RHEUMATOLOGY Flashcards
1
Q
A patient has high spiking fevers daily for the last 2 weeks. What is your differential diagnosis? (5)
-workup?
A
- Infection
- Kawasaki disease
- Periodic fever syndromes
- Malignancy (especially leukemia and lymphoma)
- Systemic JIA
Work-up:
- Full infectious workup
- Bone marrow aspirate PRIOR to starting corticosteroid treatment for query JIA
2
Q
A patient presents to you with dactylitis. What is your differential diagnosis? (3)
A
- Sickle cell anemia
- JIA (ie. enthesitis related, psoriatic arthritis)
- Trauma
3
Q
A patient presents to you with isolated Raynaud phenomenon. What are the 2 best predictive factors for future development of autoimmune diseases?
A
- Positive ANA
- Abnormal nail fold vasculature
4
Q
A patient presents to you with leg pain that occurs solely at night. What is your differential diagnosis? (3)
A
- Malignancy: osteosarcoma, Ewing sarcoma
- Osteoid osteoma (benign)
- Growing pains
5
Q
Antibiotic choice for osteomyelitis/septic arthritis:
- neonate
- 1-3 month old
- child
- teenager -sickle cell
A
-Neonate: Cloxacillin and Gentamicin
-1-3 month old: Cefuroxime (because of more gram negatives)
- Child: Cefazolin
- Teenager: ceftriaxone OR cefixime + azithromycin
- Sickle cell: cefotaxime (salmonella coverage)
6
Q
What is the diagnostic criteria for Behcet disease?
A
Needs:
- Recurrent oral ulcers AND 2 or more of the following:
a. recurrent genital ulcers
b. eye lesions (uveitis)
c. skin lesions (erythema nodosum, pseudo vasculitis, etc.)
d. Pathergy (skin papule 2 mm or more in size developing 24-48 hrs after insertion of 20-25 gauge needle into the skin)
7
Q
Differential diagnosis for fever and limb pain?
A
Reactive: -arthritis -transient synovitis Infections: -Lyme disease -Viral Malignancy Inflammatory
8
Q
Features of transient synovitis vs. septic arthritis?
A
Septic arthritis:
Clinical;
lots of pain, fever,
MARKED RESISTANCE TO MOBILITY AND SWELLING/WARMTH OF JOINT
Labs: elevated ESR/CRP, elevated WBC,
Aspiration: positive gram stain or culture of joint fluid, key is
Transient synovitis:
Clinical will still be active but limping, looks well, can elicit range of motion in joint if done gently, can have low fever or afebrile,
Labs normal WBC, mild pain usually, normal or mildly elevated ESR/CRP
****Remember that transient synovitis is a diagnosis of exclusion
9
Q
How does colchicine work in FMF?
A
Binds to microtubules to prevent activation, proliferation and functioning of inflammatory cells
10
Q
What are the clinical features of familial mediterranean fever? -hallmark?
-treatment?
A
- Fever episodes x 1-3 days q4-8 wks (very predictable)
-
Clinical:
a. Serositis - CLINICAL HALLMARK = peritonitis, pleuritis, synovitis
b. Skin: erysipelas-like rash on shins and dorsum of feet
c. MSK: monoarthrtis, myalgia
N.B. cause of AA-Amyloidosis without predisposing disease,
Treatment: colchicine
- Diagnosis: typically clinical (recurrent short fevers in right demographic, accompanied by abdominal/chest/skin/joint manifestations.
- Tel HashomerCriteria: diagnosis with > 2 major criteria, or 1 major and > 2 minor:
- Major: recurrent fever with serositis, AA-Amyloidosis without predisposing disease, favorable response to colchicine treatment.
- Minor: recurrent febrile episodes, erysipelas-like erythema, FMF in a first-degree relative.
11
Q
FMF What is the epidemiology of FMF?
Genetics, race, age
A
Most common monogenic fever syndrome
Autosomal recessive mutations in the MEFV gene on chromosome 16p
Most common in Ashkenazi Jewish, Arab, Turkish, and Italian populations.
Onset of disease typically in adolescence or childhood (> 90% start before 20y)
12
Q
How long to treat with IV antibiotics in osteomyelitis/septic arthritis?
A
1. iv Abx: Until fever resolves > 24 hrs, pain completely resolved (may need up to 4-6 weeks);
- then switch to PO abx x 2-3 wks
3. Monitor CRP and ESR for improvement
13
Q
What does the prognosis of HSP depend on?
A
Depends on severity of nephritis (worse prognosis with nephrotic syndrome)
-end stage renal disease occurs in 1-3% of patients
14
Q
What is the diagnostic criteria for HSP?
A
Need: Palpable purpura or petechiae with lower limb predominance AND 1 or more of the following:
- Diffuse colicky abdominal pain with acute onset (can include intussusception, GI bleed) = 50-75% of patients
- Skin biopsy showing IgA deposits in leukocytoclastic vasculitis OR kidney biopsy showing proliferative glomerulonephritis with IgA deposits (THIS IS REQUIRED IF PURPURA IS IN ATYPICAL DISTRIBUTION)
- Arthritis- oligoarticular or arthralgia of acute onset (75% of patients usually knees/ankles with edema)
- Renal involvement: proteinuria, hematuria impaired renal function
- CNS: headache, fits or ataxia
15
Q
What is the most common vasculitis in children?
A
HSP
16
Q
What are the diagnostic criteria for juvenile dermatomyositis? (5)
- what can be seen on capillaroscopy?
- what organ systems aside from MSK/neuro are involved?
A
-
Myositis: (you need 2 of the following)
a. Symmetrical proximal muscle weakness
b. Elevated muscle enzymes (CK, AST, LDH, aldolase)
c. Abnormal EMG demonstrating denervation and myopathy
d. Abnormal muscle biopsy demonstrating necrosis and inflammation
e. MRI - proximal muscle inflamtion -
Characteristic skin changes: NEEDED IN CRIETERIA
a. Gottron’s papules on dorsal surface of the knuckles,
b. heliotrope rash over the eyelids
c. Shawl - V sign sun areas
d. Ulcerations at Gottrons papules/corners of eyes
e. Calcinosis in subcutaneous and deep nodules - Joint: Polyarticular arthritis/joint contractures
-
Vascular;
a. Dilated/tortuous nail fold capillaries (tortuosity, dilatation, dropout)- capillaroscopy
b. Raynauds:
5. Other organ systems:
a. Cardiac: cardiomyopathy (rare)
b. Interstitial lung disease ( poor prognostic sign)
c. GI tract symptoms: GI vasculopathy ranges on a spectrum from abdominal pain to ischemic bowel (with perforation and ulceration described).
d. Lipodystrophy: loss of subcutaneous fat, accompanied by metabolic syndrome.
17
Q
JDM WHAT ARE THE INVESTIGATIONS YOU CAN ORDER?
A
- Muscle Enzymes:at least one is elevated in 80-98% of patients at time of diagnosis
- CK, AST, ALT, and LDH are main ones we order || Aldolase helpful but not done in most institutions
- CK typically elevated to the 1.5-15x range (contrast DMD typically much higher)
- Does not necessarily correlate with disease activity
- Can be falsely normal with progressive inactivity or severely delayed diagnosis
- vWFAntigen: marker of endothelial injury, and elevated in vasculopathy / vasculitis
•Is the best marker of disease activity and early flare
- Other Biomarkers:ESR > CRP; low complements on occasion; high IgG on occasion
-
Other Rheumatology Things:ANA, dsDNA, ENA, UA, etc…
- ANA elevated in 40-70%
- ENA positive in up to 75% (RNP, SSA, PM-Scl)
Jo-1 (2-5%): prototypicantisynthetaseantibody, associated with weakness, ILD, arthritis, and RP. High mortality.
Reference only!
Mi2 (2-13%): mild JDM with rashes and high CK; more common in Hispanics.
SRP (1%): acute and severe weakness, cardiac disease, and RP; refractory; more common in African-American girls.
Jo-1 (2-5%): prototypic antisynthetas eantibody, associated with weakness, ILD, arthritis, and RP. High mortality.
18
Q
JDM What is the acute management of JDM?
A
- Consider admission, NPO & with IV fluids, 1:1 nursing care, and full CRM to assess cardiopulmonary weakness-may have respiratory muscle weakness; as such they may be unable to manifest tachypnea and will drop their minute ventilation rapidly. Strongly consider a baseline gas and reassess as needed to prevent need for intubation.
- Other Services: OT/PT for rehabilitation, SLP for swallowing assessment (clinical determination), and nursing for support / care.
3. Medication:
a. Corticosteroids: typically give a pulse in all but the most mild of cases, followed by a very slow taper.
b. Methotrexate: Gold standard for the management of JDM, recommended in all cases that don’t require more aggressive treatment. Minimum of 2-3 years. SQ is preferred due to concerns of absorption with subclinical GI vasculopathy.
c. IVIG: Gold standard for severe cutaneous disease; used in addition to methotrexate and steroids, not in lieu of.
d. Cyclophosphamide: used for the more severe or refractory disease manifestations, particularly those requiring ICU admission or with progression while on methotrexate.
e. Rituximab:considered at same level (or as next step, depending on algorithm) to cyclophosphamide; may be more useful in antibody-positive disease.
f. Other:Anti-TNF biologics, Imuran, MMF, and Cyclosporine are all considered for multiple refractory disease states.
19
Q
What is the treatment for juvenile dermatomyositis?
-what are the 2 best predictors of longer time to remission?
A
- Supportive: nutrition, physiotherapy, sunscreen for photosensitive rash
-
Medications:
a. -High dose steroids 1-2 mg/kg/day with slow taper
b. -Methotrexate milder disease
c. Hydroxychloroquine for skin
d. if refractory or resistant: IVIG, cyclosporine, or rituximab
-2 predictors for longer time to remission:
- Nail fold abnormalities 2. Persistence of rash
20
Q
What specific auto-antibodies (3) can often be seen with juvenile dermatomyositis?
A
- Anti-Jo1
- Anti-SRP
- Anti-Mi-2
21
Q
What level is considered a positive ANA?
-when should specific antibodies (ie. anti-dsDNA) be ordered?
A
ANA >1:160 Positive & low titre
ANA >1:340 Positive & high titre
-specific antibodies should only be ordered if ANA is positive AND a disease other than JIA is suspected (ie. SLE)
22
Q
A patient with JIA has a positive ANA.
What 3 things does this put them at risk for?
A
- Earlier disease onset
- Asymmetric arthritis (oligoarticular + psoriatic)
- Uveitis
23
Q
A patient with JIA or SLE suddenly becomes extremely unwell with fever, splenomegaly, and pancytopenia. What is the most worrisome possible diagnosis?
A
Macrophage activation syndrome
24
Q
A young female patient of yours oligoarticular JIA with positive ANA. What does she have a high risk of developing?
-what is the chance that a patient with oligoarticular JIA will have a positive ANA?
A
Asymptomatic uveitis -need ophtho consult
-positive ANA is found in 70% of patients with oligoarticular JIA
25
How useful is rheumatoid factor (RF) in diagnosis of JIA?
-implication for prognosis?
Not useful at all!
- present in 85% of adults with rheumatoid arthritis but only 5-10% of children with JIA
- helpful in classification and prognosis of JIA but should NOT be used as screening test since arthritis is a clinical diagnosis
- **RF positive JIA** = higher risk of aggressive joint disease with erosions and functional disability
- children with **RF negative** polyarthritis are typically younger and have a much better prognosis
26
What are 3 long term orthopedic complications of JIA?
1. Leg length discrepancy
2. Joint deformity
3. Flexion contracture
27
What are the 7 types of JIA?
-definition of JIA (2 criteria)?
JIA: needs to begin b**efore the 16th** birthday and **persists for \> 6 weeks!**!!
1. Oligoarthritis 2. Polyarthritis (RF negative) 3. Polyarthritis (RF positive) 4. Enthesitis related arthritis 5. Systemic JIA 6. Psoriatic arthritis 7. Undifferentiated arthritis
28
JIA What are the drugs used to treat it? (5)
1. **Corticosteroid Injection:** direct injection with steroid results in rapid improvement or resolution of arthritis, with effects lasting 3-24 months. Is rapid and inexpensive.
2 .**NSAIDs**: Most commonly recommend Naproxen because T1/2allows for BID dosing. Adverse effects are uncommon and usually preventable. Can see dyspepsia/GI ulcer, renal injury, and pseudoporphyria.
3 **Systemic Corticosteroids**:highly effective for short term management, extremely poor long-term monotherapy. Long list of adverse effects. Will sometimes be used to induce remission while awaiting proper treatment (MTX)
\*\*Please do NOT use without d/w rheumatology, as it will mask/treat joints\*\*
4. **Methotrexate:** Disease modifying anti-rheumatic drug (DMARD). First line therapyfor all extensive JIA or disease refractory to NSAID/injection. Used in low doses (1% of SE cfchemo)– is well tolerated, safe, and efficacious in 80% of patients.
**5.Other DMARDS:**Leflunomide, Sulfasalazine, Thalidomide, +/- Plaquenil.
29
What are the general goals of therapy for treatment of JIA? (3)
- what is the first line treatment of JIA?
- what subspecialties (2) should be consulted for all patients diagnosed with JIA?
1. Eliminate inflammation AND Prevent joint damage
2. Promote normal growth and function
3. Minimize medication toxicity
**-first line treatment:**
NSAIDs
**-two consultants:**
1. Rheumatology
2. Ophthamology to rule out uveitis (ESPECIALLY those with oligoarthritis and positive ANA)
30
What are the most common sites of involvement in enthesitis-related arthritis? (4)
**Insertion sites of:**
1. Quadriceps tendon
2. Patellar tendon
3. Achilles tendon
4. Plantar fascia
N.B. can also see dactylitis, tarsitis
31
JIA What are the SE of MTX?
Adverse effects include
1. **immunosuppression** (minor degree) + **Vaccines**: Cannot have LIVE vaccinations per current recommendations
2. **transaminitis** (reversible in vast majority),
3. **GI intolerance** / oral ulcers (manageable).
4. **Pregnancy**: Is extremely teratogenic while using it. Should avoid alcohol.
32
What is the algorithm for treatment of oligoarticular JIA?
1. Try NSAID --\> if no remission,
2. try intraarticular corticosteroid injection --\> if no remission,
3. consider **methotrexate** or other second line agent
33
What is the algorithm for treatment of polyarticular JIA?
Remember that this is more severe and causes worse functional impairment since it's multiple joints so you're more aggressive!
1. Try **NSAIDs or intraarticular corticosteroid (IAC)** injections --\> no response --\>
2. start second line agent right away (**methotrexate, leflunomide or sulfasalazine**) --\> if no response,
**3. optimize second line agent** and consider
4**. IAC or low dose PO steroids** as bridging therapy --\> if no response, consider
5. **biologic anti-TNF therapy**
\*\*\*May need to start a second line agent as part of initial therapy for children with severe polyarthritis
34
What is the algorithm for treatment of systemic JIA?
1. Try **NSAIDs** first OR for severe disease, consider starting **corticosteroids** or **biologic** agent (anti-IL-1 or anti-IL-6) right away
2. -if tried NSAIDs and no response --\> **systemic corticosteroids** --\> if no response --\>
3. add **biologic anti-IL-1 or anti-IL-6** therapy
\*\*\*\*note that you DO NOT use methotrexate/sulfasalazine/etc.
35
What is the definition of oligoarthritis?
-two subcategories?
Definition: arthritis affecting 1-4 joints during first 6 months of disease
- subcategories:
1. **Persistent**: no more than 4 joints affected throughout disease course
2. E**xtended:** more than 4 joints affected after first 6 months of disease
36
What is the diagnostic criteria for **psoriatic arthritis?** -what is the clinical hallmark of this condition?
1. _Arthritis AND psoriasis_ OR
2. Arthritis with at _least 2 o_f the following:
a. dactylitis (clinical hallmark!!!)
b. nail-pitting or onycholysis
c. psoarisis in a first degree relative
37
What is the diagnostic criteria for RF-negative-polyarthritis? -what about RF-positive polyarthritis?
**RF-negative polyarthritis:**
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. RF testing negative
**RF-positive polyarthritis:**
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. **_2 or more_** positive tests for RF at least 3 months apart during first 6 months of disease
38
What is the most common subtype of JIA?
-most frequent joints involved? (4)
Oligoarthritis
- most frequent joints involved:
1. Knees 2. Ankles 3. Wrists 4. Elbows
39
Which population is usually affected by RF positive polyarthritis?
**Adolescent girls** = this is essentially adult-type rheumatoid arthritis presenting in a child or adolescent
-usually involve the **PIP and MCP joints**
40
Which population of JIA patients are at highest risk for developing uveitis?
**ANA positive oligoarthritis**
41
A 3 yo Hawaiian boy comes to your ED with peeling of fingers and toes. He had 14 days of fever, red eyes, a swollen neck, a red tongue and fussiness. You would like to order an echo but can't get one for 24 hours. What should you initiate at this time?
**LOW DOSE ASPIRIN!!!** This is a patient in the convalescent phase of kawasaki disease and thus would not benefit from IVIG or high dose aspirin.
Once they are in the convalescent phase, they need low dose aspirin at 3-5 mg/kg/day.
42
What are less common clinical manifestations of KD (ie. clinical features other than those in diagnostic criteria)? (3 categories)
**1. CNS/Eyes:** Irritability (aseptic meningitis)/Uveitis
**2. Organs:**
a. **GIT:** Gastroenteritis (abdo pain, vomiting, diarrhea)/Gallbladder hydrops
b. **Nephro**: Sterile pyuria from urethritis
c. **Cardiac**: Myocarditis/pericarditis
d. **Sytemic**: MAS
**3. MSK:** Arthritis
43
JD What is the criteria for KD Dx?
Diagnosis: Requires (A) and at least 4/5 (B)
* (A) **Fever for \> 5 days (4 if IVIG eradicates fever)**
* (B) Any **4** of the following: **CRASH**
C- **Conjunctivitis**- Bilateralnonpurulentbulbar conjunctivitis (80-90%)
R- **Rash**- Polymorphous rash (\>90%) - nonvesicular(AHA clarifies to maculopapular, erythroderma, or EM like)
A- **Adenitis** Cervical LN (50%) - at least one node \> 1.5 cm.
S- **Strawberry** tongue: Oropharyngeal changes (80-90%) - red/fissured lips, strawberry tongue, pharyngeal erythema
H - **Hands/** Feet changes: Extremity changes (80%) - erythema / edema (acute phase), desquamation (convalescent phase)
44
What is the diagnostic criteria for: -incomplete KD -atypical KD -alternate way to make diagnosis for KD aside from the 5 or more days of fever + 4/5 features
**Incomplete KD:**
if 5 or more days of fever with **2-3 features** instead of 4 -commonly seen in **infants** who are at higher risk of coronary artery involvement
**Atypical KD:**
if KD with unusual manifestation (eg. renal failure)
Alternate way to diagnose KD: in presence of **fever** AND **coronary artery** involvement on echo, \<4/5 criteria is sufficient
45
What is the most worrisome complication of KD and when does this occur?
- risk factors for developing complication? (6)
- if untreated, what is the chance of developing this complication?
What does the risk change to if treated?
**Coronary artery aneurysms** = occur 6-8 wks after acute illness
**-risk factors:** 1. Males 2. Infants 9 yo 3. Prolonged fever 4. Asian or hispanic ethnicity 5. **Lab**: Thrombocytopenia or Hyponatremia
**-if untreated:** 25% will develop coronary artery aneurysms
**-if treated** (IVIG given within 10 days): decreased to 4%
46
What is the usual age group for KD? -what is the pathophysiology?
3 mo-5 yo
-**small vessel** vasculitis with predilection for **coronary arteries;** thought to be triggered by infectious agent
47
KD What is the criteria for Incomplete KD Dx?
Diagnosis may be made in the presence of
(A) Fever for \> 5 days and
**(B)** if the following scenario are met:
•E**levated ESR (40) / CRP (30)** AND
at least _3 supplementary criteria_ confirm diagnosis
i) **Labs:** Anemia, albumin \< 31, elevated ALT, sterile pyuria, leukocytosis, thrombocytosis (after day 7)
ii) **Echo**: Positive echocardiogram (alone) is sufficient
•If ESR/CRP not high enough, clinical observation warranted; if high enough, check labs and treat accordingly.
**ALTERNATE DX:**
1. Incomplete KD is alternatively diagnosed in any patient with **fever and any feature,** who then develops **CA dilatation.**
2. In **infants \< 6 months**, consider with **fever alone.**
48
List rsik factors for DDH (5 F's and 2 T's)
Name 5 risk factors for DDH **(5 F's and 2 T's)**
1. F:
Frank Breech, Oligohydramnios (uterine)
Female, First born
Family history of DDH
2. T: Twins, Tight swaddling
49
List the clincial findings in Rickets
1. **General**: FTT and developmental delay
2. **Neuro**: proximal muscle weakness, tetany, seizures, stridor
3. **Ortho**:
a. **Skull**: craniotabes, craniosynostosis, frontal bossing, delayed fontanelle closure
b. **Chest**: rachitic rosary, Harrison groove, scoliosis, kyphosis,
c. **Limbs**: fractures, bowed legs
50
MAS Name 4 causes? What is the management of MAS?
SLE, sJIA, KD, infection, changes in therapy
**Name 4 steps in your management plan:**
1. Consult rheumatology / hematology
2. Monitor & supportive management for hemodynamic instability – IV fluid resuscitation, ventilator support, etc
3. If clinically stable, treat underlying cause and monitor closely
4. If clinically unwell, treat based on available protocol (HLH-94 or HLH-2004)
51
PFAPA Investigations and treatment
**•Investigations:**
•May see elevated WBC, ESR, CRP, and elevated IgGAM\*
**Treatment:**
1. **Conservative**:PFAPA is not a life-threatening illness; A wait-and-see approach
**Natural history:** most children outgrow over 4-8 years, usually before age 10.
2. **NSAIDs:** anti-inflammatory dosing of Ibuprofen during episodes is safe / effective.
3. **Steroids**: arguably the **gold-standard** Either reduces Sx or 100% gone
* Initial dose **prednisone** 1 mg/kg q12-24h [at first sign of episode]; can increase to 2 mg/kg.
* In about 50% of cases, episodes will become more frequent- tolerant to steroid
4. **Colchicine**:rarely used, but in select cases can be given as prophylaxis. ..diarrhea
5. **Tonsillectomy**:alternate gold-standard in the management of PFAPA
52
What are the clinical features of PFAPA? -treatment?
**Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis** -usually starts **before 5 yo** and is self-limited
Clinical features:
1. Episodes of high **fever** that occur with regular periodicity **q4-6w** , last usually **up to 5 days**
3. non scarring aphthous **ulcers, pharyngitis, cervical adenitis** with negative throat cultures
**-treatment:** single dose of corticosteroid stops symptoms but really, no consensus regarding treatment
53
Pseudomonas coverage in nail penetrating injury?
Ceftazidime or Pip Tazo
54
Septic arthritis in the hip: clinical feature?
Hip is a deep joint, will not see effusion, erythema but will still have intense pain, pain on passive movement
55
Septic arthritis of which joint is a surgical emergency?
**Hip septic arthritis** = needs debridement in OR because of the vulnerability of the blood supply to the head of the femur
56
What 4 antibodies are SPECIFIC for SLE?
-what antibody is SENSITIVE for SLE?
**Most sensitive antibody:**
ANA (if negative, very unlikely to have SLE
**Most specific:**
1. anti-dsDNA (more common, less specific than anti smith and good as a marker of disease activity)
2. anti-smith (less common, more specific than anti-dsDNA)
3. anti-Ro
4. anti-La
57
SLE What are other agents for Rx?
* Management: Other Agents.
* **Plaquenil:** The gold standard for maintenance therapy – used in almost every flavor and variety of SLE. Has been shown to decrease flare rates and severity of flares when they occur. Considered in many a life-long therapy.Only significant adverse effect is Bulls-Eye Maculopathy, which can develop in those taking it for more than 5 years, and presents as reversible loss of color vision. Can be detected prior to onset with ophthalmologic screening and reversed with adjustment or discontinuation of plaquenil.
* **Sun** Safety:UV light can trigger DNA release and cause an SLE flare, even in those without cutaneous disease. All patients with SLE should be counselled on sun safety / clothing, and to use sunscreen liberally and regularly.
* **Rituximab**:used mostly in refractory SLE or in isolated / severe hematologic SLE.
* **Azathioprine** (**Imuran)**:used for milder disease, particularly class I/II GN, systemic disease, cutaneous disease, or vasculiticdisease. Also used as a maintenance agent and stepdown from cyclophosphamide. Main AE is cytopenia and intolerance. Can cause critical neutropeniain patients with TMPT deficiency.
**ASA**: sometimes used alongside other agents in the management of APLA
58
What are possible long term complications of SLE?
1. **Death** from infection, renal, CNS, cardiac or pulmonary disease
2. Early onset **coronary artery** disease from vasculitis
3. **Bone** disease --\> osteopenia, avascular necrosis from steroid use
4. **Malignancy** from use of immunosuppressants/biologics
59
What are the 5 clinical features of NLE (neonatal lupus)?
1. **Cardiac**: complete congenital heart block (associated with non-immune hydrops)
2. **Annular, erythematous papulosquamous rash** with fine scale and central clearing -usually photosensitive -can be present at birth or develop within first few weeks of life -predilection for face and scalp -self-resolving
3. **Hematologic**: thrombocytopenia (most common), neutropenia and anemia less common
4. **Hepatic** involvement: cholestatic hepatitis with elevated liver enzymes, hepatomegaly
5. **Neurologic**: macrocephaly, hydrocephalus, spastic paraparesis, vasculopathy
60
What are the diagnostic criteria for SLE?
- what do you see in complement level in SLE?
- what lab markers are used to monitor disease activity (2)?
**SOAP BRAIN MD** -remember that these are for adults and have not been validated in children so pediatric patients may be diagnosed with SLE without meeting diagnostic criteria
Need **4/11:**
1. **Serositis** (pleuritis or pericarditis)
2. **Oral** ulcers (usually painless)
3. **Arthritis** (non erosive)
4. **Photosensitivity**
5. **Blood** disorders: autoimmune hemolytic anemia, lymphopenia, thrombocytopenia
6. **Renal** nephritis: proteinuria or cellular casts
7. **ANA** positive
8. **Immunologic**: anti-dsDNA (increases clotting risk), anti-Smith, anti-ro, anti-la, antiphospholipid antibodies
9. **Neurologic** encephalopathy (seizures and/or psychosis)
10. **Malar** rash: butterfly rash sparing nasolabial folds
11. **Discoid** rash
**what do you see in complement level in SLE?**
\*\*\*Complement is LOW in SLE
**-what lab markers are used to monitor disease activity?**
C3 and C4 are used to monitor disease activity = levels fall during a flare and return to normal after appropriate treatment
61
SLE What are the **INDUCTION** treatment options for SLE?
Overall: use minimum required treatment to maintain clinical and laboratory quiescence. More aggressive treatment needed for severe organ involvement
**INDUCTION:**
**High-dose glucocorticoids and:**
**a.** **Cyclophosphamide** or **mycophenolate** for _proliferative nephritis_
b. Consider adding **Rituximab** for _refractory nephritis_ or _cytopenias_
c. **Cyclophosphamide** for _CNS involvement_
Other disease-modifying agents for ***mild*** disease (predominantly skin and joint manifestations), such as **mycophenolate, methotrexate, or azathioprine****_Notes:_**
**Corticosteroids**: often used in initial therapy for SLE (especially for severe lupus nephritis, hematologic crisis, CNS disease)
```
Corticosteroids:used almost universally in all types of SLE with end-organ disease. Topical used for cutaneous SLE. Not as helpful for systemic manifestations.
Renal disease (excluding the uncommon class I/II) typically get steroid pulse.
```
* **Cyclophosphamide**:the best treatment for SLE in terms of length of study and efficacy. Many adverse effects, including nausea/vomiting, SIADH, hair loss, and long-term infertility (up to 10% in pubertal females). Gold standard for NPSLE **(Neuropsychiatric systemic lupus erythematosus).** Advantage is it only needs to be given for 6 months (IV) then can switch to Imuran.
* **Mycophenolate** **Mofetil**:will likely be the universal gold standard in a few years, has shown non-inferiority to IV cyclophosphamide in even severe SLE, excluding NPSLE (because of low CNS penetrance). Given orally, and main AE are cytopenias and GI intolerance.
**Hydroxychloroquine**: standard therapy for SLE; decreases frequency and severity of disease flares
Azathioprine: used for hematologic and renal manifestations 4. Mycophenolate mofetil: used for hematologic, renal and CNS manifestations
Cyclophosphamide: used for severe renal and CNS manifestations
Rituximab: used for resistant thrombocytopenia
62
SLE What are the MAINTENANCE treatment options for SLE?
1. **Mycophenolate** or **azathioprine** for nephritis and CNS disease
2. All patients receive lifelong maintenance with antimalarials **(hydroxychloroquine)**
3. Sun avoidance
4. For drug-induced lupus, treatment is to discontinue the offending agent
63
SLE What are the treatment cplns?
1. **immunosuppression**: Early mortality from infectious complications due to immunosuppression
a. Pneumococcal and meningococcal infections due to low complement levels
b. Should receive 23-polyvalent pneumococcal vaccine
c. No live vaccines while on immunosuppressive medications
d. Opportunistic infections: Pneumocystis jirovecii, cytomegalovirus (CMV) reactivation, fungal infection
2. Consequences of **chronic glucocorticoid therapy:** secondary adrenal insufficiency, bone demineralization, metabolic syndrome, Cushingoid features, and so forth
3. **Hydroxychloroquine** can cause retinal toxicity
Need annual screening ophthalmology examinations
64
What are the two most specific tests for diagnosing SLE?
1. anti-smith 2. anti-ds dna
65
SLE What geenral lab test need to be done with SLE?
**•Investigations:** Equally important: ( ? IfPxhas end organ disease)- Rheum will see them ASAP if renal dysfunction
**1. CBC**: Any/all cell lines can be down; can mimic malignancy; can present as an isolated chronic ITP that later involves other cell lines.
**2. Creatinine, Urea, Urinalysis:** The most important test for SLE; even more important than dsDNA/ANA, should always evaluate for renal disease if you ever mention the word lupus.
* Prefer a first AM sample to rule out orthostatic proteinuria.
* If positive can refer to nephrology and complete work-up as they await triage.
**3. Complements:** Low C4 is notoriously common in SLE; Low C3 also common, but has a much broader differential so in isolation is harder to interpret.
**4. General chemistries, LFT's** : screening for end organ function, MAS, and differentials (malignancy, infection) depending on presentation
66
What is the treatment for NLE?
If **fetal bradycardia** is found during pregnancy, need fetal ECHO and may require treatment with **Dexamethasone** +/- **sympathomimetics**
-**neonates** with complete heart block may need pacemaker
67
What is the underlying pathophysiology of neonatal lupus erythematosus?
- in a mother with the specific autoantibodies, what is the incidence of NLE in their infants?
- what is the risk of recurrence for future pregnancies if one child has been affected?
Transplacental passage of maternal autoantibodies (**anti-Ro and anti-La)** = remember that mothers of infants with NLE MAY have SLE but also may be healthy with no known autoimmune disease
- **incidence**: 1-2% of children with mothers with anti-ro or anti-la antibodies
- **risk of recurrence**: 16%
68
What are the clinical features of takayasu arteritis?
- diagnostic criteria?
- treatment?
**clinical features of takayasu arteritis**
Large vessel vasculitis involving the **aorta** and its branches (thoracic, abdominal, carotid)
-initially presents as non-specific inflammatory illness with **fever**, then evolves into chronic, fibrotic phase with signs and symptoms of c**hronic vascular insufficiency** (pulse deficit, claudication, BP discrepancy, bruits)
\*\*\*Diagnostic criteria: Needs:
1. **Angiographic abnormalities (MRI) of aorta** or its main branches (aneurysm/diltation, narrowing or thickened arterial wall) **AND** 1 of the following:
1. Pulse deficit or claudication
2. Discrepancy of 4 limb BP \> 10 mm Hg
3. Bruits
4. Hypertension
5. Acute phase reactants elevated (ESR or CRP)
**-Treatment:**
**corticosteroids** plus second line agent: **cyclophosphamide, methotrexate**, etc.
69
Takayasu What is the Rx?
•**DDx:**
fibromuscular dysplasia, Marfan/Loeys-Dietz, Kawasaki, other vasculitis
•
**Treatment:**
**1. corticosteroids** are used for induction;
2. **Maintenence standard of care** has typically been **methotrexate**, but relapse rates \> 50%. New evidence favoring **Tocilizumab** and is Calgary standard of care.
•
**Clinical Pearl:**high overlap with TB (as etiology vs. differential).
**Common manifestations** include
**Systemic**( fever, weight loss),
**Arthralgia**, myalgia,
- **CNS**: claudication, headaches (80%), stroke,
**CVS**: chest pain, hypertension(\> 80%), abdominal pain (ischemic).
70
Top common causes of septic arthritis in
Children? (3)
In neonates?
In sickle cell?
In teenagers?
**Children:**
1. Staph aureus
2. Strep pneumonia
3. Non Group A strep
4. Kingella Kingae
**In neonates:** GBS and gram negatives
**Sickle cell:** Salmonella
**Teenagers:** gonorrhea
71
What is the diagnostic criteria for Wegener Granulomatosis?
**Granulomatosis with Polyangiitis (GPA)**
Inv, Dx and management?
\*\*\*Overall, think small vessel vasculitis primarily affecting upper airway, lungs, and kidneys!!!!
Need 3/6:
1. **Histopathology** showing granulomatous inflammation within wall of artery
2. **Resp**:
a. **Upper airway involvement**: chronic purulent or bloody nasal discharge, recurrent epistaxis, nasal septum perforation, saddle nose deformity, chronic sinus inflammation
b. **Laryngo-tracheo-bronchial involvement:** subglottic, tracheal or bronchial stenosis
c. **Pulmonary involvement:** nodules, cavities or pulmonary infiltrates (SOB, chronic cough, hemoptysis/alveolar hemorrhage)
3. **Renal** involvement: **_proteinuria, elevated Cr, hematuria,_** necrotizing pauci-immune glomerulonephritis
4. **ANCA** positive -(**c-ANCA** + **p-ANCA)**
Notes: Granulomatosis with Polyangiitis (GPA) (formally called Wegener’s disease), is the most common **ANCA-associated** vasculitis. Affects **small and medium arteries**, particularly in the skin, kidney, and airways.
**•Investigations:**
1. typically **c-ANCA** (PR3) positive;
2. may have **p-ANCA** (MPO) too.
3. Will have elevated inflammatory markers and CRP & CBC.
**Diagnosis:** clinical + histologic + serological
**Management**: **steroids**+ **cyclophosphamide/Rituximab/PLEX**
72
What is the treatment for Wegener granulomatosis? (2)
**Initial therapy:** Corticosteroids and cyclophosphamide **Maintenance therapy:** methotrexate, azathioprin, mycophenolate and low dose corticosteroids
73
What 3 autoimmune conditions can be complicated by MAS?
1. Systemic JIA
2. SLE
3. Kawasaki disease
74
What antibody tests should be performed to diagnose recent GAS infection in a patient with suspected ARF?
1. ASOT
2. A**nti-DNase B**
3. A**ntihyaluronidase**
\*\*\*Only 80-85% of patients with ARF will have elevated ASOT at presentation whereas 9**5-100% will have elevation of one of the antibodies** if all 3 are tested
75
What are 3 ways that maternal SLE can affect neonate?
1. Rash
2. Congenital heart block
3. Thrombocytopenia
76
What are clinical features of osteogenesis imperfecta? (5) -what is the diagnostic test for OI?
1. **Face**: Eyes: Blue sclera ; Triangular facies ; Late onset hearing loss in 50% of children
2. **Bones**:
a. **Wormian** sutures: extra bones in between sutures
b. **Cortical** bone thinning
c. **Dentinogenesis** imperfecta
**-diagnostic test:**
fibroblast testing or DNA testing
77
What are clinical features of **Classic serum sickness**? -tx?
Immune complex mediated hypersensitivity reaction, **type 3** , occurs 1-2 wks after exposure to agent (usually an antibiotic)
**-clinical features:**
**1. General: F**ever, malaise, h/ache, abdo pain, LN+
2. **Joints**: Arthralgia, polyarthritis, myalgias
3. **Skin**: urticaria,echymosis, trunk then extremties, **NO** mucosal ulceration
4. **Renal**: proteinua, hematruria, raised creatinine, edema
**-dx:**
**1. CBC: Raised (**WCC & plat), +/- raised eosinophils
**2. Inflamatory:** Low C3 4; Mildly elevated ESR + CRP
**3. Renal:** Creatinine raised, proteinuria, hematuria, casts
**-tx:**
**1. Stop offending drug**
2. supportive care, especially with **benadryl** and **NSAIDs**.
3. **Steroids** if severe symptoms
NPBR pg 631
78
What are complications of uncontrolled uveitis? (3)
1. Cataracts 2. Glaucoma 3. Vision loss
79
What are examples of DMARDs? (7)
DMARDs = disease-modifying anti-rheumatic drug
1. Azathioprine 2. Cyclophosphamide 3. Cyclosporine 4. Hydroxychloroquine 5. Methotrexate 6. Mycophenolate mofetil 7. Sulfasalazine
80
What are important questions to ask on clinical history to elicit proximal muscle weakness?
- Difficulty getting up from sitting or lying, difficulty climbing stairs, frequent falls
- difficulty swallowing, choking on foods = **weakness of palate and cricopharyngeal muscles**
81
What are physical exam findings of ankylosing spondylitis? (5)
1. Loss of normal lumbar lordosis
2. Positive Schober test (decreased forward flexion)
3. Tenderness over the sacroiliac joints
4. Anterior uveitis
5. Enthesitis (ie. patellar tendon and achilles tendon)
82
What are predisposing factors to osteosarcoma? (4)
-where is the most common site of metastasis?
redisposing factors to osteosarcoma
1. Radiation exposure
2. Hx of retinoblastoma
3. Benign bone conditions can morph into osteosarcmoa
4. Fibrous dysplasia
most common site of metastasis: **lungs**!
83
What are the cardiac consequences of having acute rheumatic fever?
Occurs in 50-60% of cases. 70% of patients recover with no residual heart disease.
- if a patient presents with carditis during the initial episode, then have **risk for recurrence** with **increased risk of permanent heart damage** with **each** recurrence
- Risk of ***mitral regurgitation and aortic insufficiency*** in the **acute** phase
- Risk of valvular disease (**mitral or aortic stenosis**) in **chronic** phase
84
What are the characteristic features of Sydenham's chorea in acute rheumatic fever?
-when does it typically present in relation to GAS infection?
**1. CNS:** Emotional lability, poor school performance
2. **Neuro;** uncontrollable movements, incoordination
a. facial grimacing,
b. milkmaid's grip (irregular contractions of the muscles of the hands while squeezing the examiner's fingers like milking a teet),
c. darting movements of the tongue with protrusion
N.B. these are exacerbated by stress
**-can present 3 months after GAS infection**
-in a patient with a long latent period from GAS infection, antibody levels may have declined to normal
85
What are the clinical features of juvenile dermatomyositis? (6) -3 tests to confirm?
-initial treatment?
1. **Muscle**: Proximal muscle weakness: gradual, fatigue, decreased ADLs,
difficulty swallowing/dysphagia, neck wekness, shoulder weakness, pelvic girdle weakness +positive Gower's sign,
2. **Rash**: heliotrope rash, gottron's papules + ulcers, V shaoed shawl sign, calcinosis, raynaud's phenomenon = most commonly presents with this, periungal erythema + Nailbed changes diagnosed via capillaroscopy -tests to confirm diagnosis
3. Poly **Arthritis** with joint contractures
4. **Resp**: interstitial lung disease
5. **Cardiac**: dilated cardiomyopathy
**3 tests to confirm**
1. EMG (best test)
2. Blood: CK/LDH/Aldolase/AST,
3. muscle biopsy
**-initial treatment:**
1. steroids plus or minus methotrexate
2. IVIG for muscle and skin
3. Hydroxychroroquine for skin
4. PFTs, PT, OT
86
What are the clinical features of PAPA syndrome? (3) -mode of inheritance? -main treatment principles?
**Clinical features of PAPA syndrome**
1. **Pyogenic Arthritis** (sterile with high neutrophils)
2. **Pyoderma gangrenosum** (skin ulcerations)
3. **Acne** (severe cystic)
**Notes; -**considered a **hereditary periodic fever syndrome** but fever is not a major symptom
-can often be mistaken for **multiple episodes of septic arthritis** but cultures will be negative
**Mode of inheritance**: autosomal dominant
**Main treatment principles:**
1. **Acne** control with tetracycline or accutane
2. **Arthritis:** High dose NSAIDs: indomethacin TID
3. **Prednisone** pulse therapy for acute flare-ups
87
What are the clinical features of TRAPS? -treatment?
**TNF-receptor associated periodic syndrome**
1. **Fever**- Long duration of **fevers** lasting 3-4 weeks and occur at ***irregular*** intervals
2. **Rash**- Migrating erythematous, maculopapular **rash**
3. **Muscle**: Severe migratory **myalgias with rash, arthralgias**
4. **Eyes**: **Conjunctivitis, periorbital edema**
5. **GIT**: S**evere abdo pain**
- **treatment**: unproven therapy; steroids provide symptomatic relief but do not change frequency
88
What are the diagnostic criteria of s**ystemic JIA?**
Requires all of the following:
1. **Arthritis** affecting 1 or more joints \> 6 weeks
2. Fever of at least **2 weeks** duration that is daily
3. Accompanied with **one of more** of the following:
1. _Hepato +/- splenomegaly_
2. Generalized _lymphadenopathy_
3. _Evanescent rash_
4. _Serositis_
\*\*\*\*Arthritis may develop LATER (ie. within one year of fever) and is usually oligoarticular (knees, wrists, ankles) but can involve cervical spine and hip. N.B. (NPBR says polyarthritis and especially cervical spine and hip.)..
89
What are the features of erythema marginatum?
What condition is it seen in?
Seen in acute rheumatic fever
- erythematous, macular lesions with pale centers -non pruritic -occurs on trunk and extremities, N**OT on the face!**!!
- can be accentuated by warming the skin
90
What are the laboratory findings in Kawasaki Disease? (4)
1. CBC: Anemia + elevated platelets (2nd week), Leukocytosis with neutrophilia and left shift
2. Inflamatory: Elevated ESR & CRP
3. Liver: Elevated transaminases, GGT, hypoalbuminemia 5
4. Sterile pyuria
91
What are the precipitants of raynaud phenomenon?
- causes?
- treatment?
**Precipiants**: cold, physical or emotional stress, caffeine, smoking
**Causes**:
1. **Primary**: no underlying etiology
2. **Secondary**: due to underlying autoimmune diseases (scleroderma, mixed connective tissue disease, SLE, etc.), drugs, toxins, polycythemia
**Treatment**:
- avoid precipitants
- may need nifedipine (peripheral vasodilator)
92
What are two classic features of systemic-onset JIA?
1. High-spiking fevers once or twice daily at similar times 2. EVANESCENT (comes and goes in a few hours), salmon-colored, macular rash (usually on extremities)
93
What are two conditions to rule out in a child with fever and limb pain?
1. Osteomyelitis 2. Septic arthritis
94
What are types of: -large vessel vasculitis (2) -medium vessel vasculitis (2) -small vessel vasculitis (4)
**Large vessel vasculitis:**
1. giant cell arteritis (older adults)
2. takayasu arteritis
**Medium vessel vasculitis:**
1. Kawasaki 2. Polyarteritis nodosa
**Small vessel vasculitis:**
1. Wegener's granulomatosis 2. Churg-Strauss 3. Microscopic polyangitis 4. HSP
95
What are useful tests with high sensitivity for osteomyelitis?
Bone scan (but high exposure to radiation) MRI
96
What is CREST syndrome? -most important complication?
Aka Limited systemic sclerosis
1. Calcinosis 2. Raynaud phenomenon 3. Esophageal dysmotility 4. Sclerodactyly 5. Telangiectasias
- most important complication:
future development of **pulmonary hypertension**
97
What is Morphea?
Group of disorders with skin and subdermal connective tissue changes due to excessive accumulation of collagen
98
What is the best confirmatory test for juvenile dermatomyositis?
-other tests?
EMG = denervation & myopathy
**-other tests**:
1. Muscle biopsy = perifascicular atrophy
2. CK can be normal or high
3. ANA: 80% can be positive
99
What is the diagnostic criteria for **enthesitis-related arthritis**?
\*\*\*enthesitis: inflammation where ligament or tendon attaches to bone
- _Arthritis AND enthesitis_ OR enthesitis or arthritis with **_at least 2_** of the following:
1. Presence of S**I joint tenderness and/or back pain**
2. Onset of **arthritis in a male \> 6 yo**
3. A**cute uveitis**
4. **Family history** of ankylosing spondylitis, uveitis, sacroileitis with IBD
5. **HLA-B27 positive**
100
What is the diagnostic criteria for fibromyalgia? -treatment?
1. Generalized MSK aches at **3 or more sites** for **3 or more months**
2. Absence of **underlying condition** or **cause** and **normal lab tests**
3. **5 or more tender points**
- **treatment**:
education about avoiding triggers (change in physical activity due to injury or chronic illness), sleep hygiene, exercise therapy to reverse immobility and increase function, cognitive behavioural therapy
101
What is the diagnostic test of joint to diagnose septic arthritis? What percentage of children with septic arthritis will have a positive blood culture?
-Arthrocentesis (aspiration of joint) is diagnostic intervention of choice -WBC \> 50 x 10^9 -Gram stain positive 50% of time -Culture positive 70% of time Percentage of children with culture positive septic arthritis who will have positive blood culture: 50%
102
What is the Jones Criteria?
-what are **3 circumstances** where diagnosis can be made without strict adherence to Jones criteria?
Diagnostic criteria for acute rheumatic fever -need 2/5 major criteria OR 1/5 major and 2/4 minor criteria PLUS evidence of recent GAS infection (positive throat culture or rapid strep antigen test or elevated or increasing ASOT)
**_-Major criteria_**: PECCS
P- Poly Arthritis (poly, migrating, large joints = 75% of cases, most common feature)
E- Erythema marginatum
C- Carditis Pancarditis (endocarditis universal finding, sometimes has myocarditis or pericarditis)
C- Chorea
S- Subcutaneous nodules on extensor surfaces of tendons near bony prominences
**_Minor criteria_**: FLAP
1. **Clinical**: Fever + Arthralgia
2. **Lab**: elevated ESR, CRP
3. **ECG**: Prolonged PR interval
**3 circumstances where diagnosis of ARF can be made without Jones criteria:**
1. Chorea (may occur as the only manifestation)
2. Indolent carditis
3. Recurrent episodes
103
What is the minimum duration of fever to be called fever of unknown origin?
Fever needs t**o last \> 14 days** with standard investigations not resulting in a clear diagnosis
104
What is the most common cause of hip pain in a child?
Acute transient synovitis = diagnosis of exclusion!
105
What is the most common form of localized scleroderma inpediatric population?
**Linear scleroderma:** characterized by one or more linear streaks (following dermatomal distribution) extending over upper or lower extremity
-can involve face or scalp (**en coup de sabre**), usually forehead -**Parry-Romberg syndrome:** progressive hemi-facial atrophy, disfiguring
106
What is the most sensitive imaging test to rule out septic arthritis of the hip?
**Ultrasound** = can detect joint effusion and fluid collection, especially in the hip joint whereas plain xrays are normal in \> 50% of cases of hip septic arthritis
Bone scan is very sensitive as well
107
What is the pathophysiology behind acute phase reactants? -which lab markers increase in acute phase response? (11) -which lab markers decrease in acute phase response? (3)
Acute phase reactants: plasma proteins produced by the liver that change production during acute phase of inflammation (mediated by cytokines)
**-increased lab markers:**
1. CRP 2. ESR 3. Complement proteins 4. Fibrinogen 5. Ferritin 6. Ceruloplasmin 7. Haptoglobin 8. G-CSF 9. IL-1 receptor antagonist 10. Serum amyloid 11. Coag factors
**-decreased lab markers:**
1. Albumin 2. Transferrin 3. IGF-1
108
What is the prognosis of transient synovitis? More common in boys or girls?
Resolution within 7-10 days More common in boys
109
What is the timing of development of acute rheumatic fever in relation to when the GAS infection occurred?
2-4 wks after GAS pharyngitis
- 1/3 of patients with ARF have no history of antecedent pharyngitis
- only 10-20% of throat culture or rapid strep antigen tests will be positive at the time of presentation of ARF;
this is why ASOT is needed!
110
What is the treatment for acute rheumatic fever? (5)
1. Penicillin V PO x 10 days to eradicate GAS from upper respiratory tract
2. Long-term a**ntibiotic prophylaxis** to prevent recurrence (and thus cardiac complications): Pen V BID OR Pen G IM monthly until 21 yo or for life if they had carditis
3. **Salicylates**: only once diagnosis has been confirmed! ASA = 1*00 mg/kg/day* div q6h x 5 days, then 75 mg/kg/day div q6h x 4 wks
4. **Steroids** IF there is carditis or congestive heart failure
5. Phenobarb PO for chorea
111
What is the treatment for kawasaki disease?
-what about for patients who remain febrile after 1st course of treatment?
1. **IVIG 2 g/kg x 1 dose** and **high dose aspirin** (80-100 mg/kg/day div q6h) as soon as possible after diagnosis (ideally within 10 days of fever onset)
- IVIG and ASA together decrease risk of coronary artery disease
2. When child has been a**febrile \> 48 hrs,** then decrease dose of aspirin to 3-5 mg/kg/day single dose) x 6-8 wks until repeat ECHO and can be discontinued if ECHO is normal
\*\*\*\*If patient is still febrile despite 1 dose of IVIG and starting ASA, this is defined as **IVIG-resistant KD** (15% of patients) -increased risk for coronary artery abnormalities
1. Give 2nd dose of IVIG at 2 g/kg
2. Consider IV methylprednisolone pulse therapy or cyclophosphamide or plasmapheresis
112
What is the treatment of KD with myocarditis?
-what about KD with large coronary aneurysm?
**KD with myocarditis:** add **corticosteroids** right away to first dose IVIG
-KD with **large coronary aneurysm**: ***abciximab*** (glycoprotein IIb/IIIa receptor inhibitor) in acute phase and long term ***antiplatelet therapy***
113
What is the triad of **Felty's syndrome?**
1. Rheumatoid arthritis 2. Neutropenia 3. Splenomegaly
114
What is the usual age of onset of growing pains in children? -clinical features? -treatment?
**4-10 yo**
**Clinical features:**
deep aching cramping pain in bilateral thighs or calves, usually at night, often awaken from sleep
- treatment: improves with gentle massage, heat and/or analgesia with acetaminophen
- symptoms disappear by morning
- normal physical exam
115
What monitoring needs to occur when patients are on hydroxychloroquine?
Eye exams q6mo to assess for **retinal deposits** (retinal toxicity)
116
What percentage of children with septic arthritis have fever? Clinical features of septic arthritis?
-70% Acute, monoarthritis, erythema, warmth swelling, intense pain with passive movement
117
What specific group of auto-antibodies are associated with increased risk of thrombosis?
-two examples of these?
A**ntiphospholipid antibodies**
- this is why people with **antiphospholipid antibody syndrome** are at increased risk of clots and pregnancy loss secondary to clots
- eg.
1. lupus anticoagulant,
2. anticardiolipin
118
Where does bacteria come from in septic arthritis?
Penetrating Contiguous: from adjacent infection Hematogenous: more blood vessels in growth plates and thus bacteria can enter there 50% of neonates with osteomyelitis will also have a septic joint because of spread through blood vessels
119
Which conditions is associated with positive HLA-B27? (5)
1. JIA: enthesitis related and psoriatic arthritis
2. IBD
3. Isolated acute anterior uveitis
4. Reactive arthritis
5. Ankylosing spondylitis
120
Which disease is associated with a positive c-ANCA?
Wegener granulomatosis **"C Wegener!"**
121
Which diseases are associated with positive p-ANCA? (5)
**Think..p PSC!!**
1. Primary sclerosing cholangitis
2. Microscopic polyangitis
2. Churg-Strauss syndrome
3. Ulcerative colitis
4. SLE
