What is the new name for juvenile rheumatoid arthritis?

Juvenile idiopathic arthritis (JIA)

What is required to make a diagnosis of JIA, in terms of age & duration of symptoms?

Began before 16 years old

Arthritis in at least one joint lasting more than 6 weeks

What are the three types of JIA?

1. Oligoarticular (few joints – <4)

2. Polyarticular (many joints – >4)

3. Systemic (spikes fevers)

(Remember this is based on the presentation in the first 6 months for the polyarticular form)

What is the old name for oligoarticular JIA?

“pauci” articular JIA

(same meaning, terminology just adjusted)

How is polyarticular juvenile idiopathic arthritis (JIA) determined?

Presentation in the first 6 months after onset of the disorder

Systemic arthritis is most likely an autoimmune disorder. Which cytokines are especially important in “systemic” JIA?

IL-6 & IL-1

What is the characteristic pain/activity pattern for JIA?

(2 aspects)

Morning stiffness that improves with daytime activity

&

Worsens following rest!

Is loss of developmental milestones a feature of JIA?

It can be

(if pain or joint changes without pain limit the child’s physical capabilities)

In oligoarthritis JIA, do most children present with large joint involvement, or is it small joints?

Large joints

Positive ANA is most common in which sort of JIA?

Oligoarticular

How can you remember that ANA mainly goes with oligoarticular JIA?

Think of a girl named ANA, who has her name printed on her T-shirt, sitting on a chair with a swollen knee and elbow

Are oligoarticular JIA patients likely to have a positive rheumatoid factor?

No.

(Aside from elevated ANA, the other studies are usually normal)

Which patients with pediatric arthritis are at highest risk for uveitis & the related complications?

Oligoarticular JIA & psoriatic arthritis

Why are the ophthalmological complications of oligoarticular JIA a big deal?

Uveitis can lead to cataracts, glaucoma, & blindness

(many uveitis patients are asymptomatic, but need to be closely monitored for ocular damage)

If a patient with polyarticular JIA has facial growth abnormalities, how might that be related to the JIA?

Polyarticular can affect the axial skeleton – temporomandibular joint involvement can lead to micrognathia

Although positive rheumatoid factor only occurs in about 10 % of polyarticular JIA patients, what is its significance?

Bad prognosis – aggressive management will be needed

(likely to persist into adulthood)

How can you remember that RF is associated with polyarticular JIA?

It’s RuF (rough) to have so many joints affected

Uveitis is a common complication of what type of JIA?

Oligoarticular

(especially in females with + ANA )

Which HLA type is associated with psoriatic arthritis & enthesitis-related arthritis (such as ankylosing spondylitis)?

HLA – B27

Arthritis with anemia, but elevated white count and platelets suggests what diagnosis?

JIA

Arthritis with leukopenia & a photosensitive rash suggests which rheumatological diagnosis?

Systemic lupus erythematosus (SLE)

What are the typical ages of onset for the types of JIA?

Overall there are two peaks:

1–3 years & adolescence

(polyarticular has both peaks,

systemic is spread evenly across the age groups,

oligoarticular has just the early age peak 1–3 years)

(onset must be before age 16 to consider it any sort of JIA, though)

What is the typical age of onset for enthesitis-related arthritis is ________?

10–12 years

Like oligoarticular JIA, psoriatic arthritis typically begins in which age group?

1–3 years

Which gender is most likely to develop JIA?

Females

What physical findings are expected in systemic JIA?

(4)

Spiking fevers

Lymphadenopathy/HSM

Salmon-colored morbilliform rash

Arthritis (of course)

What is the first-line treatment for JIA?

NSAIDs

(physical therapy is also helpful)

What sorts of medications are second-line treatment for JIA?

Steroids & Immunosuppressants

If a child has systemic JIA, is the prognosis better when few joints, or many joints, are involved?

Few

Although it is unusual to have a positive rheumatoid factor with oligoarticular disease, what does it mean for prognosis when it’s positive?

Bad prognosis

RF+ JIA is most often seen in which age & gender group?

Adolescent females

Aside from uveitis, is it a good or bad prognostic to have a positive ANA with oligoarticular JIA?

Good

It is unusual for JIA patients to have a positive rheumatoid factor. Which type of JIA is most likely to have a positive RF?

Polyarticular

(Bad prognostic)

Is a positive ANA a good or bad prognostic for any type of JIA patient?

Good

(relatively common in both pauci and polyarticular disease, but most common in oligoarticular patients)

How common is systemic JIA?

Fairly uncommon –

About 10 % of JIA patients

How many joints are affected in systemic JIA?

Variable

What types of joints are affected in systemic JIA?

Any type can be affected

Uveitis and mucosal ulcers (oral and urethral), make up the findings in what rheumatological syndrome?

Behcet’s

(arthralgia, but not generally arthritis, is also seen)

Is the constellation of uveitis with oral & genital ulcers associated with a particular HLA type?

Yes.

HLA – B51 (Behcet’s syndrome)

How can you remember the antibodies that go with Sjogren’s syndrome?

Sjogren’s patients have irritated eyes – they might “RoLa” eyes in annoyance with all the burning & itching

What is the main problem in Sjogren’s syndrome?

Everything is dry – glands are infiltrated with lymphocytes –Dry eyes, dry mouth

How is Sjogren’s syndrome treated?

Immunosuppressants/corticosteroids and artificial tears

(good oral hygiene also helpful)

How is a Sjogren’s syndrome diagnosis confirmed?

Lip or salivary gland biopsy

What two preliminary tests are often used when a diagnosis of Sjogren’s is suspected?

ESR (elevated) &

Schirmer test (measures amount of tear production)

Mnemonic: “Schirmer” sounds like “swimmer.” Reminds you that the “swimmer” test measures H₂O in the eye!

Is the glandular infiltration by lymphocytes in Sjogren’s syndrome painful?

No – the glands sometimes enlarge, but it is painless enlargement

In general terms, what causes Henoch-Schonlein purpura (HSP)?

Autoimmune vasculitis

(specifically, an IgA-related leukocytoclastic vasculitis)

leukocytoclastic vasculitis means that neutrophils invade & do damage to the (small) vessels

What are the usual triggers for HSP?

Viral infections or Grp A strep

(other bacterial infections can also act as triggers)

What is the characteristic pattern for the rash of HSP?

Dependent areas – usually buttocks and lower extremities

HSP patients have purpura – do they have thrombocytopenia?

No

In addition to the skin rash, what other organ systems are often affected when HSP occurs?

Renal

GI

Joints

(& occasionally CNS)

In addition to hematuria, what other renal problems do HSP patients sometimes develop?

Glomerulonephritis & Nephrotic syndrome

(in some cases, renal insufficiency or failure & renal-related hypertension)

What are HSP’s main GI symptoms & signs?

Pain, vomiting, & bleeding

HSP patients are at risk for what serious cause of episodic abdominal pain?

Intussusception

(vasculitic lesions in the gut can serve as a lead point)

What is the prognosis for patients with HSP?

Great – goes away in 4–6 weeks

Does HSP require treatment?

No – supportive care & removal of suspected triggers, when possible

Steroids & other immunosuppressants are sometimes used, especially for severe disease, but current evidence regarding efficacy is not adequate for a clear answer.

(short-term prednisone has not been shown to improve renal disease)

What muscle symptoms do patients with dermatomyositis complain of?

Weakness (mainly in the proximal muscles)

Will dermatomyositis patients have elevated CK?

Yes (the problem is in the muscle, not in the nerves innervating them – this means CK will be up)

Does dermatomyositis present acutely or gradually?

Gradual onset

What are the two “famous” buzzword findings associated with dermatomyositis?

1. Heliotrope rash (purplish rash around the eyes)

2. Gottron papules over the small joints of the hands (looks like a red rash)

What nail finding goes with dermatomyositis?

(2)

Nailbed telangiectasias

&

Periungual erythema

How do infants develop neonatal systemic lupus erythematosus?

Antibodies passed from Mom to fetus – especially in mothers carrying anti-Ro/SSA antibodies

(Not all of the Moms who pass the antibodies are known to have lupus, however. Some develop it later, but not all of them.)

Do infants with neonatal SLE have any characteristic skin findings?

Yes –

Scaly, red, annular rash on face & upper body

70 % will have skin findings – 2/3 of those will have skin findings at birth, the rest develop over the first few months of life

Aside from skin changes, should you expect other findings on physical exam of an infant with neonatal SLE?

Hepatosplenomegaly

What is the natural course of neonatal SLE?

Resolves over time

(except for the most serious problem)

What consequence of neonatal SLE can be life threatening to the infant?

Heart block

This problem will not resolve, and often requires cardiac pacing

What are the possible cardiac effects of neonatal SLE?

(3)

Pericarditis/myocarditis

Cardiomyopathy & heart failure

Heart block

How common is it for infants with neonatal SLE to have cardiac effects?

Common – about 2/3 will have them

Children and adults with SLE often have hematological changes. Are any hematological changes seen in neonatal SLE?

Yes – near pancytopenia with anemia, thrombocytopenia, and neutropenia

How is neonatal SLE treated?

Steroids, if needed

Pacing, if needed

Otherwise supportive

What is the other name for the anti-Ro and anti-La antibodies, respectively?

Anti-SSA

Anti-SSB

SLE is more common in African Americans than it is in Caucasians. Is there an increased incidence in any other ethnic groups?

Yes – nearly all of them

(Native American, Asian, & Hispanic)

In which body cavities do lupus patients tend to build up fluid where it doesn’t belong?

Pericardial sac & pleural membranes

In addition to the butterfly malar rash, what other skin abnormalities are common with lupus?

(2)

Photosensitivity

&

Discoid lesions

If lupus affects the CNS, what sorts of problems does it create?

Many, the most common are –

Headaches (including migraine)

Neuropsych syndromes

Seizures

How many neuropsychiatric syndromes are associated with SLE, in the current SLE case definitions?

Note: This is a change!

Nineteen –

Previously only seizures & psychosis were included

What sorts of problems is the peripheral nervous system vulnerable to, with SLE?

Demyelinating disorders

Peripheral neuropathies

What are the main complications of SLE?

(5)

1. Renal failure

2. Increased infections

3. Cardiovascular disease

4. Neurological problems

5. Clot-related problems

Why might SLE patients have lowered immunity to infections?

Leukopenia & lymphopenia

+

immunosuppressant medications

How is SLE treated?

Steroids,

Hydroxychloroquine,

& Cyclophosphamide

(for severe disease)

What are spondyloarthropathies?

Ankylosing spondylitis

Reactive arthritis (previously Reiter’s syndrome)

Enteropathic arthritis (IBD related)

Psoriatic arthritis

How can you differentiate general low back pain due to muscle strain from the low back pain of the spondyloarthropathies?

Spondyloarthropathies get better with movement & worse with rest

What other body system is sometimes affected in spondyloarthropathy patients?

The eye (uveitis/iritis)

(Uveitis/iritis is seen with most HLA-B27 related disorders)

Are the ulcers of Behcet’s syndrome painful?

Yes (both in the mouth and on the genitals)

Is belly pain a part of Behcet’s syndrome?

It can be – GI ulcerations also occur

Other than the funny appearance, will kids with dermatomyositis complain about the rash?

Often, yes – it can be itchy

What sort of symptoms does the proximal muscle weakness of dermatomyositis cause?

(3 categories)

“Clumsiness”

Difficulty climbing stairs or rising from chairs

Difficulty dressing

Difficulty swallowing or voice change

What cutaneous-related finding causes significant morbidity in dermatomyositis?

Calcinosis cutis –

Calcium deposits in the skin linked to pain, skin ulceration & atrophy, nerve entrapment & contractures

(1/3 of patients develop calcinosis cutis)

How do dermatomyositis patients present, overall?

Rash

Proximal muscle weakness

Myalgia & athralgias

How is dermatomyositis treated?

High-dose steroids

Immunosuppressants including methotrexate & cyclophosphamide

IVIG

What environmental exposure is a problem for dermatomyositis patients?

Sunlight (photosensitive)

Must use sunscreen to avoid burns, worsening rash, & sometimes also worsening muscle weakness!

Reactive arthritis (previously called Reiter syndrome) is highly associated with chlamydial infections in adolescents & adults. In kids, which infectious agents are more common triggers?

Enteric pathogens

(Yersinia, Shigella, Salmonella, & Campylobacter typically)

(FYI – Giardia, & C. diff can also trigger it)

What is the famous mnemonic for reactive arthritis?

“Can’t see,

  can’t pee,

    can’t climb a tree.”

How long after the trigger infection does reactive arthritis usually appear?

1–4 weeks

Which of the pediatric rheumatological diseases are more common in males?

Enthesitis-related arthritis &

Reactive arthritis

Behcet’s syndrome (depending on location)

(the others are generally more common in females)

Why might a juvenile systemic sclerosis (commonly known as scleroderma) patient have pulmonary complaints?

It can invade the lungs, causing pulmonary fibrosis

What are the most common types of systemic sclerosis in children?

Diffuse cutaneous systemic sclerosis

&

Localized scleroderma (aka morphea)

What is the most typical presentation for a scleroderma patient?

Raynaud’s syndrome

(fingers that turn white, then blue, then red)

Not all Raynaud’s patients have scleroderma, however!

If an ichthyosis patient is diagnosed at birth, and later is found to have intellectual disability with spastic limbs, what syndrome might that be?

Sjogren-Larsson Syndrome

(autosomal recessive disorder of lipid metabolism – not related to regular Sjogren’s at all)

What is “serositis?”

Pericarditis, peritonitis, and/or pleuritis

(seen with SLE – any one or more of the three may be seen)

If SLE is suspected, but you check the ANA and it is negative, what should you conclude?

Probably not lupus.

What systemic complaints do SLE patients often have?

Fatigue, malaise, weight loss, fever

What is the most sensitive test for SLE?

ANA – it is good for screening because it is sensitive, but there will be many false positives

What is the most specific test for lupus?

Anti-Smith antibody

In addition to scleroderma, which other rheumatological disorder very often has Raynaud’s phenomenon as part of the disorder?

SLE

Which antibodies cause heart block in neonatal SLE?

Anti-Ro

(also known as anti-SSA)

Why would a lupus patient have a seizure?

Lupus cerebritis –

Vasculitis affecting the brain

If a patient has rheumatological complaints or findings, and an autoimmune hemolytic anemia , which rheumatological disorder should you think of?

SLE

(can also have anemia of chronic disease)

What is the second-line treatment for mild SLE, if NSAIDs are not sufficient?

Hydroxychloroquine

(Plaquenil™)

What are the main side effects of the second-line treatment for SLE?

Ocular complaints (blurring & retinal damage) & ototoxicity

What commonly occurs when tapering SLE steroid doses?

Flare-up of the disease symptoms

(usually not a reason to increase the steroids, though)

How long after infection with Lyme disease will serum antibody be detectable? What does this mean for testing?

4–6 weeks

Expect negative results early in the disease & treat clinically

If a patient is being treated for Lyme disease, and suddenly develops fever, shaking chills, and back pain, what should you suspect?

Jarisch-Herxheimer reaction – supportive care will get the patient through it

(caused by lysis of the little critters with successful treatment – historically seen with syphilis treatment)

It is common for the boards to present cases with a positive rheumatoid factor. Should you rely on that as an indicator of JIA?

No – not reliable

If the boards describes a child with a limp due to leg pain that is “worse at night,” and without any joint swelling or rash, what are they probably describing?

Growing pains

A child is described with frequent joint sprains and “loose joints.” What should you suspect?

Hypermobility – counsel regarding stretching and appropriate sport activities, but no treatment needed

When are steroids typically used in the treatment of JIA?

Cardiac involvement or failure with NSAID regimens

When are steroids typically used in the treatment of JIA?

For quick improvement if NSAID regimens have failed

What is the usual NSAID regimen for JIA treatment?

M Melixicam

I Ibuprofen

N Naproxen

How is the pain of acute leukemia different from the pain of JIA?

Acute leukemia wakes the patient at night, and is not focused on a joint

What is the eponymic name for systemic JIA?

Still’s disease

(can also occur in adults, but then it’s “adult-onset Still’s disease)

What is the typical age for a Kawasaki’s patient?

18–24 months

Do Kawasaki’s patients have a positive ANA?

No

What GU symptom sometimes occurs with Kawasaki’s?

Sterile pyuria

(white cells in the urine without bacteria)

How can Kawasaki’s affect the CNS?

Seizures and meningitis (aseptic)

What is the typical CBC for a Kawasaki’s patient?

High white count

High platelets (especially ≥7 days after onset)

Aspirin is one part of the treatment of Kawasaki’s. How long should a child with Kawasaki’s continue ASA?

1–2 months –

Initially high dose (80 mg/kg/day) then low dose (5 mg/kg/day)

Which joints typically swell in Henoch-Schonlein Purpura (HSP)?

Knees and ankles

What is the nature of the joint involvement in HSP?

It is “periarticular,” meaning that it affects the tissue around the joints primarily, rather than the joint itself

What is the buzzword for the skin lesions of HSP?

Palpable purpura

(think of the vasculitis inflaming & fluffing up the vessels – that’s why you can feel it)

Which rheumatological condition tends to cause ileoileal intussusception?

HSP

(often harder to diagnose & correct, due to location)

How common is renal involvement in HSP?

About 50 %

(but not necessarily very bad renal involvement)

Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is most common in which ethnic group?

Caucasians

A 9-year-old boy presents with rash, fever, and arthralgias a few weeks after he was successfully treated for a snake bite. What is wrong with him?

Serum sickness

(Type 3 hypersensitivity reaction – antivenins sometimes induce this response)

What is the most common organism to cause septic arthritis in kids older than 2 years?

Staph aureus

If the boards presents a single, inflamed joint, and you can’t tell whether it’s septic arthritis or not, what should you do?

When in doubt, or if it can’t be determined from the info given, treat for septic arthritis

(there is no time to lose – the board wants to make sure that you know this)

If you suspect rheumatic fever on the boards, what must be confirmed?

Documented history of strep infection

If a child has a negative rapid strep test, does this rule-out a recent strep infection/rheumatic fever?

No

What are acceptable ways to document strep infection?

ASO titer or streptozyme

(& culture, of course)

Emotional lability, along with purposeless rapid movements and muscle weakness, following an upper respiratory infection, suggests what diagnosis?

Sydenham’s chorea

(complication of strep infection)

How is the arthritis of rheumatic fever most likely to be described?