Juvenile Spondyloarthropathies
Arthritis and enthesitis Or Arthritis and enthesitis, or arthritis or enthesitis with at least two of the following Presence of or a history of sacroiliac joint tenderness and/or inflammatory lumbosacral…
Clinical Examination of the Child with a Rheumatic Disease
Life-threatening conditions Malignancy (leukaemia, lymphoma, bone tumour) Sepsis (septic arthritis, osteomyelitis) Non-accidental injury Joint pain with minimal or no swelling Hypermobility syndromes Orthopaedic syndromes (e.g. Osgood–Schlatter disease, Perthes’ disease) Idiopathic…
Behçet’s Disease
Fig. 38.1 Deep vein thrombosis of the right leg in a young boy Introduction Behçet’s disease is a systemic vasculitis of unknown origin, which is seen in the pediatric age…
Overview of Juvenile Idiopathic Arthritis
Inflammatory Infection: septic arthritis, osteomyelitis, tuberculosis, Lyme arthritis Reactive arthritis secondary to extra-articular bacterial (e.g., streptococcal, enteric bacteria) or viral infections (e.g., hepatitis B, parvovirus, Epstein-Barr virus, varicella, rubella) Malignancies:…
Basic Immunology for the Pediatrician
Innate immunity Adaptive immunity Physical barriers Skin, mucous membrane Mucosal immune system Cellular component Neutrophils Macrophages and dendritic cells Natural killer cells T and B lymphocytes Soluble molecules (circulating…
Autoinflammatory Syndromes in Children
Disease Gene defect Attack duration Clinical features Risk of amyloidosis Treatment Familial Mediterranean fever (FMF) MEFV 1–3 days Episodic fever, peritonitis, pleuritis, erysipeloid erythema, monoarthritis M694V and M694I mutations Colchicine…
Juvenile Localized Scleroderma
Type Subtype Features 1. Circumscribed morphea (a) Superficial Oval or round circumscribed areas of induration limited to the epidermis and dermis, often with altered pigmentation and violaceous, erythematous halo (lilac…
Pediatric Lupus Nephritis
Class – name Light microscopy Immunofluorescence/electron microscopy (location of immune deposits) Proteinuria Hematuria Hypertension I – minimal mesangial Normal Mesangial Absent Absent Absent II – mesangial proliferative Mesangial expansion and/or…
Juvenile Systemic Sclerosis
Fig. 30.1 The scleroderma patterns in systemic sclerosis are characterised by progressive loss of capillaries. In an early pattern, there is a prevalence of giant capillaries without loss of capillaries….
Macrophage Activation Syndrome
Fig. 22.1 Cytolytic pathway proteins mutated in MAS. A diagram of the immunologic synapse between a cytolytic (to the left) lymphocyte and an APC (to the right) is portrayed. Proteins…
