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  • Meeting abstracts
  • Open Access

Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part two

Genoa, Italy. 28 September – 01 October 2016
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Pediatric Rheumatology201715 (Suppl 1) :44

https://doi.org/10.1186/s12969-017-0142-8

  • Published:

P178 Features of drug therapy of patients with systemic juvenile idiopathic arthritis, according to the Russian register of the Russian union of pediatricians

Olga Lomakina1, Ekaterina Alekseeva1, Sania Valieva1, Tatiana Bzarova1, Irina Nikishina2, Elena Zholobova3, Svetlana Rodionovskaya2, Maria Kaleda2

1Rheumatology, Scientific Center of Children’s Health, Moscow, Russian Federation; 2Rheumatology, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation; 3Rheumatology, Sechenov First Moscow State Medical University, Moscow, Russian Federation
Presenting author: Olga Lomakina

Introduction: Systemic juvenile arthritis - a rare chronic disease. Register - it's an important tool to monitor the effectiveness and safety of GIBP.

Objectives: Our aim was to study features of the drug therapy of children with systemic juvenile idiopathic arthritis (sJIA)

Methods: We conducted a retrospective data analysis included in the Register of sJIA cases, for the period from 2002 to 2015

Results: The indicators of 384 children with sJIA are studied. Prior to the diagnosis verification, all patients were prescribed to intake antipyretic agents, 98% —antibiotics. After the diagnosis, non-steroidal anti-inflammatory drugs (NSAIDs) were intaken by 282 (73.4%) patients: diclofenac sodium — by 163 (40.1%), nimesulide — by 88 (22.9%) patients. The average duration of NSAID intake from 2002 to 2015 decreased from 81.5 ± 115.3 to 3.3 ± 3.7 months (p < 0.001). Prior to the diagnosis verification, glucocorticoids were received intravenously or intramuscularly by 265 (69.0%) patients, orally — 176 (45.8%). Totally, glucocorticoids were received by 330 (85.9%) patients: methylprednisolone — 300 of 384 (78.1%), prednisolone — 174 (45.3%), there were totally 1855 prescriptions in 668 cases. The average duration of glucocorticoid intake from 2002 to 2015 decreased from 13.7 ± 26.7 to 5.0 ± 3,8 months (p < 0.001). As a disease-modifying drug, methotrexate was intaken by 237 (61.7%), Cyclosporin — by 193 (50.6%) patients. There were totally 809 cases of genetically engineered biological preparations (GIBP) in 430 patients: in 2002–2005–8, in 2011–2015–602 in 397 cases (p = 0.001). Tocilizumab is intaken by 210 (52.9%) of 397 patients, kanakinumab — 37 (9.3%) patients. The disease duration from the manifestation to the prescription of immunosuppressive drugs from 2002 to 2015 decreased from 27.3 ± 23.9 to1.0 ± 0 months (p < 0.001), GIBP prescriptions — from 70.7 ± 26.3 to 0.5 ± 0.7 months, respectively (p < 0.001)

Conclusion: In 13 years there have been positive changes in the antirheumatic therapy in children with sJIA — the duration of NSAIDs and glucocorticoids intake reduced, the period between diagnosis verification and immunosuppressants and GIBP prescription decreased. However, it is still widely used antibiotics, non-selective NSAIDs and glucocorticoids.

Disclosure of Interest

None Declared

P179 Bicipital synovial cyst associated with systemic juvenile idiopathic arthritis: clinical description, sonographic and pathological findings

Yasuo Nakagishi1, Masaki Shimizu2, Mao Mizuta2, Akihiro Yachie2

1Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan; 2Department of Pediatrics, School of Medicine, Institute of Medical Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan
Presenting author: Yasuo Nakagishi

Introduction: Bicipital synovial cyst is a rare manifestation of systemic juvenile idiopathic arthritis (s-JIA). It remains unclear how bicipital synovial cysts arise.

Objectives: To describe the presentation and clinical course of bicipital synovial cysts in 2 patients with s-JIA and to assess how bicipital synovial cysts arise.

Methods: We report 2 patients with bicipital synovial cyst associated with s-JIA. We performed sonographic examinations of bicipital synovial cyst. Furthermore, we investigated pathological examination using biopsy specimen.

Results: Patient 1: Eight-year-old boy was diagnosed as s-JIA at the age of 4. His disease course was steroid-dependent and tocilizumab (TCZ) was started from the age of 5. However, his disease relapsed at the age of 7. He presented with fever and swelling of upper left arm. USG revealed a hyperechogenic cyst along the margin of the biceps muscle. The biopsy of the cyst revealed cyst was surrounded by granulation tissue with abundant macrophages infiltrate and there were no synovial tissues. The cyst disappeared with control of disease activity.

Patient 2: Twelve-year-old boy was diagnosed as s-JIA at the age of 8. His disease course was steroid-dependent and tocilizumab (TCZ) was started from the age of 12. At the time to start TCZ, he presented with swelling of upper right arm. Ultrasonography revealed a hyperechogenic cyst in the fascia of biceps muscle. The biopsy of the cyst revealed cyst was surrounded by granulation and fibrous tissue with abundant macrophages, lymphocytes and neutrophils infiltrate. There were no synovial tissues. The cyst disappeared with control of disease activity.

Conclusion: These findings indicate bicipital synovial cysts arise as follows: the fluid arises within the shoulder joint and then descends into the contiguous bicipital tendon sheath. The tendon eventually ruptures, leading to collection of fluid in the bicipital area and synovial cysts arise from the biceps muscle fasciitis. Bicipital synovial cyst is a rare manifestation of s-JIA but synovial cyst should be considered in the differential diagnosis in all children with s-JIA presenting with swelling of the upper arm.

Disclosure of Interest

None Declared

P180 Systemic juvenile idiopathic arthritis with MEFV gene mutations may have a good response to colchicine: suggestion from 5 cases in our center

Yuko Sugita, Nami Okamoto, Kousuke Shabana, Takuji Murata, Hiroshi Tamai

Osaka Medical and Pharmaceutical University, Takatsuki-city, Japan
Presenting author: Yuko Sugita

Introduction: Systemic juvenile idiopathic arthritis (SJIA) is considered to be an autoinflammatory disease in which the dysregulation of innate immune response is suggested to be the main pathogenesis, and there are some reports that show SJIA patients have a significantly higher frequency of MEFV gene mutations, which is associated to the activation of IL-1β pathway, than healthy control population1,2.

Objectives: Since the effectiveness of colchicine, which is useful in familial Mediterranean fever, in SJIA patients with MEFV gene mutations is not established, we sought to examine whether colchicine is effective in such patients.

Methods: We searched for gene mutations that is responsible for autoinflammatory disease in SJIA patients who had persistent clinical symptoms (e.g., skin rashes and arthritis) or flare even under treatment with glucocorticoid, disease-modifying antirheumatic drugs (DMARDS), and IL-6 receptor inhibitor (tocilizumab). We obtained informed consent from the patient or their caregivers, and the gene mutation analysis was performed at Medicine Department of Pediatrics, Kyoto University (Dr. Ryuta Nishikomori). Patients with MEFV gene mutations were administered colchicine.

Results: The five SJIA patients with MEFV gene mutations are summarized in Table 1. All the cases required treatment with IL-6 receptor inhibitor. Case 1 had multiple gene mutations besides MEFV gene such as NOD2, PSTPIP1, and NLRP12 which is known to be associated with other autoinflammatory diseases. Case 2 once achieved drug free remission, but had a flare and started colchicine during the flare which was not effective to control the activated disease. Case 3 and 4 had recurrent diseases but became stable after the initiation of colchicine administration. In case 5, colchicine was administered after the identification of MEFV gene mutation, and was effective to urticarial rashes. Case 3, 4, and 5 is now stable without glucocorticoid after the initiation of colchicine.

Conclusion: To SJIA patients with MEFV gene mutations who are resistant to standard treatment such as glucocorticoid, DMARDS, and biologic agents, colchicine may be a useful therapeutic options.

References

1. N. A. Ayaz, S. Özen, Y. Bilginer, M. Ergüven, E. Taşkıran, E. Yılmaz, et al. MEFV mutations in systemic onset juvenile idiopathic arthritis. Rheumatology 2009;48:23–25.

2. Hala M. Lotfy, Manal E. Kandil, Marianne Samir Makboul Issac, Samia Salah, Nagwa Abdallah Ismail, Mohamed A. Abdel Mawla. MEFV Mutations in Egyptian Children with Systemic-Onset Juvenile Idiopathic Arthritis. Molecular Diagnosis & Therapy. 2014; 18(5): 549-557.

Disclosure of Interest

None Declared.
Table 1 (abstract P180).

Summary of the five SJIA patients with MEFV gene mutations

 

Sex

Age of onset

MEFV mutations

recurrent disease

Effectiveness of colchicine

Other treatments

case 1

F

6

L110P, E148Q

+

+

TCZ, TAC, PSL

case 2

F

13

L110P, E148Q, P369S, R408Q

+

TCZ, CyA, PSL

case 3

F

3

L110P, E148Q, G304R

+

+

TCZ

case 4

F

7

G304R

+

+

TCZ

case 5

M

2

E148Q

+

TCZ

F female, M male, TCZ tocilizumab, TAC tacrolimus, PSL prednisolone, CyA Cyclosporin A

Poster Session: Systemic lupus erythematosus and antiphospholipid syndrome I

P181 Predictors of recovery from lupus nephritis in children

Eve M. Smith1, Peng Yin2, Andrea L. Jorgensen2, Michael W. Beresford1,3 on behalf of On behalf of the UK JSLE Cohort Study

1Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK ; 2Department of Biostatistics, Institute of Translational Medicine, University of Liverpool, Liverpool, UK; 3Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK
Presenting author: Eve M. Smith

Introduction: Within an adult-onset Systemic Lupus Erytematosus (SLE) context, proteinuria has been shown to take a significant period of time to normalise, with 53% of lupus nephritis (LN) patients requiring up to 2 years to recover, and 74% recovering by 5 years [1]. The recovery time from proteinuria in Juvenile-onset SLE (JSLE) has not been well described.

Objectives: 1) To describe time to recovery from proteinuria, and to elucidate if clinical/demographic factors at LN onset differentiate patients who do not fully recover. 2) To determine factors at LN onset which influence time to proteinuria recovery.

Methods: Participants of the UK JSLE Cohort Study, between 1995-2015, were included if they had biopsy defined LN or active LN based upon the renal domain of the BILAG score (A/B) AND proteinuria of >50 mg/mmol. Univariate logistic regression modelling compared clinical/demographic factors at the time of LN onset in patients who did/did-not recover from proteinuria during the follow-up period. Covariates with p-value <0.2 were included in a multivariable logistic regression model, and backward stepwise variable selection applied. Univariate Cox proportional hazard (Cox PH) regression modelling was used to explore factors associated with time to proteinuria recovery, followed by the same multivariable model selection procedure.

Results: 64/350 (18%) JSLE patients fulfilled the inclusion criteria. 25 (39%) recovered from proteinuria within a median of 17 months (min 2.4, max 78). The remaining 39 (61%) had not recovered after a median of 22 months (min 2.3, max 132). The final multivariable logistic regression model showed ethnicity, eGFR, Azathioprine and cardiorespiratory or haematological involvement at time of LN onset to be significant factors differentiating patients who did/did not recover (see Table 2, section A). Using Cox PH regression modelling, age, eGFR and haematological involvement were found to be significantly associated with time to proteinuria recovery (see Table 2, section B).

Conclusion: A significant proportion of children with LN have on-going proteinuria after 2 years. Poor prognostic factors include ethnicity, young age, low GFR, azathioprine use and concomitant haematological involvement. Consideration of such factors may help to improve LN outcomes.

Reference

1. Touma, Zahi, Urowitz, Murray B, Ibanez, Dominique, Gladman, Dafna D. Time to Recovery From Proteinuria in Lupus Nephritis Patients Receiving Standard of Care Treatment. Arthritis Rheum 2011;63 Suppl 10 :599 DOI: 10.3899/jrheum.130005.

Disclosure of Interest

None Declared.
Table 2 (abstract P181).

See text for description

A) Factors differentiating those who do / do not recover during follow up

Odds ratio (95% CI)

p-value

Interpretation

Ethnicity

(Caucasian vs non-Caucasian)

14.19 (2.52, 122.63)

0.007

Non-Caucasians – less likely to recover from proteinuria

eGFR

1.04 (1.02, 1.08)

0.007

Low eGFR at proteinuria onset - less likely to recover

Azathioprine use

0.093 (0.01, 0.78)

0.044

Use of Azathioprine at proteinuria onset – less likely to recover

Haematological involvement

0.13 (0.03, 0.53)

0.007

Haematological involvement – less likely to recover

Cardiorespiratory involvement

11.22 (1.57, 107.54)

0.022

Cardiorespiratory involvement - more likely to recover

B) Factors influencing time to recovery from proteinuria

HR (95% CI)

p-value

 

Age (years)

1.38 (1.1, 1.8)

0.007

Younger patients - less likely to recover

eGFR

1.0 (1.0, 1.1)

0.036

Lower eGFR - less likely to recover

Haematological involvement

0.3 (0.1, 0.8)

0.016

Haematological involvement - less likely to recover

P182 Are urine biomarkers able to predict changes in lupus nephritis disease activity over time? A Markov state-space model of lupus nephritis urine biomarker dynamics

Eve M. Smith1, Antonio Eleuteri1, Beatrice Goilav2, Laura Lewandowski3, Angel Phuti4, Dawn Wahezi2, Tamar Rubinstein2, Caroline Jones5, Paul Newland5, Stephen Marks6, Rachel Corkhill1, Diana Ekdawy1, Clarissa Pilkington6, Kjell Tullus6, Chaim Putterman7, Chris Scott8, Antony C. Fisher1, Michael W. Beresford1,9

1University of Liverpool, Liverpool, UK, 2Albert Einstein College of Medicine, New York, USA, 3National Institute of Health, Maryland, USA, 4University of Cape Town, Cape Town, South Africa, 5Alder Hey Children’s NHS Foundation Trust, Liverpool, UK, 6Great Ormond Street Hospital, London, UK, 7Albert Einstein College of Medicine, New York, UK, 8 University of Cape Town, Cape Town, South Africa, 9Alder Hey Children's NHS Foundation Trust, Liverpool, UK
Presenting author: Eve M. Smith

Introduction: A urine ‘biomarker panel’ comprising alpha-1-acid glycoprotein (AGP), ceruloplasmin (CP), transferrin, vascular cell adhesion molecule-1 (VCAM-1), monocyte chemoattractant protein 1 (MCP-1) and lipocalin like prostaglandin D synthase (LPGDS) has been shown to perform to an ‘excellent’ level cross-sectionally for identification of active Lupus Nephritis (LN). The ability of this panel to predict LN flare and remission warrants further investigation.

Objectives: To model urine biomarker dynamics in LN, assessing ability to predict flare and remission.

Methods: The six novel urinary biomarkers were quantified by ELISA in participants of the UK JSLE Cohort Study (Cohort 1), the Einstein Lupus Cohort (Cohort 2), and the University of Cape Town Lupus Cohort (Cohort 3). Patients were categorised as having active LN (renal domain BILAG score of A, B or C & previous histological confirmation of LN, State 2) or inactive LN (renal domain BILAG score D or E, State 1). A baseline homogeneous Markov model of state transitions was fitted, quoting a corrected Akaike Information Criterion score (AICc). A lower AICc score suggested better model accuracy. Urine biomarkers were explored as factors predicting state transitions. Bayesian multiple imputation of missing variables was used.

Results: The study included 184 observations from 57 Cohort 1 patients, 27 from 13 Cohort 2 patients, and 33 from 10 Cohort 3 patients. Across the data set there were 10 transitions from inactive to active LN (1-2 transition), 18 from active to inactive LN (2-1 transition), with 93 and 43 remaining inactive and active respectively. A baseline homogeneous multi-state Markov model of LN activity was fitted, producing a AICc score of 149.0. Each urine biomarker was individually added to the model, identifying AGP and CP as covariates producing the lowest AICc’s. Addition of both biomarkers as predictors of all state transitions led to a worsening of AICc, 157.2. The confidence intervals of hazard ratios for CP on the 1-2 transition and for AGP on the 2-1 transition included the value 1. Re-fitting of the model, whereby AGP only had an effect on 1-2 transitions, and CP on 2-1 transitions, produced a lower AICc, 135.0, favouring this model (see Table 3). Inputting individual patient AGP/CP values to the model can provide 3, 6 and 12 month probabilities of state transition.

Conclusion: Within an internationally derived Markov state-space model of LN urine biomarker disease dynamics, AGP was predictive of active LN flare or remaining active, whereas CP was predictive of remission or remaining in-active. To improve patient outcomes, this model must be tested in a larger, prospective, rigorously conducted clinical trial of biomarker led LN monitoring.

Disclosure of Interest

None Declared.
Table 3 (abstract P182).

See text for description

Model characteristics

LN state transition

Baseline

Log AGP

Log CP

AICc

Hazard ratios for changing state for each covariate and 95% confidence intervals

AGP and CP as predictors of all LN state transitions

1-2

0.25 (0.03, 1.89)

1.80 (1.08, 3.01)

0.46 (0.17,1.28)

157.2

2-1

0.19 (0.03, 1.41)

1.22 (0.78, 1.91)

0.33 (0.18, 0.62)

AGP as predictor of 1-2 transition & CP for 2-1

1-2

0.59 (0.24, 1.45)

1.49 (1.10, 2.02)

-

135.0

2-1

2.11 (0.90, 4.94)

-

0.60 (0.39, 0.93)

P183 Growing international evidence for urine biomarker panels identifying lupus nephritis in children – verification from the South African Western Cape lupus cohort

Eve M. Smith1, Laura Lewandowski2, Angel Phuti3, Andrea Jorgensen4, Chris Scott5, Michael W. Beresford6

1Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK; 2Pediatric Rheumatology, National Institute of Health, Maryland, USA; 3Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa; 4Department of Biostatistics, Institute of Translational Medicine, University of Liverpool, Liverpool, UK; 5Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa; 6Department of Women’s & Children’s Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK
Presenting author: Eve M. Smith

Introduction: The Paediatric Lupus Erythematosus in South Africa (SA) Cohort has collected clinical data in the Western Cape since 2013. They have demonstrated that their patients often initially present with severe lupus nephritis (LN). Monitoring of such patients can be problematic due to geographical, economic and social constraints faced by families. A range of urine biomarkers for LN have been explored within the UK JSLE Cohort study, with a panel of four (alpha-1-acid glycoprotein (AGP), lipocalin like prostaglandin D synthase (LPGDS), transferrin and ceruloplasmin) demonstrating an ‘excellent’ ability to identify active LN.

Objectives: To assess whether the same/different biomarker combinations are of importance for identifying active LN in the Paediatric Lupus Erythematosus in SA Cohort.

Methods: Participants of the Paediatric Lupus Erythematosus in SA Cohort attending Red Cross Memorial and Groote Schuur Hospital Hospitals, Cape Town, aged <18 years at diagnosis, were recruited between January 2015-16. Healthy controls (HC’s) with non-inflammatory non-infective diagnoses were also recruited. Patients were categorised as having active LN (renal domain of the BILAG score of A/B & previous histological confirmation of LN) or in-active LN (renal domain of the BILAG score of D/E). Novel urinary biomarkers; AGP, LPGDS, transferrin, ceruloplasmin, vascular cell adhesion molecule-1 (VCAM-1), and monocyte chemoattractant protein 1 (MCP-1) were quantified by ELISA. Measurements were standardized to urinary creatinine. The Mann Whitney-U test was used to compare biomarker levels between groups. Binary logistic regression modeling and receiver operating curve analysis was used to assess combinations of biomarkers for active LN identification.

Results: 23 JSLE patients (20 females, 3 males) with a median age of 13.5 years [IQR 12.7-14.9] and disease duration of 2.6 years [IQR 1.8-4.0] were included. 18 HC’s (14 females, 4 males) had a median age of 11 years [IQR 10-12]. All novel urine biomarkers were significantly higher in active than in-active LN patients (corrected p-values, p c all <0.01), with no difference seen between in-active LN patients and HC’s (all p c = 1.0, see Table below). Inclusion of all novel biomarkers in a binary logistic regression model and application of step AIC function to get a final model identified VCAM-1, MCP-1 and LPGDS to be the best combination of biomarkers identifying active LN within the SA cohort, with a combined area under the curve (AUC) of 0.96.

Conclusion: This is the first study to look at urine biomarkers in an African JSLE population, highlighting their potential utility in this population. The optimal panel of biomarkers may differ to that of the UK, but requires further investigation in a prospective longitudinal study given the samples size. In SA, it is anticipated that use of a point of care testing device to monitor LN activity in the home or local clinic could help to improve patient monitoring, treatment and outcomes.

Disclosure of Interest

None Declared.
Table 4 (abstract P183).

See text for description

Marker

(ngmgCr)

Active LN

[med, IQR], n = 9

Inactive LN

[med, IQR], n = 14

HC

[med, IQR], n = 18

Act vs Inact (p c )

Inact vs HC (p c )

VCAM-1

59 [42-119]

4 [2-11]

4 [2-6]

0.0003

1.0

MCP-1 (pgmgCr)

1020 [410-3642]

219 [150-334]

296 [186-448]

0.0258

1.0

LPGDS

2683 [1640-3602]

601 [151-900]

577 [314-765]

0.0054

1.0

AGP

145435 [54746-250367]

680 [394-2985]

605 [408-1458]

0.0002

1.0

CP

51714 [34861-177503]

1901 [1140-3276]

1700 [1324-2999]

0.00003

1.0

TF

63630 [38071-156026]

433 [221-1020]

425 [234-928]

0.00003

1.0

P184 Whole exome sequencing in early onset systemic lupus erythematosus

Ezgi Deniz Batu1, Can Kosukcu2, Ekim Taskiran2, Sema Akman3, Kubra Ozturk4, Betul Sozeri5, Erbil Unsal6, Zelal Ekinci4, Yelda Bilginer1, Mehmet Alikasifoglu2, Seza Ozen1

1Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 2Department of Medical Genetics, Hacettepe University Faculty of Medicine, Ankara, Turkey; 3Department of Pediatrics, Division of Nephrology-Rheumatology, Akdeniz University Faculty of Medicine, Antalya, Turkey; 4Department of Pediatrics, Division of Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey; 5Department of Pediatrics, Division of Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey; 6Department of Pediatrics, Division of Rheumatology, Dokuz Eylul University Faculty of Medicine, İzmir, Turkey
Presenting author: Ezgi Deniz Batu

Introduction: Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder with different genetic and environmental factors playing role in its pathogenesis. Early onset SLE, familial SLE, and syndromic SLE are rare situations which may lead to identification of monogenic defects responsible for the disease. İdentification of monogenic causes through these cases can help us to understand the pathogenic mechanisms in SLE.

Objectives: We aimed to discover monogenic defects causing SLE by performing whole exome sequencing (WES) in familial or early-onset SLE cases.

Methods: We enrolled 12 pediatric SLE cases (from 7 different families) who had disease onset before 5 years of age and a family history consistent with an autosomal recessive inheritance (affected siblings or parenteral consanguinity). Whole exome sequencing and bioinformatic analyses were performed in six index cases and the suspected mutations were confirmed by Sanger sequencing. Only C1Q gene was analyzed in patient 4 since he had similar features with the first three cases.

Results: There was consanguinity in all families. The characteristics of index SLE cases are presented in Table 5. We have demonstrated a homozygous nonsense mutation (c.622C > T/p.Gln208Ter) in C1QA gene in two patients; homozygous nonsense mutation (c.79C > T/p.Gln27Ter) in C1QC gene in one; homozygous missense mutation (c.100G > A/p.Gly34Arg) in C1QC gene in one; homozygous stop codon mutation (c.1945G > C/p.Ala649Pro) in C1S gene in one; homozygous frameshift mutation (c.290_291delCA/p.Thr97Ilefs*2) in DNASE1L3 gene in one patient. There was a candidate novel gene in one patient and functional studies on this gene are ongoing.

Conclusion: Five of our patients had homozygous mutations in the genes coding for early complement proteins. The risk to develop pediatric SLE is estimated to be 93% for C1q and 66% for C1s/r. There are less than 90 published cases with homozygous C1q deficiency. The clinical presentations are variable; however, they usually had cutaneous involvement, normal C3, C4 levels and negative anti-dsDNA which was the case in our patients. C1s deficiency is much rarer. The nonsense mutation in C1S gene of our patients was novel. DNASE1L3 gene encodes for DNase1 enzyme which functions as an endonuclease cleaving DNA. Deletion in DNASE1L3 gene has been previously reported to be associated with SLE in the study on seven SLE families where it was shown that protein encoded by the mutant DNASE1L3 completely lacked DNase activity. The variant in DNASE1L3 gene detected in our patient was the same as the one reported. We suggest that monogenic causes/associations should be sought for an early-onset SLE.

Disclosure of Interest

None Declared.
Table 5 (abstract P184).

Characteristics of pediatric patients with early-onset systemic lupus erythematosus

 

Pt 1

Pt 2

Pt 3

Pt 4

Pt 5

Pt 6

Pt 7

SLE in sibling

+

+

-

-

+

-

+

Mutant genes

C1QA

C1QC

C1QC

C1QA

C1S

DNASE1L3

Novel gene (?)

Malar/discoid rash/photosensitivity

+

+

+

+

+

+

-

Oral ulcers

+

+

+

-

+

-

-

Arthritis

+

+

-

-

+

+

-

Nephritis

-

-

-

-

+

+

-

Hematologic involvement

+

+

+

-

+

+

-

Decrease in C3, C4

-

-

-

-

+

+

+

Autoantibodies*

Anti-SM

RF

Anti-SM, anti-SSA, LA

Anti-SM, U1RNP

AntidsDNA, Anti-SSA, U1RNP, anti-histon

AntidsDNA, Anti-CL

AntidsDNA, DC

Pt patients, SLE systemic lupus erythematosus, CL cardiolipin, LA lupus anticoagulant, RF rheumatoid factor

* ANA was positive in all patients

P185 Fatigue in JSLE: analysis of prevalence and associations in a large, national cohort of patients

Hanna Lythgoe1,2, Michael W. Beresford2

1Institute of Translational Medicine, University of Liverpool, Liverpool, UK; 2NIHR Alder Hey Clinical Research Facility, Alder Hey Children's NHS Foundation Trust, Liverpool, UK
Presenting author: Hanna Lythgoe

Introduction: Fatigue is widely recognised as a common, debilitating symptom for patients with systemic lupus erythematosus (SLE) with significant impact on health-related quality of life. The cause of fatigue in SLE patients is poorly understood and likely to be multifactorial. Evidence is conflicting regarding whether fatigue in SLE patients is associated with factors such as disease activity, pain and mood and there are very few studies of fatigue in children with JSLE.

Objectives: To define the prevalence of fatigue and its associations within a large, national cohort of children with JSLE.

Methods: Patients meeting ≥4 ACR criteria in the UK JSLE Cohort Study were included. Data from the paediatric BILAG (p-BILAG) (completed by clinician), Short Form-36 (SF36) (completed by patient) and the Childhood Health Assessment Questionnaire (CHAQ) was analysed. The SF36 includes a vitality domain measuring energy/fatigue which cumulates in a score from 0–100 where higher scores indicate less fatigue; following review of published normative data we used a conservative estimate of 50 as a cut-off for significant fatigue. Associations between variables was assessed using Spearman’s rank correlation co-efficients. Correlation co-efficients of 0-0.19, 0.2-0.39,0.4-0.59, 0.6-0.79, 0.8-1 were considered as very weak, weak, moderate, strong and very strong respectively.

Results: 350 patients were included, for whom there were 1428 and 355 completed p-BILAGs and SF36s respectively. 81% of patients had suffered with fatigue at some point during their disease course, with patients suffering some degree of fatigue on 42% of all pBILAG scores.

Correlation was moderate to strong between fatigue and disease activity where disease activity is recorded by the patient or physician but was weak/very weak when disease activity is measured by ESR as a biochemical indicator of disease activity (Table 6). Moderate to strong associations are seen when comparing fatigue with pain and physical function. Strong correlations are seen on all correlations between patient-reported fatigue and patient-reported disease activity, pain and physical function. Weak/very weak correlations are seen when comparing fatigue with mood and anaemia.

Data from the SF36 vitality scores had a mean score of 56.7 (SD 25.5) which is lower than published normative mean scores in young adults aged 16–19 years (mean 69.5, SD 19.8) with a mean difference of 12.8 (confidence interval 9.8–15.8). Almost half (45.3%) of patients had an SF36 score of less than 50 during their disease course, indicating significant fatigue.

Conclusion: Fatigue affects most patients with JSLE and almost half suffer with significant fatigue. Fatigue is associated with increased disease activity, pain and functional disability. Importantly, the strongest associations are seen when these are measured using patient-reported outcomes. These associations need further evaluation of their causality in order to consider future treatment strategies for this resistant and problematic symptom.

Disclosure of Interest

None Declared.
Table 6 (abstract P185).

Associations of BILAG fatigue scores and SF36 vitality scores

 

Spearman’s rank correlation coefficients

Assessed association

Measure used as comparator

BILAG fatigue

SF36 vitality

Disease activity

Physician global activity VAS score

0.53

-0.5

Patient global VAS score

0.48

-0.72

ESR

0.25

-0.12

Pain

SF36 bodily pain

-0.45

0.69

Physical function

CHAQ

0.45

0.62

Anaemia

Haemoglobin

0.18

-0.21

Mood

Mood disorder, anxiety disorder or organic depressive illness on p-BILAG

0.17

-0.23

VAS visual analogue scale, ESR erythrocyte sedimentation rate. Strong correlations are shown in bold. All correlation co-efficients were statistically significant (P < 0.05)

P186 Evidence of altered blood brain barrier permeability in systemic lupus erythematosus using magnetic resonance imaging

Hermine I. Brunner1, Gaurav Gulati2, Jordan T. Jones3, Mekibib Altaye4, Jamie Eaton5, Mark Difrancesco6

1Pediatrics, Cincinnati Children's Hosptial Medical Center, Cincinnati, USA; 2Internal Medicine, University of Cincinnati, Cincinnati, USA; 3Pediatrics, University of Kansas, Kansas, USA; 4Pediatrics, Cinccinnati Children's Hospital Medical Center, Cincinnati, USA; 5Pediatrics, Cincinnati Children'sHosptial MEdical Center, Cincinnati, USA; 6Radiology, University of Cincinnati, Cincinnati, USA
Presenting author: Hermine I. Brunner

Introduction: Neurocognitive dysfunction is a common manifestation of childhood-onset Systemic Lupus Erythematosus (cSLE). Murine models suggest that loss of the blood-brain barrier (BBB) integrity allows brain-reactive proteins to enter the CNS and contribute to SLE-associated pathology. Contrast magnetic resonance imaging (MRI) can provide a measure of BBB integrity, but has risk associated with gadolinium use. We have previously identified multiple areas of gray matter (GM) loss on structural MRI in cSLE patients with neurocognitive deficits. Our aim was to evaluate safe, non-invasive MRI-methods of measuring regional BBB permeability and its relationship with neurocognitive function and regional GM volume in cSLE.

Objectives: To evaluate safe, non-invasive MRI-methods of measuring regional BBB permeability and its relationship with neurocognitive function and regional GM volume in cSLE.

Methods: Twelve cSLE patients and 12 healthy controls (age, gender, race and socioeconomic status matched) were enrolled. Those with diseases or medications (except prednisone) affecting neurocognitive function were excluded. Cognitive performance was assessed using the cSLE Neurocognitive Battery, which probes four cognitive domains: working memory, psychomotor speed, attention, and visuoconstructional ability. Performance in each of these was standardized and expressed as a Z-score. We almost concurrently performed arterial spin labeling (ASL) and diffusion-weighted imaging to measure regional BBB permeability. Voxel-based morphometric analysis was done to measure regional GM volume. Voxel-wise comparisons of capillary permeability were made between the cSLE and control groups. Correlation analysis was performed between regional BBB permeability and cognitive performance Z-scores, as well as local GM volume for the cSLE group.

Results: Among the cSLE patients (11 females, 7 African American, mean age 18 ± 6.8 years), 9 were treated with prednisone (median dose 5 mg/d). None was diagnosed with active neuropsychiatric SLE. Group comparison revealed clusters of voxels with significantly greater BBB permeability for cSLE patients than controls, in three regions: the parahippocampal gyrus, the right fusiform and inferior occipital region, and the caudate head. Correlations between BBB permeability and regional GM volume or overall and individual domain Z-scores for neurocognitive performance were not statistically significant, although locations of significant increases in permeability for cSLE closely match our previously identified areas of GM loss and functional changes associated with clinically overt neurocognitive impairment.

Conclusion: We present imaging evidence of altered regional BBB permeability in cSLE, using a novel non-invasive MRI technique. The absence of correlation with GM volume or cognitive performance Z-scores, yet similar location to GM loss in previous work in our cSLE cohort suggests that BBB breakdown may precede clinically overt neurocognitive impairment and brain tissue loss. Longitudinal studies are needed to confirm the change in GM volume in relation to BBB permeability over time.

Disclosure of Interest

None Declared.

P187 Imbalance of regulatory immunome influences disease activity of juvenile systemic lupus erythematosus

Joo Guan Yeo1,2, Jingyao Leong2, Loshinidevi D/O Thana Bathi2, Thaschawee Arkachaisri1,3, Salvatore Albani1,2,3

1Rheumatology and Immunology Service, Department of Paediatric Subspecialties, KK Women’s and Children’s Hospital, Singapore, Singapore; 2SingHealth Translational Immunology and Inflammation Centre, Singapore Health Services, Singapore, Singapore; 3Duke-National University of Singapore Medical School, Singapore, Singapore
Presenting author: Joo Guan Yeo

Introduction: The pathogenesis of Systemic Lupus Erythematosus (SLE) hinges on multiple disturbances that perturb the fine balance between immunity and regulation. Traditional investigational approaches have largely been focused on the pathogenic or protective role of individual cell types or even individual molecules. For a multifactorial disease like SLE, this mono-dimensional approach is inadequate. A holistic understanding of the immunome is a critical