Small fiber neuropathy in children, adolescents, and young adults with chronic orthostatic intolerance and postural orthostatic tachycardia syndrome: A retrospective study.

PURPOSE: To determine in children, adolescent and young adult (CAYA) patients presenting with Orthostatic Intolerance (OI) or Postural Orthostatic Tachycardia Syndrome (POTS) associated with the additional symptoms of neuropathic discomfort (pain, paresthesia and/or allodynia): 1) the incidence of small fiber neuropathy, and 2) assess if there was serologic evidence for an underlying inflammatory or autoimmune state. METHODS: A cohort of 109 CAYA patients with the above symptoms underwent epidermal skin biopsy for nerve fiber density. Blood biomarkers for inflammation were tested (CRP, ESR, ANA, complement (C3), thyroid function testing with antibodies (thyroid peroxidase antibody and thyroglobulin antibody), and cytokine panel 13). Patients completed a Quality of Health questionnaire. Statistical analysis was performed using Wilcoxon rank sum tests. RESULTS: In CAYA patients with OI or POTS and neuropathic symptoms, skin biopsy for small fiber neuropathy was abnormal in 53 %. The sample population was predominantly female and Caucasian with moderately decreased perceived quality of health. OI /POTS patients with small fiber neuropathy had a 3-fold probability of having a positive ANA or anti-thyroid antibody, suggesting an underlying autoimmune or inflammatory process. CONCLUSION: Our data suggest a link between OI and POTS and small fiber neuropathy. Small fiber neuropathy was found by skin biopsy in over half of the patients tested. OI and Postural orthostatic tachycardia patients with small fiber neuropathy expressed multiple markers suggesting an underlying autoimmune or inflammatory process. Future research will be done to evaluate the symptomatic implication of SFN and whether immune or pharmacologic manipulation can alter patient symptoms.

The Challenges of Identifying Fibromyalgia in Adolescents.

Aim: Fibromyalgia (FM) is a noninflammatory disorder of the nervous system characterized by widespread musculoskeletal pain and somatic complaints of at least 3 months duration. There are no current diagnostic criteria for fibromyalgia in children to guide clinicians in recognition, thus leading to many subspecialty referrals and extensive imaging and tests. The purpose of this retrospective review is to compare two diagnostic criteria for juvenile fibromyalgia. Methods: A retrospective chart review of 20 children diagnosed with juvenile fibromyalgia from a singular pain physician practice was performed. Both the Yunus diagnostic criteria and the 2016 American College of Rheumatology (ACR) diagnostic criteria were applied and compared. Results: 85% of patients met criteria for fibromyalgia under both criteria. 15% of patients met only ACR criteria as the Yunus criteria excluded those with underlying conditions. Of the children who fulfilled criteria with use of both diagnostic tools, this cohort reported a high somatic symptom burden as demonstrated by the ACR symptom severity scales of 12 and satisfaction of at least 4 Yunus and Masi minor criteria on average. Widespread pain was noted with an ACR Widespread Pain Index (WPI) of 7, and tender points were 4.8 on average across the cohort. Effective therapeutic regimens among patients varied widely from medical monotherapy to multimodal treatment. Patients presented with pain for 1.8 yrs on average prior to a diagnosis. All of the cohort had a normal laboratory evaluation; half the cohort received additional imaging and testing. Conclusion: This case series suggests the need for an updated diagnostic tool for pediatric fibromyalgia to facilitate recognition and treatment.

Persistent low albumin and temporary vascular access in pediatric patients with SLE on hemodialysis.

Pediatric patients with systemic lupus erythematosus (SLE) often present with significant kidney disease. In a previous cross-sectional analysis, we showed that pediatric patients with ESRD secondary to SLE have lower serum albumin levels and less permanent vascular access for hemodialysis (HD) compared to pediatric patients on HD secondary to other causes. The goal of this longitudinal study was to determine if there was an improvement in these targets over time. To this end, we performed a longitudinal analysis of patients receiving HD in the ESRD Clinical Performance Measures Project 2000-2004 study years, comparing achievement of clinical targets between pediatric patients with SLE and pediatric patients with other causes of ESRD. In the longitudinal follow-up, pediatric patients with SLE were less likely to reach target albumin levels than other children with ESRD maintained on HD [odds ratio (OR) 0.18, 95% confidence interval (CI) 0.09, 0.35] and were less likely to have arteriovenous fistulas or grafts than other pediatric patients (OR 0.45, 95% CI 0.23, 0.89). Pediatric patients with SLE maintained on HD are at particularly high risk for failing to meet some clinical targets that have been associated with improved long-term outcomes in other populations. This is true even as they remain on dialysis over time.

Slow speed resistance exercise training in children with polyarticular juvenile idiopathic arthritis.

Background: Juvenile idiopathic arthritis (JIA) is an inflammatory autoimmune disease that can cause severe impairment and disability. Exercise is recommended to preserve joint mobility and function. Our objectives were to assess the safety, feasibility, and effects of slow speed resistance exercise in children with polyarticular JIA. Methods: Patients were recruited from a pediatric rheumatology clinic at an urban hospital and randomized to exercise or control groups. In the intervention group, slow speed resistance exercise with individualized instruction by a certified trainer was performed 1-2 times per week for 12 weeks. The control group performed home-based aerobic exercise 3 days per week for 12 weeks. Pre and post-body composition measurements by dual-energy X-ray absorptiometry; aerobic fitness by peak oxygen uptake during cycle ergometry; isometric muscle strength; and quality of life measures were obtained. Results: In the exercise group, 9/17 (53%) completed any exercise training. Of these nine subjects, five (55%) completed all 12 weeks of the protocol. In the control group, 8/16 (50%) reported compliance with the recommended aerobic exercise training at least one time per week. Only 2 subjects (12%) reported exercising more than once per week. There was no significant difference between pre- and post-measurements in any category in the exercise group. There was also significantly elevated body fat in both groups with only 17% in the control group and 23% in the exercise group meeting recommended <30% total body fat levels. Conclusions: Children with JIA participated safely in this resistance exercise protocol. The exercise was well-tolerated with no serious adverse events noted. While individual subjects reported improvement in fatigue and improved energy, there was no statistical difference in pre- and post-exercise measures of body composition or quality of life. Identifying ways to improve adherence and encourage exercise in children with JIA is important.

Approach to Membranous Lupus Nephritis: A Survey of Pediatric Nephrologists and Pediatric Rheumatologists.

OBJECTIVE: To describe treatment practices for childhood pure membranous lupus nephritis (MLN). METHODS: Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members. RESULTS: There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN. Mycophenolate mofetil was the favored initial NSI. Nephrologists used steroids (60% vs 93%) and NSI (53% vs 87%) less often than did rheumatologists for MLN without nephrotic syndrome (NS). CONCLUSION: Pediatric rheumatologists and nephrologists both recommend steroids and NSI for children with MLN, with or without NS.

Treatment of scleroderma: an update.

The goal of this article is to update the reader and focus on novel therapies and clinical trials published since our last review [6]. Evidence suggests that drug intervention should target one or all of three biological processes: vascular disease, autoimmunity and tissue fibrosis. Efforts should be made to classify the subtype of scleroderma, to determine the activity of the disease process and the degree of specific organ involvement before specific treatment decisions are made. Cyclophosphamide in fibrosing alveolitis, intravenous prostaglandins and other vasodilators for the vascular disease, endothelin-1 inhibition in pulmonary hypertension and immunosuppressive therapy for early inflammatory disease, all appear to have benefit. Several agents used in vitro and in animal models of fibrosis also show promise including anti-transforming growth factor-beta, the statins and anti-integrins. More experience in well-designed clinical trials is needed to define the role of these agents in treating scleroderma.

Reduced albumin levels and utilization of arteriovenous access in pediatric patients with systemic lupus erythematosus (SLE).

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects between five and ten thousand children in the USA. Kidney disease may progress to end-stage renal disease (ESRD) and subsequent need for dialysis therapy in a significant number of children with SLE. We performed a cross-sectional analysis comparing achievement of National Kidney Foundation/Kidney Disease Outcomes Quality Initiative clinical targets in pediatric patients with SLE maintained on hemodialysis (HD) to pediatric patients with other causes of ESRD. Ninety-seven unique SLE patients and two control groups-1,823 unique pediatric patients with other causes of ESRD and 694 pediatric patients with glomerulonephritis-were identified in the End Stage Renal Disease Clinical Performance Measures 2000-2004 Project Years. SLE patients were older, with a female and black race predominance compared with both control groups. Pediatric patients maintained on HD secondary to SLE were less likely to meet albumin targets and more likely to have vascular catheters than were pediatric patients on HD secondary to other causes. These findings may be associated with increased morbidity and mortality in pediatric patients with SLE maintained on HD and deserve further study.

Preoperative autologous blood donation by arthritis patients is associated with preoperative anemia and perioperative transfusion.

BACKGROUND: Preoperative autologous blood donation (PAD) became a common practice in the 1990s in an attempt to reduce the risk of transmission of infectious agents from allogeneic blood. However, a potential risk of PAD is the development of preoperative anemia that may lead, in turn, to an enhanced need for transfusion. Furthermore, the ready availability of autologous blood may predispose to more liberal transfusion peri-/postoperatively. OBJECTIVES: To examine these hypotheses, we retrospectively examined a cohort of knee and hip arthroplasty patients. METHODS: Charts of patients of 2 orthopedic surgeons from the mid 1980s and 1990s were reviewed for transfusions needed and hematocrits before and after arthroplasties. RESULTS: PAD proved to be a significant risk factor for the development of preoperative anemia and for peri-/postoperative blood transfusion even after adjusting for confounders. CONCLUSIONS: These results suggest that PAD may lead to a self-defeating cycle of blood donation followed by blood transfusion. With the improved safety of the allogeneic blood supply, rheumatologists may want to play a more active role in considering PAD in patients in whom elective arthroplasty is planned.