Kabita Nanda

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

Comparing presenting clinical features of 48 children with microscopic polyangiitis (MPA) against 183 having granulomatosis with polyangiitis (GPA). An ARChiVe study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

Assessing the performance of the Birmingham vasculitis activity score at diagnosis for Children with antineutrophil cytoplasmic antibody-associated vasculitis in a registry for childhood vasculitis (ARChiVe)

Objective. There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician’s global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods. Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman’s rank correlation coefficient (rs) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. Results. A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0–40). The BVAS v.3 correlations were rs = 0.379 (95% CI 0.233 to 0.509) with PGA, rs = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and rs = 0.403 (95% CI 0.253 to 0.533) with ESR. Conclusion. Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.

Novel Method to Collect Medication Adverse Events in Juvenile Arthritis: Results From the Childhood Arthritis and Rheumatology Research Alliance Enhanced Drug Safety Surveillance Project

Few data are available regarding the rates of serious adverse events (SAEs) and important medical events (IMEs) outside of product‐based registries and clinical trials for juvenile idiopathic arthritis (JIA). The Enhanced Drug Safety Surveillance Project (EDSSP) was developed to pilot a novel system to collect SAEs/IMEs in children with JIA. This analysis reports the results from this 4‐year (2008–2012) EDSSP.

Novel method to collect medication adverse events in juvenile arthritis

Few data are available regarding the rates of serious adverse events (SAEs) and important medical events (IMEs) outside of product‐based registries and clinical trials for juvenile idiopathic arthritis (JIA). The Enhanced Drug Safety Surveillance Project (EDSSP) was developed to pilot a novel system to collect SAEs/IMEs in children with JIA. This analysis reports the results from this 4‐year (2008–2012) EDSSP.

A 4-Year-Old Amish Boy With Weakness, Arthritis, Rash, Verbal Delay, and Failure to Thrive

The patient was in his usual state of health until March 2010, when he developed purplish discoloration with nodules on his fingers and toes. Over the next few months, he was noted to have progressive weakness with poor appetite, fatigue, and headache. Medical care was sought for these symptoms and he was initially diagnosed with a viral illness and subsequently with streptococcal pharyngitis. He had been noted to have delayed speech development in comparison with his 7 siblings.

Cartilage-protective effects of C-type natriuretic peptide over expression in K/BxN TCR arthritis model

Purpose The c-type natriuretic peptide (CNP) signaling pathway is known as a major contributor to skeletal growth in children. CNP is produced and secreted by both growth plate and joint chondrocytes, and has a paracrine regulatory effect on cartilage tissue. CNP increases matrix production by chondrocytes and promotes their proliferation. In this study, we investigated whether over-expression of CNP in chondrocytes would be protective of joint cartilage degradation during chronic inflammatory arthritis in vivo.

Case of juvenile dermatomyositis (JDM), thrombotic thrombocytopenic purpura (TTP), and Purtscher retinopathy

Methods Here we describe a previously healthy and athletic 12 year old female who was diagnosed with JDM following a period of two weeks of pruritic rash, fever, headaches, muscle pain and weakness. Her work-up was significant for elevated muscle enzymes and an abnormal MRI of bilateral quadriceps that demonstrated increased signal intensity. The patient was hospitalized for intravenous pulse methylprednisolone and intravenous immunoglobulin (IVIG) treatment. She showed significant improvement in her skin rash and muscle weakness, and was discharged with oral prednisone, naproxen and methotrexate. The patient returned to the hospital one week after discharge with complaints of blurry vision and headaches. In the emergency department the patient was noted to have retinal infarcts and was admitted directly to the Pediatric Intensive Care Unit (PICU). She was found to have thrombocytopenia, hemolytic anemia and renal failure. During her PICU stay she developed seizures and pulmonary edema and was subsequently diagnosed with TTP and severe retinopathy. Her ADAMTS-13 activity was found to be low (64%). She was treated with pulse steroid therapy, hemodialysis, plasmapheresis, multiple infusions of Fresh Frozen Plasma (FFP) and IVIG, but her condition continued to deteriorate. After four days of plasmapheresis and three days of hemodialysis she was given rituximab. Within 48 hours after rituximab treatment her condition dramatically improved. Rituximab was given at the same dose once weekly for 4 total doses, and no additional courses of rituximab were necessary.