scholarly journals POS1315 USEFULNESS OF SYNOVIAL BIOPSY IN THE DIFFERENTIAL DIAGNOSIS AND AS POSSIBLE PREDICTOR OF RESPONSE TO TREATMENT IN JUVENILE IDIOPATHIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 940.1-940
Author(s):  
S. Costi ◽  
F. Pregnolato ◽  
A. Parafioriti ◽  
E. Armiraglio ◽  
T. Giani ◽  
...  

Background:While synovial biopsy is an invasive procedure and is not required for the diagnosis of juvenile idiopathic arthritis (JIA), it may be useful in doubtful cases.Objectives:Aims of the study were i.to verify the role of synovial biopsy in the differential diagnosis of JIA and ii. to review the pathology slides in order to evaluate possible associations of a histologic pattern with response to treatment.Methods:We reviewed data from medical records of patients under the age of 18 years who underwent a synovial biopsy requested by our Pediatric Rheumatology Unit over the last 10 years. We collected information on demographic, clinical, laboratory, radiological, histopathological characteristics, as well as treatment response (in particular, remission at the last visit and number of examination, number of biologic drugs used). Among variables in the histologic score, number of layers in the synovial lining and inflammatory infiltrate (0-5) were compared to clinical status at last visit. Potential differences in variables between responders and non responders were assessed by unpaired t-test or non-parametric Mann-Whitney test, as appropriate.Results:We identified 64 patients (40F, 24M) with a median age at disease onset of 9 years (range 1-15) and a median follow-up time of 161 months (range 8-1160). We recognized two groups of interest: patients with a known JIA diagnosis (28/64) and patients with unknown diagnosis (36/64) at the moment of synovial biopsy. In the group with known JIA, most underwent the procedure during orthopedic surgery, and in all cases the histology was consistent with JIA. Among the unknown diagnosis group, in 19 cases results were consistent with a chronic synovitis, while among the other 17 histology could lead to a diagnosis of other conditions in 6 cases (foreign body and villonodular synovitis n=2 each, sarcoidosis and osteochondromatosis n=1 each). In the remaining 11 the final diagnoses were varied (mostly genetic forms eg skeletal dysplasia, CACP, Thiemann disease).Between the two groups we identified 46 patients with a definite JIA diagnosis. At the last follow-up visit 29 of them were in clinical remission, albeit on medication. The remaining 17 had a severe course of disease, with persistent activity and use of at least two biologic drugs. In 26 cases we could evaluate the correlation between status at last visit and number of layers/inflammatory infiltrate, but no statistical significant correlation was found.Conclusion:Despite its limited use nowadays, synovial biopsy may still be a useful tool in patients whose diagnosis is unclear. In our study, while it confirmed the suspicion in most cases, in other instances it allowed the diagnosis of rare conditions that would have been otherwise missed. No association between disease course and histological features in a small JIA cohort was found. We are currently expanding the study with a larger series.Disclosure of Interests:None declared

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Rana Harhay ◽  
Wajiha Jeelani ◽  
Barbine Tchamba Agbor Agbor ◽  
Teresa Hennon ◽  
Brian H. Wrotniak ◽  
...  

Abstract Background Oligo-articular juvenile idiopathic arthritis (Oligo JIA) is the most common subtype of juvenile idiopathic arthritis. Intra-articular corticosteroid (IAC) injection is a mainstay treatment of oligo JIA providing pain relief, improving mobility and preventing further joint destruction in the majority of patients. In 2015, production of triamcinolone hexacetonide (TH) an intra-articular corticosteroid was discontinued in the United States leading to use of triamcinolone acetonide (TA) as an alternative. In this study, we compared response to treatment in children with oligo JIA who underwent therapy with intra-articular TA and TH injection. Methods Our study is a retrospective chart review of children with oligo JIA who were treated with IAC injections with TH between January 2012 and June 2015 and TA between J uly 2015 and December 2018. The two groups were followed at John R. Oishei Children’s Hospital of Buffalo and were evaluated for response to treatment, side effects and predictors of response including duration of disease before treatment, erythrocyte sedimentation rate (ESR), and c-reactive protein (CRP). Response to treatment was defined as at least 6 months follow up without evidence of active arthritis in injected joints. Patients were considered to be non-responders if they continued to show active arthritis during their first follow up after joint injection. The primary objective was to evaluate whether there was a significant difference in rate of response between TH and TA. Results Forty-nine patients, 38 female and 11 male with oligo JIA were included in the study. The average age was 6.7 years. A total of 111 joints were injected includin g 78 knees, 13 ankles, 9 wrists, 4 hips, 4 elbows, 2 TMJ and one subtalar joint. In the TA group, 49% (29/59) did not show response to injection compared to 27% (14/52) in the TH group. After 6 months, response rates were better for individuals injected with TH compared to TA (73% vs. 51%). In general, response to intra-articular TH was superior to TA with P = .016 using chi-square test of independence. This difference in outcome was not influenced by other variables such as duration of illness before treatment (P value 0.784) or elevated ESR and CRP. No difference in side effects between the two groups were noted. Conclusion Our results in conjunction with prior published data suggests that TH intra-articular joint injection in oligo JIA is superior to TA, although future controlled trials are necessary for confirmation. An effective, long lasting treatment can have a great impact on the outcome of these children.


2010 ◽  
Vol 43 (5) ◽  
pp. 599-601 ◽  
Author(s):  
Maria Stella Amorim da Costa Zöllner ◽  
Karla Mayra Pinto e Carvalho Rezende ◽  
Simone Birman ◽  
Chelna Paolichi Ferro Elias ◽  
Emília Ângela Loschiavo Arisawa ◽  
...  

The type of pulmonary histoplasmosis presents limited lesions to the lungs, with symptoms that are clinically and radiological similar to chronic pulmonary tuberculosis. This paper describes the clinical features of four cases of pulmonary histoplasmosis. Aspects of diagnostic and clinical, epidemiological, laboratorial and imaging exams are discussed, in addition to the clinical status of the individuals five years after disease onset. The treatment of choice was oral medication, following which all the patients improved. It is important to understand the clinical status and the difficulties concerning the differential diagnosis of histoplasmosis, to assist the proper indication of cases, thus reducing potential confusion with other diseases.


2021 ◽  
Author(s):  
Céline La ◽  
Phu Quoc Lê ◽  
Alina Ferster ◽  
Laurence Goffin ◽  
Delphine Spruyt ◽  
...  

Abstract IntroductionIn the management of juvenile idiopathic arthritis (JIA), there is a lack of diagnostic and prognostic biomarkers. This study assesses the use of serum calprotectin (sCal) as a marker to monitor disease activity, and as a classification and prognosis tool of response to treatment or risk of flares in patients with JIA. MethodsEighty-one patients with JIA from the CAP48 multicentric cohort were included in this study, as well as 11 non-pediatric healthy controls. An enzyme-linked immunosorbent assay (ELISA) method was used to quantify sCal with a commercial kit.ResultsPatients with an active disease compared to healthy controls and to patients with inactive disease showed an 8-fold and a 2-fold increased level of sCal respectively. sCal was found to be correlated with the CRP and even more strongly with the ESR. Evolution of DAS28 scores correlated well with evolution of sCal, as opposed to evolution of CRP. With regard to CRP, sCal could differentiate forms with active oligoarthritis from polyarthritis and systemic forms. However, sCal brought an added value compared to the CRP as a prognosis marker. Indeed, patients with active disease and reaching minimal disease activity (according to JADAS) at 6 months following the test had higher sCal levels, while patients with inactive disease had higher sCal levels if a flare was observed up to 3 to 9 months following the test.ConclusionsThis study confirms the potential uses of serum calprotectin as a biomarker in the diagnosis and follow-up of JIA.


2017 ◽  
Vol 103 (5) ◽  
pp. 437-443 ◽  
Author(s):  
Amir Rashid ◽  
Lis Cordingley ◽  
Roberto Carrasco ◽  
Helen E Foster ◽  
Eileen M Baildam ◽  
...  

ObjectivesPain is a very common symptom of juvenile idiopathic arthritis (JIA). Disease activity alone cannot explain symptoms of pain in all children, suggesting other factors may be relevant. The objectives of this study were to describe the different patterns of pain experienced over time in children with JIA and to identify predictors of which children are likely to experience ongoing pain.MethodsThis study used longitudinal-data from patients (aged 1–16 years) with new-onset JIA. Baseline and up to 5-year follow-up pain data from the Childhood Arthritis Prospective Study (CAPS) were used. A two-step approach was adopted. First, pain trajectories were modelled using a discrete mixture model. Second, multinomial logistic regression was used to determine the association between variables and trajectories.ResultsData from 851 individuals were included (4 years, median follow-up). A three-group trajectory model was identified: consistently low pain (n=453), improved pain (n=254) and consistently high pain (n=144). Children with improved pain or consistently high pain differed on average at baseline from consistently low pain. Older age at onset, poor function/disability and longer disease duration at baseline were associated with consistently high pain compared with consistently low pain. Early increases in pain and poor function/disability were also associated with consistently high pain compared with consistently low pain.ConclusionsThis study has identified routinely collected clinical factors, which may indicate those individuals with JIA at risk of poor pain outcomes earlier in disease. Identifying those at highest risk of poor pain outcomes at disease onset may enable targeted pain management strategies to be implemented early in disease thus reducing the risk of poor pain outcomes.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 935.2-936
Author(s):  
S. Lanni ◽  
O. De Lucia ◽  
S. Orsi ◽  
S. Costi ◽  
G. Beretta ◽  
...  

Background:The ankle is one of the most commonly affected sites in juvenile idiopathic arthritis (JIA). This region has a complex anatomical structure owing to the presence of multiple joint recesses and surrounding tendons. While the prognostic value of ultrasound (US)-detected arthritis has been investigated in recent studies, the role of tenosynovitis in JIA remains still unexplored.Objectives:To investigate: 1) US features of ankle involvement in JIA at disease onset; 2) the predictive value of US-detected tenosynovitis in ankles with clinically active disease of children with new-onset JIA.Methods:The clinical charts of all consecutive patients with new-onset JIA between May 2018 and January 2020 at study centres (Policlinico and G.Pini Hospitals of Milan) and with clinically active ankle disease among the joints affected were reviewed retrospectively. Data on ankle US assessment were retrieved and patients were then stratified as follows: 1) patients with detection on US of isolated arthritis in at least one of the joint recesses of the ankle region; 2) patients with detection on US of tenosynovitis in at least one of the tendon compartments of the ankle irrespective of the presence of concomitant arthritis. For each of these two categories, estimation of patients who were able to achieve clinical disease remission at 12 months since disease onset was evaluated.Results:Twenty-seven new-onset JIA patients were found to have clinical involvement of the ankle among the joints affected. Nine of them (33.3%) showed on US isolated arthritis of the ankle, whereas US-detected tenosynovitis was found in 18 (66.7%) patients. The amount of patients who were able to achieve disease remission at 12-months was the same (66.7%) for both patients with and without US-detected tenosynovitis in the ankle (12/18 and 6/9 patients, respectively). In patients with US-detected tenosynovitis and clinical disease remission at 12 months, the lateral tendon compartment (LTC) was the tendon site more frequently affected by pathology (75.0%). Patients with US-detected tenosynovitis that did not achieve clinical disease remission at follow-up had the highest frequency of tendon pathology on US in the medial tendon compartment (MTC) (83.3%). The anterior tendon compartment was the less frequently affected tendon compartment of the ankle in all patients (33.3% in both patients with and without clinical remission of disease at the 12-months follow-up visit).Conclusion:US-detected tenosynovitis of the ankle is a common finding in patients with new-onset JIA with clinically ankle disease activity and is more frequent than the detection on US of isolated arthritis. The MTC and LTC are the tendon compartments more commonly affected on US. The detection on US of tenosynovitis at disease onset in ankles with clinical disease activity did not seem to affect the change to achieve the overall clinical disease remission compared to patients without tendon pathology but with joint disease in the ankle region.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 948.3-949
Author(s):  
S. Pastore ◽  
S. Della Paolera ◽  
A. Zabotti ◽  
A. Tommasini ◽  
A. Taddio

Background:Tenosynovitis can occur in patients with Juvenile Idiopathic Arthritis (JIA) and may be clinically difficult to distinguish from joint synovitis. The role of musculoskeletal ultrasound (msk-US) is still discussed in the management of JIA but recent studies supported the utility of msk-US especially in detection of tenosynovitis. There is no consensus treatment for tenosynovitis in children with JIA and almost all studies focused on tendon sheath injection with glucocorticoids.Objectives:The aims of the study were: i) to define the prevalence of tenosynovitis of the ankle in JIA patients and ankle swelling; ii) to describe the clinical characteristics of patient with tenosynovitis and to analyze different response to treatment.Methods:We conducted an observational cross-sectional study of a group of patients with JIA followed at the Rheumatology Service of the Maternal and Child Institute “Burlo Garofolo” of Trieste. We enrolled all the patients who reported a swelling of the ankle at least once during the follow-up period and, among these patients, we included only those who underwent msk-US. Based on both clinical and sonographic examination, we identified patients with tenosynovitis and we described their demographical and clinical characteristics as well as the therapeutic approach undertaken in this group of patients.Results:On December 31st 2019, 56 swollen ankles of 48 patients were assessed with msk-US: 22 ankles showed sonographic signs of synovitis (39%), 16 ankles of both synovitis and tenosynovitis (28%), 14 ankles of tenosynovitis only (25%). Overall, tenosynovitis was detected on twenty-seven (56%) out of 48 children with at least a swollen ankle. The majority of patient were females (70%) and the most affected tendon was the tibialis posterior (66%). Twenty-five patients with tenosynovitis (92%) achieved clinical and radiological remission: seven out of 26 patients (26%) treated with methotrexate achieved clinical and radiological remission without the addition of other therapies; fifteen out of seventeen patients (88%) treated with a biological drug responded to the therapy, of which eleven (73%) were in combination therapy with methotrexate.Conclusion:We observed that more than 50% of the patients with a swelling of the ankle presented a tenosynovitis and among these patients about 50% did not show sonographic sign of synovitis. Msk-US was decisive in order to identify tenosynovitis and to characterize ankle swelling in JIA patients. Among patients with tenosynovitis biological therapy alone or in association with immunomodulating therapy showed effectiveness in inducing disease remission.References:[1]Peters SE, Laxer RM et al. Ultrasound-guided steroid tendon sheath injections in juvenile idiopathic arthritis: a 10-year single-center retrospective study. Pediatr Rheumatol Online J. 2017 Apr 11;15(1):22.[2]Lanni S, Bovis F et al. Delineating the Application of Ultrasound in Detecting Synovial Abnormalities of the Subtalar Joint in Juvenile Idiopathic Arthritis. Arthritis Care Res (Hoboken). 2016 Sep;68(9):1346-53.[3]Cimaz R, Giani T et al. What is the real role of ultrasound in the management of juvenile idiopathic arthritis? Ann Rheum Dis. 2020 Apr;79(4):437-439. doi: 10.1136/annrheumdis-2019-216358[4]Lanni S, Marafon DP et al. Comparison between clinical and ultrasound assessment of the ankle region in juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2020 Apr 27.[5]Laurell L, Court-Payen M et al. Ultrasonography and color Doppler in juvenile idiopathic arthritis: diagnosis and follow-up of ultrasound-guided steroid injection in the ankle region. A descriptive interventional study. Pediatr Rheumatol Online J. 2011 Jan 29;9(1):4.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 941.1-941
Author(s):  
L. Marelli ◽  
F. Minoia ◽  
G. Beretta ◽  
C. Mapelli ◽  
G. Leone ◽  
...  

Background:Uveitis is the main extraarticular complication of juvenile idiopathic arthritis (JIA) with still a significant impact on JIA morbidity, despite continuous improvement in systemic treatment. Although antinuclear antibody positivity and early onset of JIA have been associated with a high risk of uveitis onset, so far no clinical features have been widely recognized as predictive factors for JIA-associated uveitis (JIA-U) response to treatment.Objectives:To investigate clinical features associated with response to systemic treatment in a long-term cohort of patients with JIA-UMethods:Clinical records of patients with JIA-U were retrospectively reviewed with regard to clinical features, therapeutic choices and outcome. Clinical and laboratory variables were compared by means of Mann-Whitney U test or chi-square/Fisher exact test, as appropriate.Results:Data from 164 JIA-U patients were analysed (81.7% female), with a median follow up of 12.1 years (7.1-17.3). Median age at JIA and uveitis onset was 2.6 (1.6-4.8) and 4.8 (2.9 – 7.0) years, respectively. Monotherapy with a conventional disease-modifying antirheumatic drug (DMARD) was used in 25.0% of patients, while 111 patients (67.7%) received at least one biologic DMARD (bDMARDs). Compared to patients responsive to DMARDs, children requiring a bDMARDs for uveitis had a lower median age at both JIA (2.4 vs 4.3 years, p 0.0234) and uveitis onset (4.1 vs 6.2 years, p 0.0023). Despite no differences in ocular damage at onset and median disease duration, patients not responsive to conventional DMARDs showed a higher frequency of ocular damage at the last visit (66.2% vs 33.3%, p 0.011). Children requiring more than one bDMARD for uveitis presented a more frequent polyarticular course (87.0% vs 20.2%, p 0.0022), a longer disease duration (median follow-up: 14.2 vs 10.4 years, p 0.0397) and a higher frequency of visual loss (best corrected visual acuity < 4/10: 23.3% vs 6.3%, p 0.0069).Conclusion:JIA-U patients with a lack of response to conventional DMARDs were significantly younger both at JIA and uveitis onset. Severe JIA-U requiring more than one bDMARDs was associated with polyarticular JIA course and longer disease duration. Children resistant to conventional treatment need prompt recognition and additional strategies to improve long-term outcome.References:[1]Heiligenhaus et al. Predictive factors and biomarkers for the 2-year outcome of uveitis in juvenile idiopathic arthritis. Rheumatology 2019.Disclosure of Interests:None declared


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Ellen Dalen Arnstad ◽  
◽  
Mia Glerup ◽  
Veronika Rypdal ◽  
Suvi Peltoniemi ◽  
...  

Abstract Background To study fatigue in young adults with juvenile idiopathic arthritis (JIA) 18 years after disease onset, and to compare with controls. Methods Consecutive children with onset of JIA between 1997 and 2000, from geographically defined areas of Norway, Sweden, Denmark and Finland were followed for 18 years in a close to population-based prospective cohort study. Clinical features, demographic and patient-reported data were collected. Inclusion criteria in the present study were a baseline visit 6 months after disease onset, followed by an 18-year follow-up with available self-reported fatigue score (Fatigue Severity Scale (FSS), 1–7). Severe fatigue was defined as FSS ≥4. For comparison, Norwegian age and sex matched controls were used. Results Among 377 young adults with JIA, 26% reported severe fatigue, compared to 12% among controls. We found higher burden of fatigue among participants with sleep problems, pain, poor health, reduced participation in school/work, physical disability, active disease, or use of disease-modifying anti-rheumatic drugs (DMARDs)/biologics/systemic steroids. In contrast, participants without these challenges, had fatigue scores similar to controls. Active disease assessed at all three time points (baseline, 8-year and 18-year follow-up) was associated with higher mean fatigue score and higher percentage of severe fatigue compared to disease courses characterized by periods of inactive disease. Predictors of fatigue at the 18-year follow-up were female sex and diagnostic delay of ≥6 months at baseline, and also pain, self-reported poor health, active disease, and previous/ongoing use of DMARDs/biologics at 8 years. Conclusions Fatigue is a prominent symptom in young adults with JIA, with higher fatigue burden among participants with poor sleep, pain, self-reported health problems, active disease, or use of DMARDs/biologics. Participants without these challenges have results similar to controls. Patient- and physician-reported variables at baseline and during disease course predicted fatigue at 18-year follow-up.


2020 ◽  
Author(s):  
Rana Masoud ◽  
Wajiha Jeelani ◽  
Barbine Tchamba Agbor Agbor ◽  
Teresa Hennon ◽  
Brian H Wrotniak ◽  
...  

Abstract Background: Oligo-articular juvenile idiopathic arthritis (Oligo JIA) is the most common subtype of juvenile idiopathic arthritis. Intra-articular corticosteroid (IAC) injection is a mainstay treatment of oligo JIA providing pain relief, improving mobility and preventing further joint destruction in the majority of patients. In 2015, production of triamcinolone hexacetonide (TH) an intra-articular corticosteroid was discontinued in the United States leading to use of triamcinolone acetonide (TA) as an alternative. In this study, we compared response to treatment in children with oligo JIA who underwent therapy with intra-articular TA and TH injection. Methods: Our study is a retrospective chart review of children with oligo JIA who were treated with IAC injections with TH between January 2012 and June 2015 and TA between J uly 2015 and December 2018. The two groups were followed at John R. Oishei Children’s Hospital of Buffalo and were evaluated for response to treatment, side effects and predictors of response including duration of disease before treatment, erythrocyte sedimentation rate (ESR), and c-reactive protein (CRP). Response to treatment was defined as at least 6 months follow up without evidence of active arthritis in injected joints. Patients were considered to be non-responders if they continued to show active arthritis during their first follow up after joint injection. The primary objective was to evaluate whether there was a significant difference in rate of response between TH and TA. Results: Forty-nine patients, 38 female and 11 male with oligo JIA were included in the study. The average age was 6.7 years. A total of 111 joints were injected includin g 78 knees, 13 ankles, 9 wrists, 4 hips, 4 elbows, 2 TMJ and one subtalar joint. In the TA group, 49 % (29/59) did not show response to injection compared to 27 % (14/52) in the TH group. After 6 months, response rates were better for individuals injected with TH compared to TA (73 % vs. 51%). In general, response to intra-articular TH was superior to TA with P = .016 using chi-square test of independence. This difference in outcome was not influenced by other variables such as duration of illness before treatment (P value 0.784) or elevated ESR and CRP. No difference in side effects between the two groups were noted. Conclusion: Our results in conjunction with prior published data suggests that TH intra-articular joint injection in oligo JIA is superior to TA, although future controlled trials are necessary for confirmation. An effective, long lasting treatment can have a great impact on the outcome of these children.


2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Mia Glerup ◽  
◽  
Steffen Thiel ◽  
Veronika Rypdal ◽  
Ellen Dalen Arnstad ◽  
...  

Abstract Background To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from a population-based Nordic juvenile idiopathic arthritis (JIA) cohort. Additionally, to assess the predictive value of lectin pathway proteins with respect to remission status. Methods A population-based cohort study of consecutive cases of JIA with a disease onset from 1997 to 2000 from defined geographical areas of Finland, Sweden, Norway and Denmark with 17 years of follow-up was performed. Clinical characteristics were registered and H-ficolin, M-ficolin, MASP-1, MASP-3, MBL and CL-K1 levels in serum were analyzed. Results In total, 293 patients with JIA were included (mean age 23.7 ± 4.4 years; mean follow-up 17.2 ± 1.7 years). Concentrations of the lectin protein levels in serum were higher at baseline compared to the levels 17 years after disease onset (p ≤ 0.006, n = 164). At baseline, the highest level of M-ficolin was observed in systemic JIA. Further, high M-ficolin levels at baseline and at 17-year follow-up were correlated to high levels of ESR. In contrast, high MASP-1 and MASP-3 tended to correlate to low ESR. CL-K1 showed a negative correlation to JADAS71 at baseline. None of the protein levels had prognostic abilities for remission status 17 years after disease onset. Conclusion We hypothesize that increased serum M-ficolin levels are associated with higher disease activity in JIA and further, the results indicate that MASP-1, MASP-3 and CL-K1 are markers of inflammation.


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