The history can identify symptoms that reflect the source of the inflammation, including whether it is localized or systemic. Symptoms of systemic inflammation tend to be nonspecific. Fever, caused by cytokine release, can take many forms. A hectic fever, without periodicity or pattern, is commonly found in vasculitides such as Kawasaki disease but also occurs in children with underlying infection. Certain illnesses, such as systemic-onset JIA, produce a patterned fever with regular temperature spikes once or twice a day. Other rheumatic illnesses cause low-grade fevers. Charting the child’s fever pattern, particularly in the absence of antipyretics, is useful. Rashes occur in many forms (see Table 86-1). Other systemic symptoms (malaise, anorexia, weight loss, and fatigue) can vary from mild to debilitating.
Symptoms of localized inflammation vary depending on the involved site. Arthritis, or inflammation of the synovium (synovitis), leads to joint pain, swelling, and impaired ability to use the affected joint. Morning stiffness or gelling is described. The child may be slow to arise in the morning and may have a limp. Children may refrain from usual activities and athletics. Enthesitis is inflammation at the insertion of a ligament to a bone. Serositis, inflammation of serosal lining such as pleuritis, pericarditis, or peritonitis, gives rise to chest pain, shortness of breath, or abdominal pain. Myositis,inflammation of the muscle, may lead to symptoms of muscle pain, weakness, or difficulty performing tasks of daily living. Vasculitis, inflammation of the blood vessels, leads to nonspecific symptoms of rash (petechiae, purpura) and edema when small vessels deep in the papillary dermis are involved; involvement of medium-sized vessels results in a circumscribed tender nodule.
A thorough history and physical examination is frequently sufficient to narrow the differential diagnosis and elicit the diagnosis. The child’s overall appearance, evidence of growth failure, orfailure to thrive may point to a significant underlying inflammatory disorder. The head and neck examination may showevidence of mucosal ulceration, seen in diseases such as SLE. The eye examination may show pupillary irregularity and synechiae from uveitis or the conjunctivitis of Kawasaki disease.Diffuse lymphadenopathy may be found and is nonspecific. Therespiratory and cardiac examinations may show pericardial orpleural friction rubs, indicating serositis. Splenomegaly or hepatomegaly raises suspicion of activation of the reticuloendothelial system that occurs in systemic-onset JIA or SLE.
The joint examination is crucial for the diagnosis of arthritis and may identify evidence of joint swelling, effusion, tenderness, and erythema from increased blood flow. Joint contractures may be seen. The joint lining, or synovium, may be thickened from chronic inflammation. Activation of epiphyseal growth plates in an area of arthritis can lead to localized bony proliferation and limb length discrepancies. Conversely inflammation at sites of immature growth centers may lead to maldevelopment of bones, such as the carpals or tarsals, resulting in crowding, or the temporomandibular joints, resulting in micrognathia. A rash or evidence of underlying skin disorders, such as skin thickening from scleroderma or sclerodactyly, may be noted. Chronic Raynaud phenomenon may result in nail-fold capillary changes, ulceration, or digital tuft wasting.
The rheumatic diseases of childhood encompass a heterogeneous group of diseases with a shared underlying pathogenesis: disordered functioning of the immune system leading to inflammation directed against native proteins, with secondary increases in numbers of activated lymphocytes, inflammatory
Monarticular, Yes 50% No Yes Yes Yes —