Barbara Edelheit

Systemic lupus erythematosus in a pediatric patient with congenital acquired immunodeficiency syndrome.

The coexistence of congenital HIV infection with primary rheumatologic disease is rare. We have described a child with congenital AIDS and concurrent systemic lupus erythematosus who presented with small vessel vasculitis with no renal involvement. Oral corticosteroid therapy resulted in significant improvement in her clinical state. The child also responded strongly to potent antiretroviral therapy both virologically and immunologically.

Preliminary outcomes of a cross‐site cognitive‐behavioral and neuromuscular integrative training intervention for juvenile fibromyalgia

Cognitive–behavioral therapy (CBT) is effective in reducing disability among youth with juvenile fibromyalgia (FM); however, engagement in moderate to vigorous physical activity remains poor, even after CBT. The purpose of this study was to evaluate the feasibility and preliminary outcomes of an innovative program combining CBT with specialized neuromuscular exercise: the Fibromyalgia Integrative Training for Teens (FIT Teens) program.

Cranial nerve VI palsy as an initial presentation of necrotizing sarcoid granulomatosis in a 14-year-old female: case report and literature review.

OBJECTIVES We describe a case of pediatric necrotizing sarcoid granulomatosis (NSG) presenting with right cranial nerve VI palsy and multiple lung nodules, successfully treated with corticosteroids. METHODS This is a descriptive case report of one patient with review of the literature. RESULTS A 14-year-old Caucasian female presented with complaints of pain on inspiration and dyspnea on exertion, as well as diplopia that was worse with right gaze. The patient presented to our emergency department with persistent diplopia and was found to have stable right cranial nerve VI palsy. CTA showed multiple pulmonary nodules. Despite continued extensive multispecialty work-up, the patient׳s cranial nerve VI palsy had not resolved, thus tissue confirmation via lung biopsy was performed. Pathologic diagnosis revealed necrotizing sarcoid granulomatosis. The patient was subsequently started on intravenous corticosteroids, which led to the rapid resolution of her presenting symptoms. CONCLUSIONS Necrotizing sarcoid granulomatosis is a multisystem organ disease that is rare in children. Pathology commonly reveals epithelioid noncaseating granuloma and granulomatous vasculitis with necrosis. We report an unusual presentation involving sixth nerve palsy in a 14-year-old girl. Diagnosis was determined and confirmed by histopathology of a pulmonary nodule biopsy. This is the first case to our knowledge of NSG presenting with cranial nerve palsy in a pediatric patient.

Risk, Timing, and Predictors of Disease Flare After Discontinuation of Anti-Tumor Necrosis Factor Therapy in Children With Polyarticular Forms of Juvenile Idiopathic Arthritis With Clinically Inactive Disease

To determine the frequency, time to flare, and predictors of disease flare upon withdrawal of anti–tumor necrosis factor (anti‐TNF) therapy in children with polyarticular forms of juvenile idiopathic arthritis (JIA) who demonstrated ≥6 months of continuous clinically inactive disease.

A12: The Role of Serum S100A12 Protein Levels in Disease Flare After Withdrawal of Anti‐tumor Necrosis Factor Therapy in Polyarticular Forms of Juvenile Idiopathic Arthritis

Anti‐TNF therapy for polyarticular forms (extended oligo‐, rheumatoid factor +/− polyarthritis) of JIA (PF‐JIA) results in up to 50% of patients (pts) demonstrating clinically inactive disease (CID). This study determined the pattern of serum S100A12 levels at the time of withdrawal of anti‐TNF therapy.

Age and fecal microbial strain-specific differences in patients with spondyloarthritis

BackgroundPrior studies have demonstrated abnormalities in the composition of the gastrointestinal microbiota in pediatric and adult patients with spondyloarthritis (SpA). In particular, diminished fecal abundance of Faecalibacterium prausnitzii and abnormalities in both directions in the abundance of the Bacteroides genus have been identified.MethodsWe obtained fecal specimens from 30 children with treatment-naïve enthesitis-related arthritis (ERA) and 19 healthy controls, as well as specimens from 11 adult patients with longstanding SpA and 10 adult healthy controls. All of the samples underwent sequencing of the 16S ribosomal DNA. A subset of the pediatric fecal samples was subjected to shotgun metagenomics sequencing.ResultsERA patients had decreased abundance of the anti-inflammatory F. prausnitzii A2-165 strain (41 ± 28% versus 54 ± 20% of all sequences matching F. prausnitzii, p = 0.084) and an increased abundance of the control F. prausnitzii L2/6 strain (28 ± 28% versus 15 ± 15%, p = 0.038). Similar trends were observed in adults with longstanding SpA (n = 11) and controls (n = 10). In contrast, the fecal abundance of Bacteroides fragilis was increased in ERA subjects (2.0 ± 4.0% versus 0.45 ± 0.7% of all sequences, p = 0.045), yet was diminished in adult subjects (0.2 ± % versus 1.0 ± % of all sequences, p = 0.106). Shotgun metagenomics sequencing of the fecal DNA in the pediatric subjects revealed diminished coverage of the butanoate pathway (abundance normalized to controls of 1 ± 0.48 versus 0.72 ± 0.33 in ERA, p = 0.037).ConclusionsThe anti-inflammatory F. prausnitzii A2-165 strain appears to be depleted in both pediatric and adult SpA. In contrast, B. fragilis may be depleted in adult disease yet abundant in pediatric SpA, suggesting developmental effects on the immune system.

Serum S100A8/A9 and S100A12 Levels in Children with Polyarticular Forms of Juvenile Idiopathic Arthritis: Relationship to Maintenance of Clinical Inactive Disease During and Flare after Discontinuation of Anti‐TNF Therapy

To determine the relationship between serum levels of S100A8/A9 and S100A12 and the maintenance of clinically inactive disease during anti–tumor necrosis factor (anti‐TNF) therapy and the occurrence of disease flare following withdrawal of anti‐TNF therapy in patients with polyarticular forms of juvenile idiopathic arthritis (JIA).

High Levels of DEK Autoantibodies in Sera of Patients With Polyarticular Juvenile Idiopathic Arthritis and With Early Disease Flares Following Cessation of Anti-Tumor Necrosis Factor Therapy

The nuclear oncoprotein DEK is an autoantigen associated with juvenile idiopathic arthritis (JIA), especially the oligoarticular subtype. DEK is a secreted chemotactic factor. Abundant levels of DEK and DEK autoantibodies are found in inflamed synovium in JIA. We undertook this study to further characterize the nature of DEK autoantibodies in screening serum samples from 2 different cohorts that consisted mostly of patients with JIA.

Understanding the biology and use of TNF therapy in jia-clinical outcomes

Treatment with anti-TNF therapies (anti-TNF) for polyarticular forms (extended oligo, Poly RF +/-) of JIA (PF-JIA) results in >50% demonstrating clinical inactive disease (CID).

High levels of DEK autoantibodies may predict early flare following cessation of anti-TNF therapy in juvenile idiopathic arthritis

The nuclear oncoprotein DEK is a biochemically distinct, pro-inflammatory protein that is a chemoattractant for neutrophils and T-cells. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA), but their role in disease pathogenesis is unclear.