Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.

Phenotypic and Genotypic Characterization and Treatment of a Cohort With Familial Tumoral Calcinosis/Hyperostosis-Hyperphosphatemia Syndrome.

Familial tumoral calcinosis (FTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is a rare disorder caused by mutations in the genes encoding fibroblast growth factor-23 (FGF23), N-acetylgalactosaminyltransferase 3 (GALNT3), or KLOTHO. The result is functional deficiency of, or resistance to, intact FGF23 (iFGF23), causing hyperphosphatemia, increased renal tubular reabsorption of phosphorus (TRP), elevated or inappropriately normal 1,25-dihydroxyvitamin D (1,25D), ectopic calcifications, and/or diaphyseal hyperostosis. Eight subjects with FTC/HHS were studied and treated.

High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound.

Objective: To determine the prevalence of temporomandibular joint (TMJ) disease in a cohort of children with new-onset juvenile idiopathic arthritis (JIA), and to compare magnetic resonance imaging (MRI) with ultrasound (US) for the detection of acute and chronic changes of TMJ arthritis.

Methods: Between January 2005 and April 2007, children with newly diagnosed JIA were prospectively evaluated for TMJ arthritis. Prior to imaging, jaw pain and disability were assessed with questionnaires and physical examination.

Reactive arthritis of the temporomandibular joints and cervical spine in a child.

Background: Temporomandibular joint (TMJ) arthritis is frequently seen in children with chronic arthritis. It has rarely been described in a non-infectious acute setting. We report a case of reactive arthritis isolated to the TMJs and cervical spine.

CT-guided percutaneous steroid injection for management of inflammatory arthropathy of the temporomandibular joint in children.

Objective: The purposes of this study were to retrospectively review an injection technique, to develop a grading system for evaluation of imaging findings, and to report preliminary outcome related to percutaneous CT-guided steroid injection into the temporomandibular joints of children with inflammatory arthropathy.

Conclusion: CT-guided steroid injection into the temporomandibular joint of children with inflammatory arthropathy results in clinical and imaging improvement in a substantial proportion of children treated.

Benefit of intraarticular corticosteroid injection under fluoroscopic guidance for subtalar arthritis in juvenile idiopathic arthritis.

Objective: To determine the demographics of subtalar arthritis, the response to intraarticular corticosteroid injection, and the injection complication rate in a clinic sample of children with juvenile idiopathic arthritis (JIA).

Methods: A chart review was performed of all patients at a tertiary medical center who underwent subtalar corticosteroid injection during the past 5 years. Injection of 1 ml of triamcinolone hexacetonide or acetonide into the midsubtalar joint was performed using a lateral oblique approach under fluoroscopic guidance.

Temporomandibular joint arthritis in juvenile idiopathic arthritis: the forgotten joint.

Purpose Of Review: This review explores the prevalence, clinical and radiographic signs, and treatment of temporomandibular joint arthritis in children with juvenile idiopathic arthritis.

Recent Findings: Temporomandibular joint arthritis seems to be a more frequent manifestation in patients with juvenile idiopathic arthritis than previously believed, in part due to the paucity of clinical symptoms and poor sensitivity of conventional radiographs used for diagnosis. Antinuclear antibody positivity, early onset of disease, and presence of systemic or polyarticular disease are all risk factors for temporomandibular joint arthritis but may underpredict temporomandibular joint involvement in juvenile idiopathic arthritis.

Utility of corticosteroid injection for temporomandibular arthritis in children with juvenile idiopathic arthritis.

Objective: To assess the effects of computed tomography (CT)-guided injection of corticosteroid into the temporomandibular joint (TMJ) in children with juvenile idiopathic arthritis (JIA) and clinical and magnetic resonance imaging (MRI) evidence of TMJ inflammation.

Methods: Twenty-three children ages 4-16 years with JIA and MRI evidence of TMJ inflammation received CT-guided TMJ injections of corticosteroid (triamcinolone acetonide [n = 16] or triamcinolone hexacetonide [n = 7]). Jaw pain or dysfunction and maximal incisal opening (MIO) distance were assessed before and after injection.