Peds-Rheumatology

-autoimmune

H and P

-JRA

-rule out malignancies, infections, IBD

-JRA, SLE, Rheumatic fever, juvenile dermatomyositis scleroderma

-rule out hypothyroidism, trauma, reactive arthritis, endocarditis, and other infections

-juvenile dermatomyositis

-rule out muscular dystrophy and other myopathies

-SLE

-rule out phosensitivity dermatitis and 5ths disease

-JRA, SLE

-rule out pericarditis, isolated costchondritis, and rib fx

-JRA, spondyloarthropathy

-vertebral fracture, diskitis, intraspinal fx

because manifestations can take a long time

symptoms of weakness

rash characterized by pertibial tender erythametous nodules found deep in the dermis and subcutaneous tissues

ESR and CRP

increased lymphocyte activity found in Crohns, hepatitis, Graves, Leukemia and rheumatic diseses

JRA

synovitis of the peripheral joints manifesting in soft tissue swelling and effusion

-oligoarthritis or Pauciarticular disease
-polyarthritis
-systemic onset disease

-before 16
-swelling or effusion or 2 or more of the following:
-limitation in range of motion
-tenderness
-pain on motion
-increased heat

type of articular involvement in the first 6 monthss after onset

->5 inflammed joints
-< 4 inflammaed joints
-arthritis with characterisitic fever

-morning stiffness and gelling
-easy fatigability
-objective joint swelling
-involved joints are often warm, resist full range of motion and are painful on motion
-there is usually no redness

-lower extremities
-single joint is involved at onset
-hip

1-involves small and large joints of both upper and lower extremities
2-rheumatoid nodules on teh extensor surfaces of the elbows and achilles tendon
3-TMJ involvement

-arthritis with visceral involvement including hepatosplenomegaly
-intermittent fever >102 for > 2 weeks followed by normal temperature
-each febrile episode is usually accompanied by a rash that is salmon colored and asymptomatic

-acture rheumatic fever
-Lyme disease

-NSAIDS
-Steroids
-Methotrexate

auto antibodies directed against self antigens leading to inflammatory damage of amny target organs including the joints, kidneys, blood forming cells and the nervous system

meticulous and frequent re-evaluation of clinical signs and lab data especially kidney function

hepatotoxicity

hydrochloroquin

antiphospholipid antibodies

improve kidney function and prolong survival

juvenile dermatomyositis

a characteristic rash and symmetric muscle weakness

hx of infection in the 3 months before disease presents

-URI and gastroenteritis are most common

class II HLA antigen

-heliotrope rash that is in sun exposed areas, mostly the face

-florid rash palpable over joints

Gower sign, or refusal to be hugged due to muscle pain

characteristic rash

edema of muscles and inflammation

calcification on muscles

muscle derived enzymes (creatine kinase, aldolase, and LDH)

paletorespiratory dysfunction and demonstrate unprotected airway

-chloroquin


-steroids, and methotrexate

the sun and should use sun screen even in winter or cloudy days

fibrosis affecting the dermis, and the arteries of the lungs, GI and kidneys

skin involved and interal organs involved

1-Raynaud phenomenon for years
2-skin involvement limited to hands, face, feet, and forearms
3-dilated nailfold capillary loops, usually w/out capillary drop out
4-significant late incidence of pulmonary HTN, with or w/out skin calcification, GI disease, CREST syndrome, or interstitial lung disease
5-renal disease is rare
6-ACA in present in 70-80%

1-Raynaud's followed by puffy or hidebound skin changes
2-truncal and acral skin involvement; tendon friction rubs
3-nailfold capillary dilation and capillary drop out
4-early and significant incidence of renal, interstitial lung, diffuse GI and myocardial disease
5-Anti-Scl-70 and anti RNA polymerase I, II or III antibodies

1-presentation with pulmonary fibrosis or renal ,cardiac, or GI disease
2-no skin involvement
3-Raynauds may be present
4-ANA may be present -Anti Scl 70, ACA, or anti-RNA polymerase I, II or III may be present

-generally diffuse distribution of skin slcerosis and a hx of exposure to an environmental agent suspected of causing scleroderma

-SLE
-RA
-dermatomyositis
-vasculitis
-Sjorgren's Syndrome

1-Raynauds
2-nailfod capillary changes and evidence of digitsl ischemia
3-specific circulating autoantibodies (anti-Scl 70; ACA, or anti RNA polymerase I, II, or III

-first you get edema of the involved skin
-edema resolves leaving fibrosis
-fibrosis leads to tightening of the skin
-later atrophic skin can become shiny and waxy in appearance

arterial and interstitial involvement leads to dyspnea and right sided HF

1-in children morphea usually happens in the face
2-leg length discrepancies is seen when an entire leg is involved due to flexion contractures

typical skin changes involving the areas proximal to the MCP or MTP joints

1-slcerodactyly-skin changes limited to digits
2-digital pitting scars resulting from ischemia
3-bibasilar pulmonary fibrosis not attributable to primary lung disease

-used to curb inflammation but are ineffective at the later stage of the disease

to help tx Raynauds

inflammation of blood vessels

size of vessels involved, the type of inflammation and the major organ involved

1-medium vessels
2-hematuria, GI pain and arthritis
3-associated with poststrep infections

1-presents with pulomonary and upper-respiratory manifestations
2-mononeuritis multiplex

18 months to 2 years

5 or more days

1-bilateral conjunctival injection
2-changes in mucous membranse of the oropharyns such as injected pharynx, injected lips, dry fissured lips, strawberry tongue
3-cahgnes of the peripheral extremities such as peripheral edema, peripheral erythema, desquamation, and periungual desquamation
4-rash, primariliy truncal; polymorphic but nonvesicular
5-cervical lymphadenopathy

1-7-14 days
2-fever, irritability, conjunctivitis, oropharyngeal erythema, Rahs lymphadenopathy, and distal extremitiy and edema
3-fever
4-fever is sudden and very high up to 104*
5-conjunctivitis lasts 2 weeks
6-red cracked lips, diffuse mouth erythema, and strawberry tongue

1-hallmark is finger or toe desquamation
2-artheritis of small and large joints can happen
3-CNS involved with menignitis signs and lethargy
4-combination of coronary artery aneurysms and thrombocytosis makes it concernign
5-develop aneurysms and possibly die
6-first 40 days

1-symptoms dissipate, and lab values return to normal
2-risk of aneurysms decline but never to zero
3-beau lines form on finger nails
4-risk of atherosclerotic heart disease remains

anaphylactoid purpura

non-thrombocytopenic purpura, arthritis, arthralgia, GI symptoms, and vairety of renal findings

milder disease with less renal and GI symptoms

actuely develops a ksin rash, arthritis, and abdominal pain

the skin rash

4 weeks

normal