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Flashcards in Rheumatology Deck (96):

Diagnostic criteria for JIA

1. Age of onset less than 16 y/o
2. Arthritis in one or more joints
3. Duration of disease for 6 weeks or greater


definition of arthitis

joint swelling or effusion
2 or more:
1. limited ROM
2. tenderness or pain with motion
3. warmth


JIA is associated with:

1. Fhx of other autoimmune dz
2. 50% concordance w/ monozygotic twins
3. certain HLA types


synovial tissue is the target of this autoimmune response, resulting in: ___ and ___

inflammation with synovial tissue hypertrophy and increased amounts of joint fluid


If synovitis persists, __, __, and __ may occur.

-permanent destruction of articular cartilage,
-subchondral bone and
-other joint structures


An example of ____ JIA, extended is a patient who initially has 4 joints involved, but then over time, develops more involved joints



The type of JIA is defined by

the manifestations which occur in the first 6 months of disease


Types of JIA

1. Systemic
2. Polyarticular
-Rheumatoid factor negative
-Rheumatoid factor positive
3. Oligoarticular
4. Enthesitis-related
-Ankylosing spondylitis
-Psoriatic arthritis
-IBD-associated arthritis

*defined by the manifestations which occur in the first 6 months of disease


Presentation of Systemic JIA

1. polyarticular- affecting both small and large joints
2. fever occurring daily or twice daily, usually in the afternoon or evening
3. Fever accompanied by Still's rash
4. Fever over 39C with rapid return to base
5. Hepatosplenomegaly
6. LAD
7. Serositis
8. Hepatitis
9. Tenosynovitis


Describe a Still's Rash

*associated w/ Systemic JIA
1. well-demarcated salmon pink macules of various sizes
2. rarely persisting in any location for over 1 hr (evanescent)
3. Usually on trunk. proximal extremities, and pressure areas


With these additional systemic features, it is not unusual for these patients to initially be thought to have __ or __

cancer or serious infectious disease


Presentation of Oligoarticular JIA

1. involves 4 or less joints, usually larger joints
2. asymmetrical joint involvement
3. Accelerated growth in affected limb leading to leg discrepancy
4. Keep joint flexed- possibly due to pain vs compensation for leg length discrepancy
5. muscle atropy-- suggests disease of the involved joint
6. Uveitis-- ASYMTOMATIC


Why do kids with oligo JIA often have leg length discrepancy?

Due to the increased blood flow to the inflamed joint, these children will have accelerated growth in the affected limb, leading to a leg length discrepancy


Oligo JIA is rarely associated with disease outside of the joint itself, with the exception of ___.



What is uveitis

Uveitis involves inflammation of the iris and ciliary body and in oligo JIA, is typically chronic anterior uveitis.


Changes that are involved with uveitis

1. uneven pupil resulting from the posterior synechiae, which is scarring of the iris to the lens.
2. Other late changes of uveitis include cataracts
3. glaucoma, and
4. vision loss


Uveitis occurs in ___% of oligo JIA patients, and is often asymptomatic, so they require careful surveillance.
-If they are ANA titer +, the risk increases to ___%.
-Up to ___% if they are ANA titer -





What doe surveillance for uveitis in olgio JIA consist of?

1. slit-lamp exams every 3 months if ANA +
2. ANA - should still be screened with slit lamp exams using the following criteria:
-7y/o or less: every 3 months
-over 7y/o: every 6 months

*The referral for the ophthalmologist is done by the rheumatologist, but is important to be aware of in primary care should you notice this important eye finding, in a JIA patient, during an exam for another reason


treatment of uveitis

1. topical steroids
2. in severe cases- systemic meds used to treat JIA are helpful in eye inflammation as well


Presentation of polyarticular JIA

1. may begin with only 1 joint, but by the 6 month mark we talked about earlier, it will involve 5 or more joints (both large and small)
2. symmetrical joint involvement (contrast to oligo)


While __ status is an important criteria for oligo JIA, ___ status is important in poly JIA.


rheumatoid factor (RF)


RF + poly JIA is associated with:

1. rheumatoid nodules
2. chronic arthritis
3. joint destruction


Patients with poly JIA may have mild systemic symptoms such as

1. fatigue,
2. anemia of chronic disease and
3. growth failure


___ is inflammation at the insertion of tendons.



Arthritides which start with an enthesitis component may later develop into __ or ___

1. ankylosing spondylitis or
2. IBD-associated arthritis


What comprises the enthesitis-related arthropathies?

ankylosing spondylitis or IBD-associated arthritis, combined with psoriatic arthritis


Who most common presents with juvenile ankylosing spondylitis

1. male predominance (6:1)
2. late childhood/early adolesence onset


Presentation of juvenile ankylosing spondylitis

It begins with peripheral arthritis, with frequent enthesitis, and later into axial involvement


What is associated with juvenile ankylosing spondylitis?

1. RF and ANA negative
2. HLA-B27 is + in 90% of pts
3. + Fhx


Inflammatory manifestations of juvenile ankylosing spondylitis outside the joints include:

1. acute uveitis (5-10%)-- SYMPTOMATIC unlike oligo JIA
2. aortic valve insufficiency.


Laboratory studies, in primary care, are not particularly helpful in diagnosing JIA. USE the __ instead

diagnostic criteria instead.

*Laboratory studies, in primary care, are helpful in ruling in or out alternative diagnoses for inflamed joints and we’ll cover the differential diagnosis of inflamed joints

*Additionally, the typical wait to see a pediatric rheumatologist is 3 months or more (many states do not even have a pediatric rheumatologist), so reassuring or frightening parents with RF and ANA test results is not productive.


RF is frequently negative (remember, there is an RF-negative poly JIA) and ANA is notorious for weakly positive results. These tests should be used to:

predict the course or potential complications of the patient’s JIA, not to diagnose JIA in a primary care provider’s office


tests which may occur in the work-up of a patient with inflamed joints, whether in a primary care office, ED or rheumatologist office

1. acute phase reactants (CRP and ESR)
2. joint aspiration
3. Xrays (not particularly helpful in JIA, as they are not diagnostic, however x-rays are used in primary care to rule out other causes of joint swelling or extremity pain)


When will CRP and ESR be elevated

typically elevated in systemic (markedly) and poly JIA (mildly), however will also be elevated in septic arthritis and reactive arthritis


When and with that JIAs are joint aspirations abnormal?

1. increased polymorphonuclear (PMN) cells and low glucose, in the setting of JIA. Anti-cyclic citrullinated peptide (CCP) antibody is a very helpful test for diagnosing JIA.

*If it is positive, it is almost certain the patient has JIA (high specificity), however it is falsely negative 25-40% of the time (moderate sensitivity).


When may it be reasonable to begin lab workup for JIA in primary care?

In a situation with a patient in primary care, whose arthritis has been ongoing, with no alternative diagnoses identified, but maybe hasn’t quite met the 6 week timeframe, it may be reasonable, in consultation with a pediatric rheumatologist, to begin lab work for JIA.


Early non-specific findings in JIA xrays include

osteoporosis and soft tissue swelling


Late changes of JIA on xray include

1. narrowed joint space,
2. erosions of subchondral or juxta-articular bone and
3. various degrees of joint destruction.


The conventional approach to treatment of JIA is to

begin with the safest, most conservative treatment with NSAIDs being the mainstay.


Advancing up the pyramid in the conventional approach for tx of JIA may include

1. DMARDs may be used, 2. systemic steroids for exacerbations,
3. methotrexate,
4. followed by biologics (TNF and interleukin inhibitors) and
5. stem cell transplant, which is still in the experimental phase.


What does the inverted pyramid approach for tx of JIA suggest?

The inverted pyramid approach essentially recommends being very aggressive initially with combo therapy first


Patients with JIA benefit from __ and __ to minimize pain, maintain and restore function and prevent deformity/disability.

physical and occupational therapy


Patients with JIA benefit from physical and occupational therapy to __, __, and ___

1. minimize pain,
2. maintain and restore function and
3. prevent deformity/disability.


Describe the prognosis of JIA

quite good- 75-80% of children surviving without serious disability


The most common reason in primary care for a painful extremity or swollen joint is ___.

acute trauma


When acute or chronic pain affects the lower limb in children, think about ___

the lower spine down to the feet


__ and __ arthritis most frequently affect the hip in children

Septic arthritis and reactive arthritis


Generalized bone pain may be:

1. malignancy, either of the bone itself, or the bone marrow infiltration that occurs in leukemia.
2. A common cause of generalized lower extremity pain in children is growing pains


When do growing pains typically occur

at night


tx of growing pains

-relieved quickly by massage or tylenol


The diagnosis of growing pains is a diagnosis of exclusion so ask about:

1. pain during the day
2. limping,
3. swollen or
4. warm joints,
5. waxing/waning fevers and
6. do a physical exam for joint ROM, LAD and hepatosplenomegaly.


Common bacterial causes of joint effusion/extremity pain

1. Sepsis
2. septic arthritis
3. osteomyelitis


Common viral causes of joint effusion/extremity pain

1. parvovirus
2. EBV
3. reactive arthritis


Malignancy that cause joint effusion/extremity pain

1. Leukemia
2. Neuroblastoma
3. Lymphoma
4. Rhabdo
5. Osteosarcoma
6. Ewing's


Rheumatic disease that cause joint effusion/extremity pain

1. Rheumatic fever
2. SLE
3. Dermatomyositis
4. Vasculitis
5. mixed CT disease


DDX for joint effusion/extremity pain

1. infectious diseases
2. Malignancy
3. Rheumatic diseases
4. Growing pains
5. Complex regional pain syndrome


Presentation of transient synovitis of the hip

1. acute onset of difficulty walking/weight baring
2. holding affected leg rotated out
3. Well appearing but hx of recent URI sx w/ or w/o fever
4. complain of groin pain for unilateral hip pain


Who typically presents with transient synovitis of the hip?

1. 3-6y/o
4. Male predominance


What does the PE show for transient synovitis of the hip?

1. difficulty weight bearing and a definite limp to the affected side.
2. In younger children, they may refuse to weight bear thus limiting the exam of their gait.
3. limited internal rotation of the affected side, compared to the unaffected side


Treatment for transient synovitis of the hip?

1. supportive-- usually resolve in 1 week (67% at 1 week and 88% of children resolving in 4 weeks)
2. ibuprofen dosed at 40-50mg/kg divided TID is effective at reducing inflammation


When a child presents with an acute onset of limp or refusal to weight bear, in the presence of fever, a provider’s first concern is always a ___.

septic joint


While any joint can become septic, the most common affected joint in children is the __



How does one develop a septic joint?

bacteria enter the synovial space through hematogenous spread or sometimes from trauma or a contiguous infection


Why is a septic joint a surgical emergency requiring incision and drainage?

1. , as the increased pressure in the joint capsule compromises blood flow in the femoral head, leading to avascular necrosis.
2. Additionally, the enzymes in the bacterial and PMN’s are damaging to the articular cartilage.


Presentation of a septic joing

1. rapid onset of limp pain in an ill appearing child
2. affected leg is held rigidly and
3. they will have major resistance to any ROM


Imaging for a septic joint

X-rays or ultrasound might prove helpful, as they may show the widened joint space


Labs for a septic joint

1. CBC- elevated WB w/ L shift
2. elevated ESR and CRP
3. blood culture- as it will be positive for the offending organism 50-70% of the time
4. Joint aspiration w/ cell count, gram stain, and culture


Common organisms that cause septic arthritis

1. The most common organism is S. aureus followed by GAS and S. pneumoniae
2. Group B strep and N. Gonorrhea should be considered in neonates.
3. Adolescents may also have N. Gonorrhea and if they use IV drugs, gram negative bacteria are a possibility.


Tx of septic joints

1. surgical emergency with incision and drainage
2. Antibiotics are started empirically, pending the culture and sensitivity results, and therapy continued for 3-4 weeks.


__ is a multi-system disease with affected persons having a different constellation of symptoms, all characterized by remissions and exacerbations.



Who most commonly gets SLE

1. female predominance
2. AA>asian>lation>caucasians
3. peak onset: late adolescence to young adult


cause of SLE

1. unknown, although there is a positive FH 10% of the time, and 13% of the time patients have another autoimmune disorder.
2. Sex hormones may play a role, as onset of disease is usually in reproductive years and pregnancy and OCP exacerbate SLE.


The primary pathological findings in patients with SLE are those of

1. inflammation,
2. vasculitis,
3. immune complex deposition, and
4. vasculopathy


What is the criteria for SLE

*must have 4 criteria-- Some patients with only 2-3 symptoms may be considered “lupus-like” and still benefit from treatment.

1. Malar rash
2. discoid rash
3. photsensitivity
4. oral/nasal ulcerations (typically painless)
5. serositis
6. arthritis
7. nephritis
8. CNS diseas
9. Hematologic abnormalities
10. ANA positivity
11. Positive SLE serologies


The malar rash, aka “butterfly rash”, encompasses the

cheeks and nose, but spares the nasolabial folds.


What does the discoid rash in SLE look like

is rare in children, but are raised, red patches with scaling and plugging of hair follicles.


Where do the ulcers typically present in SLE

Painless ulcerations may occur in the mouth, usually the hard palate but can also occur on the nasal septum


Other sx of SLE that are not necessarily specific criteria

1. vasculitis (in the fingers)
2. fixed, lacy rash of livedo reticularis
3. fever
4. decreased appetite/wt loss
5. fatigue
6. pulmonary hemorrhage (especially in CO)
7. restrictive lung disease
8. gastritis
9. mesenteric arteritis
10. peritonitis
11. hepatitis
12. pancreatitis
13. CV findings


Serositis (in SLE) may often present as

pleuritis or pericarditis


Arthritis is SLE usually involves

2 or more peripheral joints and is not destructive.


Nephritis (in SLE) consists of

1. 3+ proteinuria,
2. hematuria,
3. cellular casts in the urine,
4. possibly nephrotic syndrome

*may progress to RI HTN or renal failure


CNS manifestations seen with SLE

1. seizures
2. psychosis
3. strokes
4. chorea
5. HA
6. pseudotumor
7. depression/anxiety

*but these are not diagnostic


Hematologic abnormalities seen with SLE

1. Coombs + hemolytic anemia
2. leukopenia (less than 4K)
3. thrombocytopenia (less than 100K)


___% of patients with SLE will have a positive ANA titer, so it is a highly sensitive test for SLE, unfortunately it has very poor specificity and is positive for reasons other than SLE, incl. __% of healthy kids.




Positive SLE serologies include

1. anti-double-stranded DNA antibody,
2. anti-Smith antibody, and
3. antiphospholipid antibodies.


cardiovascular findings sometimes found with SLE

1. myocarditis
2. endocarditis
3. premature MI
4. Raynaud's phenomenon



1. Infections (subacute bacterial endocarditis, Gonorrhea, EBV)
2. Malignancy (Lymphoma, leukemia)
3. Rheumatic (rheumatic fever, JIA, mixed CT disease, dermatomyositis, Scleroderma)

*SLE is a disease that is helpful to keep, even peripherally, on your differential of multiple presentations and keep considering if the patient exhibits any other criteria.


Laboratory workup for possible SLE in primary care

1. probably limited to tests which look for the presence of other criteria for diagnosis
2. i.e. CBC,
3. U/A with microscopy.
4. Prior to obtaining an ANA titer or SLE-specific serologies, consider consulting a pediatric rheumatologist for guidance.


After diagnosis of SLE, __ are used to monitor disease activity and drug side effects.



After diagnosis of SLE, labs are used to monitor disease activity and drug side effects. These include

1. CBC,
2. ESR,
3. UA,
4. double-stranded DNA, and
5. complement (C3,C4) as SLE is a complement-consuming disease


treatment of SLE

1. of avoidance of sun exposure,
2. use of > 30spf sunscreen,
3. hydroxychoroquine,
4. NSAIDs,
5. prednisone, and
6. possibly cytotoxic drugs.


The medications for SLE have significant side effects including an increased risk of __



The prognosis for SLE is ___% 10 year survival rate, about __% for 20 year survival

over 90%



Predictors of mortality for SLE include

1. race, with african and asian ancestry worse than caucasian,
2. the presence of renal or CNS disease
3. infection
4. GI bleeding
5. MI
6. malignancy


The leading cause of death for SLE is__.


* Prior to dialysis and transplant, renal disease was the leading cause


Additional morbidities of SLE include

1. osteoporosis,
2. infertility and
3. psychosocial impairment.