Radiologic Investigation of Rheumatic Diseases
Section snippets
Juvenile idiopathic arthritis
Clinically diagnosed, JIA is a chronic inflammatory arthritis that begins in patients younger than 16 years and that persists for longer than 6 weeks. All other diseases that can cause arthritis need to be considered and excluded before the diagnosis of JIA is made. JIA is the most important rheumatic disease affecting children and one of the most common chronic diseases of childhood. Although JIA may be self-limited, with most patients having no active synovitis in adulthood, many children
Juvenile spondyloarthropathies/enthesitis-related arthritis
The juvenile spondyloarthropathies are a group of disorders that affect the axial and extra-axial joints and are associated with the HLA-B27 antigen [27]. They currently fall under the classification of JIA and the subclassification of enthesitis-related arthritis. The spondyloarthropathies, which include juvenile ankylosing spondylitis, reactive arthritis, and arthritis associated with inflammatory bowel disease, all with onset in persons younger than 16 years, constitute the second most
Septic arthritis
Acute purulent infection of the joints is more common in infancy and early childhood [7] because of the greater blood flow to the joints during the active stages of growth. The usual cause is hematogenous dissemination related to upper respiratory infection or pyoderma. Infection may also spread from adjacent osteomyelitis, cellulitis, abscess, or traumatic joint invasion [7], [40]. In children, septic arthritis develops commonly from osteomyelitis in metaphyses that are intra-articular, such
Systemic lupus erythematosus
Systemic lupus erythematosus is an autoimmune disease characterized by a persistent nonspecific polyclonal B-cell activation that results in widespread tissue deposition of immune complexes that may cause organ damage [86]. Antiphospholipid antibodies, which are associated with a hypercoagulable state, have been associated with a variety of abnormalities including coronary artery and microvascular thrombotic occlusion, myocardial and cerebral infarction, and diffuse cardiomyopathy [87], [88].
Musculoskeletal system
Juvenile dermatomyositis
Juvenile dermatomyositis (JDM) is an autoimmune inflammatory myopathy characterized by diffuse, nonsuppurative inflammation of muscle fibers and skin [105]. Clinical findings include severe proximal muscle weakness, fatigue, heliotrope rash, and underlying vasculitic pathology [106], [107].
Scleroderma
Scleroderma is an autoimmune connective tissue disease that involves the microvascular system. Childhood onset is uncommon and accounts for only a small proportion of patients [121]. Two main categories of scleroderma have been described: systemic (also known as systemic sclerosis) and localized disease [122], the latter being the most common form of disease in children (rate of localized versus systemic scleroderma, 9:1). With increasing severity of the disease, the excessive production of
Vasculitis
Vasculitis is an inflammatory process that affects arteries, veins, and capillaries. The vessel wall is infiltrated by inflammatory cells that may or may not produce vascular destruction [138]. The vessels involved may be small, medium, or large. Most of the vasculitides affect vessels of varying sizes, although one particular size can be more commonly affected. Among the vasculitides described here, Kawasaki disease is the most common in the pediatric age group [138].
Kawasaki disease
Kawasaki disease is an acute vasculitis characterized by fever, rash, conjunctival injection, exanthem, redness and swelling of the hands and feet, and cervical adenitis [139]. This disease is seen almost exclusively in young children; approximately 80% of affected patients are under the age of 5 years [139]. The histopathogic features of vasculitis involving arterioles, capillaries, and venules appear in the earliest phase of the disease. Coronary aneurysms or ectasia develop in approximately
Polyarteritis nodosa
Polyarteritis nodosa is characterized clinically by a necrotizing vasculitis caused by immune complex deposition in vessel walls that predominantly involve medium-sized arteries of kidneys, central nervous system, muscles, and viscera [143]. The inflammation may skip areas along the course of the involved vessel [138].
Wegener's granulomatosis
Wegener's granulomatosis is a multisystemic disorder characterized by necrotizing granulomas in the upper or lower respiratory tract, with or without focal necrotizing glomerulonephritis and a systemic vasculitis [148]. This vasculitis predominantly affects medium and small blood vessels [138].
Takayasu's arteritis
Takayasu's arteritis is a chronic, progressive, and obliterative arteritis of large vessels that has a predilection for the aorta and its major branches but may also involve the coronary and pulmonary arteries [138]. It is characterized by a granulomatous inflammation of the arterial wall with marked intimal proliferation and fibrosis of the media and adventitia. This may lead to stenosis, occlusion, poststenotic dilatations, and aneurysm formation [154]. This arteritis is uncommon in children,
Osteoporosis
Osteoporosis is a skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue, which leads to increased bone fragility and fracture [160]. In JIA, multiple risk factors are associated with decreased bone mass including severity of inflammatory disease, decreased mobility, and use of glucocorticoids. Fractures may occur with low or minimal trauma, particularly in skeletal sites rich in trabecular bone (wrist, femoral neck, and spine) [161]. In adults,
Summary
Extensive evidence now supports the increasing role of all the cross-sectional imaging modalities, but especially MRI and sonography, in investigating rheumatic disease. CT plays a more limited role because of its need for radiation and its relatively poorer soft tissue contrast. MRI provides exquisite detail of all joint and periarticular soft tissues, which can be used to provide earlier and more accurate diagnosis and follow-up of inflammatory arthritides. The application of these techniques
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A version of this article originally appeared in the 52:2 issue of Pediatric Clinics of North America.