Pediatric Rheumatology

·         Naproxen, more often than other nonsteroidal anti-inflammatory drugs, can produce pseudoporphyria skin lesion, characterized by small hypopigmented flat scars after a blister formation.

·         Approximately 45% of children with juvenile rheumatoid arthritis will have active disease that persists into early adulthood.

·         Systemic-onset JRA often manifests with prolonged fevers, a salmon-pink macular rash, asymptomatic pericarditis, leukocytosis, anemia of chronic inflammatory disease, hepatosplenomegaly, and lymphadenopathy. Arthralgia or myalgia may be present, but arthritis does not usually develop until later in the course of the illness. SLE and rheumatic fever may also produce rheumatologic symptoms and pericardial effusion.

·         Arthritis is not a feature of Sjogren syndrome.

·         Colchicine is the mainstay of treatment for familial Mediterranean fever.

·         Henoch-Schonlein purpura is not associated with risk for developing amyloidosis.

·         The platelet count in Kawasaki disease is generally normal initially but rapidly rises by wk 2 or 3 of the illness; counts may exceed 1,000,000/mm3.

·         End-stage renal disease is an uncommon sequela of HSP. Nonetheless, 1% of patients do have persistent renal abnormalities.

·         Gorlin's sign is the ability to touch the tip of the nose with the tongue. It is seen in conditions associated with hypermobility syndromes, such as Ehlers-Danlos syndrome.

·         Fibromyalgia is a condition that is characterized by musculoskeletal aches and pains, fatigue, disturbed sleep patterns, and tenderness over various parts of the body. These tender points are specific for the diagnosis. There should be tenderness over at least four of these 11 points for proper classification of individuals. In addition, there should be no tenderness over nonspecific sites such as the forehead or the pretibial region.

·         The presence of rash distinguishes dermatomyositis from polymyositis. Three out of four criteria plus a pathognomic rash establish the diagnosis of dermatomyositis, and a confirmatory biopsy is not necessary. If fewer criteria are met, a biopsy may be needed for diagnosis.

·         JUVENILE RHEUMATOID ARTHRITIS

Sine qua non: Persistence of =6 weeks

Three main subtypes: Pauciarticular (=4 joints), polyarticular (>4 joints), and systemic-onset (spiking fevers)

Characteristic finding: Morning stiffness or soreness that improves during the day

No diagnostic laboratory tests are diagnostic

Patients <7 years old with ANA-positive pauciarticular JRA at highest risk for developing uveitis

JRA & Leukemia: Up to 20% of patients with leukemia have some degree of musculoskeletal symptoms, including joint pain and occasional swelling. In both diseases there is anemia, fever, and weight loss. Both can appear with hepatosplenomegaly and lymphadenopathy. In leukemia, however, the fever is not usually spiking, and platelets tend to be low to low normal. A good examination of a peripheral smear is crucial. A high lactic dehydrogenase level is very suggestive of leukemia, and the Tc99 bone scan shows a different pattern of uptake. More than one bone marrow biopsy may be necessary.

·         Macrophage activation syndrome (MAS). This is new conceptualization of an old problem seen in children with systemic-onset JRA both at onset (even at presentation) and late during the course of disease. It is characterized by a massive upregulation of T-cell and macrophagic function, with vast release of proinflammatory cytokines leading to hemophagocytosis (the hallmark). It is believed that, in most cases, MAS is triggered by a viral infection. MAS is the single most important contributor of mortality (which lately has improved), together with gastrointestinal bleeding and infection among patients with systemic JRA. The name and nosologic classification of this entity are currently being debated by experts in the field.

·         PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection), in which OCD and Tourette's syndrome in some children appeared to be triggered or exacerbated by streptococcal infections in the absence of classical chorea or other manifestations of rheumatic fever. The existence of PANDAS remains controversial.

·         Enthesopathy is unique to the spondyloarthropathies (JSA) and appears as painful localized tenderness at the tibial tubercle (which may be mistaken for Osgood-Schlatter disease), the peripheral patella, and at the calcaneal insertion of the Achilles tendon and plantar fascia (which may be mistaken for Sever's disease). Thickening of the Achilles tendon and tenderness of the metatarsophalangeal joints are associated findings.

·         The syndrome of seronegative enthesopathy and arthropathy (SEA) was described in 1982 in a group of children, mostly boys, with enthesitis and arthralgia or arthritis who were seronegative for ANA and rheumatoid factor. Many were positive for HLA-B27, but they did not fulfill criteria for definite spondyloarthritis. Long-term follow-up disclosed that about half went on to develop definite ankylosing spondylitis.

 ·         The dimples of Venus are prominent paravertebral indentations in the lower back of some individuals. A line drawn between the dimples marks the lumbosacral junction; this is the midpoint for the Schober test, which is a measure of anterior flexion of the lumbosacral spine.