AB1045 MULTICENTRIC CARPOTARSAL OSTEOLYSIS (MCTO) IN PRACTICE OF PEDIATRIC RHEUMATOLOGIST: DIFFERENTIAL DIAGNOSIS WITH JUVENILE IDIOPATHIC ARTHRITIS

Hepcidin is a 25-amino acid peptide, which is a key systemic regulator of iron metabolism. As considered, overproduction of hepcidin in the liver is controlled by high levels of proinflammatory cytokines. Is it known that interleukin-6 play a key role in the development of anemia in patients with juvenile idiopathic arthritis (JIA). However, IL-6 blockers itself may provoke the onset of Macrophage Activation Syndrome (MAS), manifesting, particularly, also with anemia. In view of the fact that macrophages involved in MAS express CD163 - a scavenger receptor that binds hemoglobin-haptoglobin complexes - and activate the pathways which are important for adaptation to oxidative stress induced by free iron, we can consider hepcidin as a welcome challenge to predict the development of MAS early in the course of the disease. During the study of 35 patients with JIA treated with biological disease-modifying drugs (tocilizumab and golimumab), direct relations between the level of hemoglobin and hepcidin were not found. However, it was found that the mean corpuscular hemoglobin level is associated with hepcidin concentration in patients with JIA. It was found that concentration of serum hepcidin may be used for early differential diagnosis of anemia and MAS in patients with JIA. High probability of evolution of anemia to MAS was found when concentration of hepcidin was <140 ng/mL and ferritin >160 g/L. The following combinations were associated with high probability of evolution of slight anemia to a severe form, but not to MAS: 1. hepcidin >200 ng/mL and ferritin <140 g/L; 2. hepcidin >140 ng/mL and soluble transferrin receptor <1.6 mg/L; 3. hepcidin >140 ng/mL and total iron binding capacity >46 mcmol/L. Misclassification should be assessed during further studies.

Download Full-text

Differential diagnosis portfolio of a pediatric rheumatologist: eight cases, eight stories

Clinical Rheumatology ◽

10.1007/s10067-020-05287-x ◽

2020 ◽

Author(s):

Mustafa Çakan ◽

Şerife Gül Karadağ ◽

Nuray Aktay Ayaz

Keyword(s):

Differential Diagnosis ◽

Pediatric Rheumatologist

Download Full-text

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2989 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 940.1-940

Author(s):

S. Costi ◽

F. Pregnolato ◽

A. Parafioriti ◽

E. Armiraglio ◽

T. Giani ◽

...

Keyword(s):

Differential Diagnosis ◽

Juvenile Idiopathic Arthritis ◽

Inflammatory Infiltrate ◽

Clinical Status ◽

Disease Onset ◽

Synovial Biopsy ◽

Response To Treatment ◽

Biologic Drugs ◽

Number Of Layers

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

Download Full-text

Community poverty level influences time to first pediatric rheumatology appointment in Polyarticular Juvenile Idiopathic Arthritis

Pediatric Rheumatology ◽

10.1186/s12969-021-00610-5 ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Nayimisha Balmuri ◽

William Daniel Soulsby ◽

Victoria Cooley ◽

Linda Gerber ◽

Erica Lawson ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Pediatric Rheumatology ◽

Proportional Hazards ◽

Cox Proportional Hazards ◽

Pediatric Rheumatologist ◽

Poverty Level ◽

Polyarticular Juvenile Idiopathic Arthritis ◽

Kaplan Meier ◽

Community Poverty ◽

The Impact

Abstract Background The impact of social determinants of health on children with polyarticular juvenile idiopathic arthritis (pJIA) is poorly understood. Prompt initiation of treatment for pJIA is important to prevent disease morbidity; however, a potential barrier to early treatment of pJIAs is delayed presentation to a pediatric rheumatologist. We examined the impact of community poverty level, a key social determinant of health, on time from patient reported symptom onset to first pediatric rheumatology visit among pJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods This is a cohort study of pJIA patients in the CARRA registry who lived in the United States from July 2015–February 2020. The primary exposure was community poverty level derived by geocoding patient addresses. The primary outcome was time to first rheumatology appointment. Kaplan-Meier analysis was performed to analyze time to first rheumatologist visit, stratified by community poverty and family income. Log-rank tests were used to identify differences between groups. Adjusted cox proportional-hazards models were used to determine the relationship between community poverty level and time from onset of disease symptoms to date first seen by rheumatologist. Results A total of 1684 patients with pJIA meeting study inclusion and exclusion criteria were identified. Median age of onset of pJIA was 7 years (IQR 3, 11), 79% were female, 17.6% identified as minority race and/or ethnicity, and 19% were from communities with ≥20% community poverty level. Kaplan-Meier analysis by community poverty level (< 20% vs ≥20%) yielded no significant differences with time to initial presentation to a pediatric rheumatologist (p = 0.6). The Cox proportional hazards model showed that patients with ≥20% community poverty level were 19% less likely (adjusted HR 0.81, 95% CI 0.67–0.99, p = 0.038) to be seen by a rheumatologist compared to patients with < 20% community poverty level, at the same time point, after adjusting for sex, race/ethnicity, insurance, education level, morning stiffness, RF status, and baseline CHAQ. Conclusion In this study of pJIA patients in the CARRA registry, increased community poverty level is associated with longer time to presentation to a pediatric rheumatologist after symptom onset.

Download Full-text