Paula W. Morris

Juvenile dermatomyositis as a paraneoplastic phenomenon: an update.

Approximately 33% of adult patients with dermatomyositis develop malignancy with up to 42% presenting after the diagnosis has been made; careful evaluation for malignancy is often undertaken at the time of dermatomyositis diagnosis. This phenomenon has rarely been noted in pediatric patients and extensive workup for malignancy is not indicated in pediatric patients. In 1993, 6 cases were reported in which juvenile dermatomyositis/polymyositis (JDM/PM) appeared to be part of a paraneoplastic phenomenon. Our objective was to update the literature for reported cases of malignancy associated with JDM/PM; we reviewed the literature over the last 15 years and located 6 additional cases. In 9 of 12 reported patients an unusual physical finding such as splenomegaly or lymphadenopathy was noted at the time of diagnosis, and in the entire group, the malignancy occurred within a mean of 12 months. It is less likely that JDM/PM in pediatric patients is a paraneoplastic phenomenon as it is in adult patients. However, if the physical examination at the time of diagnosis of JDM/PM is atypical the presence of malignancy must be considered and a more in-depth evaluation to rule out malignancy should be performed prior to the initiation of treatment.

Disappearing enhancing brain lesion in a child with neurofibromatosis type I

Abstract Neurofibromatosis type 1 (NF1) in children can produce a variety of parenchymal signal abnormalities on cranial MR. Areas of abnormal signal in these patients may represent regions of disordered myelination, “hamartomatous” change or frank neoplasia. The presence of contrast enhancement in intracranial lesions in patients with NF1 is usually strongly suggestive of tumor. We report the case of a child with NF1 and a focal enhancing brain parenchymal lesion which spontaneously resolved without specific therapy.

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.

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.

A44: High Levels of DEK Autoantibodies May Predict Early Flare Following Cessation of Anti-TNF Therapy

The nuclear oncoprotein DEK is a biochemically distinct protein, modulating heterochromatin integrity, chemoattractant of neutrophils and T‐cells and vital for the formation of neutrophil extracellular traps (NETs). NETs are important for resolution of inflammation suggesting that DEK contributes to the development of autoimmune diseases. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA) but their role in disease pathogenesis is not clear. Since DEK and DEK autoantibodies can contribute to the development of immune complexes and NET formation we suggest that DEK antibody levels can predict flare with the discontinuation of anti‐TNF therapy.

Understanding the biology and use of anti-TNF agents in JIA – interim results

Purpose Current treatment for children with polyarticular forms of Juvenile Idiopathic Arthritis (Poly JIA) results in approximately 50% of the patients demonstrating clinically inactive disease (CID) (Wallace C, et al. J Rheumatol 2004;31:2290-4) while on treatment. Over 40% of the children with Poly JIA are treated with a TNF antagonist biologic often started ≤ 6 months (mos) of disease onset. Currently we are unable to accurately predict which children demonstrating CID while on an anti-TNF therapy will or will not have a flare of the JIA upon discontinuation of the anti-TNF agent. Anti-TNF therapy has known shortand medium-term toxicities in children with JIA; the long-term toxicities are unknown and the treatment is expensive. The ultimate goal of this project is to validate biomarkers that are predictive. These interim results demonstrate clinical aspects of assessing CID and stopping anti-TNF therapy.