with the following subtypes: Polyarticular (n = 2), enthesitisenthesitis-related arthritis (n = 2), and systemic (n = 1). Clinical manifestations were neck pain (n = 3) and limited range of motion (n = 4). Neurological examination noted brisk deep tendon reflexes (n = 6), positive Babinski reflex (n = 1) and positive Hoffmann reflex (n = 2). No patient had a neurological deficit. The conventional radiographs of the cervical spine showed: loss of cervical lordosis (n = 2), diastasis C1-C2 (n = 3), erosion of the odontoid process (n = 1), and anterior ankylosis (n = 3). Subsequent cervical spine MRI confirmed these findings and showed pannus at the craniocervical junction in one case and block vertebra of C6-C7 in another case. Atlanto-axial subluxation was anteroposterior in 3 patients and rotatory in one. Conclusion Cervical spine involvement is frequent and underestimated in JIA, and its radiological features are various. Hence, regular radiographic monitoring of the cervical spine is required to prevent the development of this complication.

scholarly journals AB0740 IMPACT OF DIAGNOSIS DELAY ON DISEASE PARAMETERS DURING JUVENILE IDIOPATHIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1399.2-1399
Author(s):  
H. Bettaieb ◽  
H. Ferjani ◽  
K. Maatallah ◽  
D. Kaffel ◽  
W. Hamdi
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Bone Loss ◽  
Disease Activity ◽  
Activity Index ◽  
Joint Destruction ◽  
Disease Onset ◽  
Atlantoaxial Subluxation ◽  
P Value ◽  
Diagnosis Delay ◽  
Delay In Diagnosis

Background:Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in childhood (1). Prompt diagnosis is mandatory to avoid joint destruction and growth abnormalities. However, it’s often misdiagnosed by pediatricians and general practitioners leading to longer diagnosis delay (2).Objectives:The aim of this study was to evaluate the lag time between JIA symptoms onset and diagnosis and its impact on disease activity and bone loss.Methods:A retrospective monocentric study was carried out on JIA patients (ILAR criteria). Diagnosis delay was collected from the patient’s medical files. Disease activity at JIA diagnosis was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis related enthesitis form. The data were analyzed using the SPSS statistical package. A p value < 0.05 was considered significant.Results:We enrolled 48 JIA (31 male and 17 female) with a mean age at disease onset of 11.2 ± 3.8 years. The median disease duration was 84 months [2-408]. The median JIA diagnosis delay was 8 months [1-108]. The JIA subgroups were in decreasing order of frequency: Enthesitis-related Arthritis (n=32), Polyarticular RF- (n=4), Polyarticular RF+ (n=2), Oligoarticular (n=6), Systemic (n=2), Psoriatic Arthritis (n=1) and Undifferentiated (n=1).At diagnosis, median ESR and CRP were 44 mm/hour [2-100] and 24 mg/l [2-86] respectively. Median JADAS10 score was 4 [0-21]. Median BASDAI score was 6.2 [2-9.4].At follow-up, five patients (10.4%) had atlantoaxial subluxation and 17 had coxitis (43.8%).At bone densitometry, 45% of patients had osteroposis and 27.5% had osteopenia.An agreement was assessed between a long diagnosis delay and the following parameters: male gender (p=0.04) and osteoporosis (p=0.018). A Significant positive correlation was found between delay in JIA diagnosis and BASDAI score (p=0.047, r=0.63). No association was found between JIA diagnosis delay and JADAS score (p=0.56). Neither ESR (p=0.19) nor CRP (p=0.42) was associated with JIA diagnosis delay.Finally, no link was observed with the occurrence of hip arthritis (p=0.281) or atlantoaxial subluxation (p=0.137).Conclusion:In this study, delay in diagnosis was associated with higher disease activity scores and bone loss. Our results suggest that early identification and treatment of JIA leads to improved outcomes as well as bone mass.References:[1]Petty R.E., Southwood T.R., Manners P. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31(2):390.[2]Foster HE, Scott C, Tiderius CJ,et al. Improving musculoskeletal health for children and young people - A ‘call to action’. Best Pract Res Clin Rheumatol. 2020 Oct;34(5):101566.Disclosure of Interests:None declared

P009 Osteoporosis is a frequent complication in enthesitis related arthritis patients

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Hanene Ferjani ◽  
Hiba Bettaieb ◽  
Kaouther Maatallah ◽  
Dorra Ben Nessib ◽  
Wafa Triki ◽  
...  
Keyword(s):  
Disease Activity ◽  
Corticosteroid Therapy ◽  
Disease Duration ◽  
Activity Index ◽  
Demographic Data ◽  
Disease Onset ◽  
Osteoporotic Fractures ◽  
P Value ◽  
Bone Mass Loss ◽  
Enthesitis Related Arthritis

Abstract Background Enthesitis related arthritis (ERA) represents a clinical entity of juvenile idiopathic arthritis. This chronic rheumatic disease may lead to early bone mass loss and increase risk fracture. The aims of this study were to evaluate the prevalence of clinical osteoporosis in patients with ERA and to identify what factors are associated with increased occurrence of osteoporosis. Methods We reviewed the medical records of patients with confirmed ERA. We analyzed their demographic data and the clinical characteristics. Dual-energy X-ray absorptiometry (DEXA) was used to determine bone status. Osteoporosis was defined as Z score &lt;-2.5DS. Disease activity was evaluated by: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Data were analyzed using the SPSS statistical package. A P-value &lt; 0.05 was considered significant. Results Thirty-three patients (27 male and 7 female) with a mean age at of 23.8 ± 7.5 years were enrolled. The mean age at disease onset was 12 ± 2.6 years. Median disease duration was 108 months [12–408]. The median ESR and CRP levels were 35 mm/h [8–90] and 20 mg/l [1–70] respectively. Median BASDAI score was 4.7 [1–9.7]. At bone densitometry, osteoporosis and osteopenia were found in 44.1% and 23.5% cases respectively. None of the patients had a history of osteoporotic fractures. Long term corticosteroid therapy and sedentarily were noted in 18.2% and 47.1% of patients respectively. On statistical analysis, osteoporosis was associated with these parameters: age at ERA onset (P = 0.035), disease duration (P = 0.04), CRP (P = 0.009), BASDAI score (P = 0.05) and sedentarily (P = 0.031). Neither corticosteroid therapy (P = 0.68) nor high ESR level (P = 0.73) were associated with osteoporosis. Conclusion In this study, osteoporosis was a common extra articular feature during ERA. As adult spondyloarthritis, disease activity, duration and sedentarily seem to be associated with the bone loss.

scholarly journals AB0731 CERVICAL SPINE INVOLVEMENT IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1396.1-1396
Author(s):  
S. Bouden ◽  
N. Ben Chekaya ◽  
A. Ben Tekaya ◽  
O. Saidane ◽  
R. Tekaya ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Cervical Spine ◽  
Rheumatoid Factor ◽  
Disease Onset ◽  
Odontoid Process ◽  
Basilar Invagination ◽  
X Rays ◽  
Radiological Data ◽  
Adult Rheumatoid Arthritis ◽  
The Mean

Background:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. In contrast to adult rheumatoid arthritis, where numerous studies have shown a high prevalence of involvement of the cervical spine, few studies have been published examining this entity in JIA.Objectives:We aimed to analyze clinical and radiological findings of cervical spine involvement in patients with JIA.Methods:A retrospective study including 35 patients was conducted between 2010 and 2019. The patients enrolled met the ILAR criteria for the diagnosis of JIA. Clinical, biological, and radiological data were collected. Patients had a radiological evaluation that included cervical spine x-rays in antero-posterior, lateral and lateral views with flexion.Results:Thirty-five patients were enrolled. The mean age of the disease onset was 9 years [3-15]. The mean age of the patients at the time of the study was 37.8 years [17-69]. The mean duration of the disease was 27 years [2-56]. These patients were assigned to discrete JIA categories: rheumatoid factor positive polyarthritis (43.5%), rheumatoid factor negative polyarthritis (21.7%), enthesitis-related arthritis (17.4%), oligoarthritis (13%) and psoriatic arthritis (4.4%). Sixteen patients (45%) reported neck pain. Cervical spine involvement occurred on average 7 years [0-13] after the JIA onset. Cervical spine radiographs showed anterior atlantoaxial subluxation (> 5mm) in 8 patients. Magnetic-resonance imagining was performed in 9 patients that had abnormal neurological examination showing a pannus formation of C1-C2 junction (3 cases), a basilar invagination (4 cases) and erosions of the odontoid process (2 cases). A cervical collar has been used for immobilization in patients with significant cervical spine damage. A C1-C2 arthrodesis was proposed to 4 patients.Conclusion:These findings suggest that the presence of cervical involvement in JIA patients is frequent. Radiologic assessment of cervical spine should be systematically performed for early detection and to prevent its complications.Disclosure of Interests:None declared

scholarly journals AB0741 PREVALENCE AND ASSOCIATED FACTORS OF ATLANTO-AXIAL INSTABILITY IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1399.3-1400
Author(s):  
H. Bettaieb ◽  
H. Ferjani ◽  
K. Maatallah ◽  
H. Boussaa ◽  
D. Kaffel ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Cervical Spine ◽  
Disease Activity ◽  
Disease Onset ◽  
Atlantoaxial Subluxation ◽  
Upper Cervical Spine ◽  
Diagnosis Delay ◽  
Factors Associated ◽  
Increased Risk

Background:Atlanto-axial instability (AAI) is a serious complication during Juvenile Idiopathic Arthritis (JIA). It can lead to severe neurological morbidity or mortality if left untreated (1).Objectives:To determine the prevalence of AAI in patients with JIA and to identify factors associated with an increased risk of its occurrence.Methods:A retrospective monocentric study was carried out on JIA patients (ILAR criteria). Data, including age at disease onset, JIA type, disease activity at AAI diagnosis and treatment were collected. Disease activity at JIA diagnosis was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis related enthesitis form. Standard radiographs of the cervical spine were analyzed. The data were analyzed using the SPSS statistical package. A p value < 0.05 was considered significant.Results:We enrolled 48 JIA (31 male and 17 female) with a mean age at disease onset of 11.2 ± 3.8 years. The median disease duration was 84 months [2-408]. The median JIA diagnosis delay was 8 months [1-108]. The JIA subgroups were in decreasing order of frequency: Enthesitis-related Arthritis (n=32), Polyarticular RF- (n=4), Polyarticular RF+ (n=2), Oligoarticular (n=6), Systemic (n=2), Psoriatic Arthritis (n=1) and Undifferentiated (n=1).At diagnosis, median ESR and CRP were 44 mm/hour [2-100] and 24 mg/l [2-86] respectively. Median JADAS10 score was 4 [0-21]. Median BASDAI score was 6.2 [2-9.4].At follow-up, five patients (10.4%) had atlantoaxial subluxation and 17 had coxitis (43.8%).At bone densitometry, 45% of patients had osteroposis and 27.5% had osteopenia.An agreement was assessed between a long diagnosis delay and the following parameters: male gender (p=0.04) and osteoporosis (p=0.018). A Significant positive correlation was found between delay in JIA diagnosis and BASDAI score (p=0.047, r=0.63). No association was found between JIA diagnosis delay and JADAS score (p=0.56). Neither ESR (p=0.19) nor CRP (p=0.42) was associated with JIA diagnosis delay.Finally, no link was observed with the occurrence of hip arthritis (p=0.281) or atlantoaxial subluxation (p=0.137).Conclusion:In our study, the prevalence of AAI was 10.4%. Prolonged corticosteroid use and elevated inflammatory markers were the major factors associated with an increased risk of upper cervical spine involvement. Hence, targeted treatments are required to prevent cervical spine instability.References:[1]Hospach T, Maier J, Müller-Abt P, Patel A, Horneff G, von Kalle T. Cervical spine involvement in patients with juvenile idiopathic arthritis - MRI follow-up study. Pediatr Rheumatol Online J. 2014;12:9.Disclosure of Interests:None declared

Elevated levels of IL-24 in systemic lupus erythematosus patients

Lupus ◽  
2019 ◽  
Vol 28 (6) ◽  
pp. 748-754 ◽  
Author(s):  
R C Li ◽  
J Guo ◽  
L C Su ◽  
A F Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Inflammatory Cytokine ◽  
Activity Index ◽  
Clinical Manifestations ◽  
Mrna Levels ◽  
Healthy Controls ◽  
Systemic Lupus ◽  
Good Ability

Objective This study aimed to assess IL-24 levels and their association with clinical manifestations in patients with systemic lupus erythematosus (SLE). Methods There were 75 patients with SLE and 58 healthy controls recruited in this study. Serum levels of IL-24 were measured by enzyme-linked immunosorbent assays, and mRNA levels of IL-24 were tested by quantitative real-time polymerase chain reaction . The area under the curve of the receiver operating characteristic (ROC) curve was used for diagnostic ability of the inflammatory cytokine. Results Serum IL-24 levels were significantly higher in SLE patients than that in healthy controls. SLE patients with nephritis had higher IL-24 levels than those without nephritis. Active SLE patients showed higher expression of IL-24 as compared to less active disease patients. The mRNA levels of IL-24 were much higher in SLE patients. Correlation analysis showed significant correlation between serum IL-24 levels and SLE disease activity index. In addition, ROC analysis may suggest good ability of serum IL-24 in differentiating SLE. Conclusion The inflammatory cytokine correlated with SLE disease activity, and may be involved in this disease pathogenesis.

scholarly journals AB0669 PARTICULARITIES OF TUNISIAN FEMALE AXIAL SPONDYLOARTHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1629.2-1629
Author(s):  
K. Ben Abdelghani ◽  
Y. Gzam ◽  
A. Fazaa ◽  
S. Miladi ◽  
K. Ouenniche ◽  
...  
Keyword(s):  
Ankylosing Spondylitis ◽  
Disease Activity ◽  
Axial Spondyloarthritis ◽  
Activity Index ◽  
Age At Onset ◽  
Disease Onset ◽  
Disease Activity Index ◽  
Reactive Protein ◽  
Significant Difference ◽  
The Mean

Background:Axial spondyloarthritis (ax-SpA) is a chronic rheumatic disease that mainly affects men. However, the female form of ax-SpA remains insufficiently studied.Objectives:The aim of this study was to determine the clinical characteristics, the disease activity and the functional impact of female ax-SpA in comparison with male ax-SpA.Methods:This is a retrospective study including patients diagnosed with ax-SpA fulfilling the criteria of the Assessment of SpondyloArthritis international Society (ASAS) 2009.Clinical parameters, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Bath ankylosing spondylitis disease activity index (BASDAI) and Bath ankylosing spondylitis functional index (BASFI) were compared between groups of female and male ax-SpA.Results:Two hundred ax-SpA patients were included with 31% of female (n=62) and a mean age of 43,3 ± 11,2 years.The mean age at onset of symptoms was 31,8 ± 8,9 years for women and 25,3 ± 9,1 years for men (p <0,0001). The mean age at diagnosis was 36,4 ± 9,6 years for women and 31,7 ± 10,4 years for men (p = 0,003). Ax-SpA with juvenile onset was noted in 1,7% of women and 12,1% of men (p = 0,02). Male ax-SpA were significantly more smokers (46.8% vs 5.4%; p <0.001). The mean duration of morning stiffness was 11,3 ± 9,2 minutes for women versus 21,6 ± 19,3 minutes for men (p = 0,005).The mean ESR was 42,4 ± 29,8 mm for women and 28,3 ± 23,4 mm for men (p = 0,001). Radiographic sacroiliitis was present in 69,3% of women versus 84,7% of men (p = 0,01). The use of anti-TNF alpha was less frequent in women (29% vs 48,5%; p = 0,01).Our study didn’t found a statistically significant difference in peripheral manifestations, extraarticular manifestations, CRP, BASDAI and BASFI between the two groups.Conclusion:Female ax-SpA seems to have a better prognosis than male with older age in disease onset, less inflammation, less radiographic sacroiliitis and less use of biological treatments.References:[1]Rusman T, et al. Curr Rheumatol Rep. 2018; 20(6).[2]Siar N, et al. Curr Rheumatol Rev. 2019;Disclosure of Interests:None declared

scholarly journals Impacts of FcγRIIB and FcγRIIIA gene polymorphisms on systemic lupus erythematous disease activity index

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Mansoor Karimifar ◽  
Khosro Akbari ◽  
Reza ArefNezhad ◽  
Farshid Fathi ◽  
Mohammad Moosaeepour ◽  
...  
Keyword(s):  
Disease Activity ◽  
Activity Index ◽  
Clinical Manifestations ◽  
Disease Activity Index ◽  
Unknown Etiology ◽  
Systemic Lupus Erythematous ◽  
Nucleotide Polymorphisms ◽  
Systemic Lupus ◽  
Single Nucleotide ◽  
Chronic Autoimmune Disease

Abstract Objective Systemic lupus erythematous (SLE) disease is a chronic autoimmune disease with unknown etiology that can involve different organs. Polymorphisms in Fcγ receptors have been identified as genetic factors in susceptibility to SLE. This study was aimed to investigate effects of two single nucleotide polymorphisms (SNPs) within FcγRIIB and FcγRIIIA genes on systemic lupus erythematous disease activity index (SLEDAI) in an Iranian population. Results Our findings indicated TT and GG genotypes were the common genotypes of FcγRIIB and FcγRIIIA SNPs in SLE patients, respectively. There were no significant differences in genotype and allele frequencies of FcγRIIB and FcγRIIIA SNPs in SLE and healthy subjects. However, the frequencies of genotypes and alleles of FcγRIIB and FcγRIIIA SNPs were significantly associated with some clinical manifestations used to determine SLEDAI (P < 0.001–0.5).

scholarly journals Systemic lupus erythaematosus in a multiethnic US cohort (LUMINA) LIII: disease expression and outcome in acute onset lupus

2007 ◽  
Vol 67 (4) ◽  
pp. 500-504 ◽  
Author(s):  
A M Bertoli ◽  
L M Vilá ◽  
J D Reveille ◽  
G S Alarcón ◽  
Keyword(s):  
Disease Activity ◽  
Renal Involvement ◽  
Multivariable Analysis ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Acute Onset ◽  
Acute Disease ◽  
Stepwise Logistic Regression ◽  
Systemic Lupus ◽  
Damage Accrual

Objective:To determine the features associated with acute onset systemic lupus erythaematosus (SLE).Methods:A total of 631 SLE patients from LUMINA (for “lupus in minority populations: nature vs nurture”), a multiethnic (Hispanics, African–Americans and Caucasians) cohort, were studied. Acute disease onset was defined as the accrual of ⩾4 American College of Rheumatology (ACR) criteria for the classification of SLE in ⩽4 weeks. Socioeconomic demographic features, clinical manifestations, disease activity, damage accrual, mortality, autoantibodies, HLA class II and FCGR alleles, behavioural/psychological variables were compared between patients with acute and insidious disease onset by univariable (χ2 and Student t test) and multivariable (stepwise logistic regression) analyses.Results:A total of 94 (15%) patients had acute disease onset. In the multivariable analysis, patients with acute onset lupus had more renal involvement (odds ratio (OR) = 1.845, 95% CI 1.076–3.162; p = 0.026) and higher disease activity (OR = 1.057, 95% CI 1.005–1.112; p = 0.030). By contrast, age (OR = 0.976, 95% CI 0.956–0.997; p = 0.025), education (OR = 0.901, 95% CI 0.827–0.983, p = 0.019), health insurance (OR = 0.423, 95% CI 0.249–0.718; p = 0.001) and skin involvement (OR = 0.346, 95% CI 0.142–0.843; p = 0.019) were negatively associated with acute onset lupus. No differences were found regarding the serological, genetic and behavioural/psychological features; this was also the case for damage accrual and mortality.Conclusions:Patients with acute onset lupus seem to be younger, have a lower socio-economic status and display more severe disease in terms of clinical manifestations and disease activity. However, intermediate (damage) and long-term (mortality) outcomes appear not to be influenced by the type of disease onset in SLE.

The Association Between Disease Activity and Duration in Systemic Sclerosis

2010 ◽  
Vol 37 (11) ◽  
pp. 2299-2306 ◽  
Author(s):  
JENNIFER G. WALKER ◽  
RUSSELL J. STEELE ◽  
MIREILLE SCHNITZER ◽  
SUZANNE TAILLEFER ◽  
MURRAY BARON ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Disease Activity ◽  
Disease Duration ◽  
Activity Index ◽  
Disease Onset ◽  
Depression Scale ◽  
Disease Activity Index ◽  
Cross Sectional ◽  
Diffuse Disease ◽  
Patient Reported

Objective.The absence of a standardized disease activity index has been an important barrier in systemic sclerosis (SSc) research. We applied the newly derived Valentini Scleroderma Disease Activity Index (SDAI) among our cohort of patients with SSc to document changes in disease activity over time and to assess possible differences in activity between limited and diffuse disease.Methods.Cross-sectional study of a national cohort of patients enrolled in the Canadian Scleroderma Research Group Registry. Disease activity was measured using the SDAI. Depression scores were measured using the Centre for Epidemiologic Studies Depression Scale (CES-D).Results.A total of 326 out of 639 patients had complete datasets at the time of this analysis; 87% were female, of mean age 55.6 years, with mean disease duration 14.1 years. SDAI declined steeply in the first 5 years after disease onset and patients with diffuse disease had 42% higher SDAI scores than patients with limited disease with the same disease duration and depression scores (standardized relative risk 1.42, 95% CI 1.21, 1.65). Patients with higher CES-D scores had higher SDAI scores relative to patients with the same disease duration and disease subset (standardized RR 1.22, 95% CI 1.14, 1.31). Among the 10 components that make up the SDAI, only skin score (standardized OR 0.59, 95% CI 0.43, 0.82) and patient-reported change in skin (standardized OR 0.64, 95% CI 0.45, 0.92) decreased with increasing disease duration. High skin scores (standardized OR 32.2, 95% CI 15.8, 72.0) were more likely and scleredema (standardized OR 0.58, 95% CI 0.37, 0.92) was less likely to be present in patients with diffuse disease. High depression scores were associated with positive responses for patient-reported changes in skin and cardiopulmonary function.Conclusion.Disease activity declined with time and patients with diffuse disease had consistently higher SDAI scores. Depression was found to be associated with higher patient activity scores and strongly associated with patient self-response questions. The role of depression should be carefully considered in future applications of the SDAI, particularly as several components of the score rely upon patient recall.

Development and preliminary validation of a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis

2010 ◽  
Vol 70 (3) ◽  
pp. 440-446 ◽  
Author(s):  
Clara Malattia ◽  
Maria Beatrice Damasio ◽  
Angela Pistorio ◽  
Maka Ioseliani ◽  
Iris Vilca ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Construct Validity ◽  
Disease Activity ◽  
Scoring System ◽  
Bone Erosion ◽  
Joint Damage ◽  
Targeted Mri ◽  
Mri Score ◽  
Responsiveness To Change ◽  
Assessment Of Disease Activity

ObjectivesTo develop and validate a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis (JIA). To compare the paediatric MRI score with the adult-designed. Outcome Measures in Rheumatology Clinical Trials—Rheumatoid Arthritis MRI Score (RAMRIS), whose suitability for assessing growing joints was tested.MethodsIn 66 patients with JIA the clinically more affected wrist was studied. Thirty-nine patients had a 1-year MRI follow-up. Two readers independently assigned the paediatric score and the RAMRIS to all studies. Validation procedures included analysis of reliability, construct validity and responsiveness to change. A reduced version of the bone erosion score was also developed and tested.ResultsThe paediatric score showed an excellent reproducibility (interclass correlation coefficient >0.9). The interobserver agreement of RAMRIS was moderate for bone erosions and excellent for bone marrow oedema (BMO). The paediatric score and RAMRIS provided similar results for construct validity. The responsiveness to change of the paediatric score was moderate for synovitis and bone erosion, and poor for BMO and did not improve when RAMRIS was applied. The reduced version of the bone erosion was valuable for the assessment of joint damage, and provided time-saving advantages.ConclusionThe results demonstrate that the paediatric MRI score is a reliable and valid method for assessing disease activity and damage in JIA. Unexpectedly, the RAMRIS provides acceptable suitability for use in the paediatric age group. Further work, especially in a longitudinal setting, is required before defining the most suitable MRI scale for assessing growing joints.

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