Objectives
To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) versus healthy controls (HC).
Methods
In consecutive jRMD children and matched HC from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. 95 patients with juvenile idiopathic arthritis (JIA), 22 with dermatomyositis (JDM), 20 with systemic lupus erythematosus (cSLE), 13 with systemic sclerosis (jSSc), 21 with localized scleroderma (lSc), 18 with mixed connective tissue disease (MCTD) and 20 with primary Raynaud’s phenomenon (PRP) were included. NVC differences between juvenile subgroups and HC were calculated through multivariable regression analysis.
Results
A total number of 6474 images were assessed from 413 subjects (mean age 12.1-years, 70.9% female). The quantitative NVC-characteristics were significantly lower (↓) or higher (↑) in the following subgroups compared to HC: For density: ↓ in jSSc, JDM, MCTD, cSLE and lSc; For dilations: ↑ in jSSc, MCTD and JDM; For abnormal shapes: ↑ JDM and MCTD; For haemorrhages: ↑ in jSSc, MCTD, JDM and cSLE. The qualitative NVC-assessment of JIA, lSc and PRP did not differ from HC, whereas the cSLE and jSSc, MCTD, JDM, cSLE subgroups showed more non-specific and scleroderma patterns respectively.
Conclusion
This analysis resulted from a pioneering registry of NVC in jRMD. The NVC-assessment in jRMD differed significantly from HC. Future prospective follow up will further elucidate the role of NVC in jRMD.
Chronic recurrent multifocal osteomyelitis (CRMO) (OMIM # 259680) is a common genetically heterogenous autoinflammatory disease of the bone, first described in 1972 (1) with the typical mean age at diagnosis 9 years (2). Syndromic CRMO presents early in life and includes three monogenic recessively inherited conditions: Majeed syndrome (MJS), deficiency of interleukin-1 receptor antagonist (DIRA), and CRMO-related to the FBLIM1 gene (2).
Optic Neuritis is the most common presentation of MOG Antibody Disease (MOG-AD). We share our experience with a 11-year-old boy who developed MOG associated Optic Neuritis temporally associated with SARS- CoV-2 infection. He responded well to intravenous methylprednisolone therapy followed by oral prednisolone. While various neurological and ophthalmological manifestations of COVID-19 have been described previously, there are few case reports of Optic neuritis associated with COVID-19. Our case further supports the evidence to suggest that SARS CoV-2 is another such virus that triggers MOG-AD.
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