RA/JIA/SpA
Which arthritis is the only one that improves with rest?
Which arthritis is the only one that has symmetrical joint involvement?
OA- improves w/ rest (RA/JIA improve w exercise)
RA is symmetrical (OA and SpA are asymmetrical)
Give me two large vessel, two medium vessel, and two small vessel vasculitides?
General tx for vasculitides?
LARGE - Takayasu, GCA
Medium - Polyarteritis Nodosa, Kawasaki
Small - IgA Vasculitis, ANCA-associated vasculitides
When in doubt- usually tx with steroids! high dose prednisone to be specific
What type of scleroderma is more common in adults vs kids? What does the prognosis of each look like?
ADULTS- Circumscribed Morphea (under localized scleroderma, more likely to resolve on its own)
KIDS - linear SLE (under localized scleroderma, more likely to develop into SLE) :(
MCTD is a combination of what other conditions?
What antibody is associated with MCTD?
MCTD = Scleroderma (systemic sclerosis), SLE, and polymyositis
MCTD = Anti- U1 RNP Abs
Name the most sensitive Ab?
The most specific Ab?
The Ab that's both specific and sensitive?
The 3 Anti-Phospholipid Ab's (seen 50% of cases)??
Sensitive - ANA+
Sensitive and Specific - anti-dsDNA (correlates well w/ nephritis!)
Specific - Anti-Smith
AP Abs: Anti Cardiolipin, Anti Beta 2, Lupus Anticoagulant (gives false elevated PTT)
What are 4 general treatment medications for rheumatic diseases?
Which medication is most recommended for initial vs severe presentations?
Glucocorticoids, NSAIDS, DMARDs (methotrexate, sulfasalazine, hydroxychloroquine), Anti-TNF agents (ex. Inflixumab and Adalimumab)
Initial - NSAIDS
Severe - Anti-TNF agents
How does the most common childhood vasculitis present as? How do you treat it?
IgA vasculitis presents with palpable purpura on lower limbs with at least one of the following: abdominal pain, joint pain, or renal disease.
Most commonly in White boys in the winter and spring post-strep infection
TX - resolves spontaneously, use steroids if severe
Most important clinical predictor for scleroderma?
Look at their nails!
Do a nail fold capillaroscopy
(its not Antibodies, I know)
What is Sjogren's Syndrome?
Most common underlying disorder in Sjogren's Syndrome?
Sjogren's Syndrome = autoimmune destruction of exocrine glands bc of T and B cell infiltration
Rheumatoid arthritis
What is the recommended treatment for all stages of SLE? What are the recommended treatments for severe SLE?
ALL severities- hydrochroloquine
Severe SLE - cyclosphosphamide and mycophenolate mofeti
What 3 antibodies are associated with RA?
Which antibody has antibodies against IgG?
1. RF (a type of IgM antibody against IgG)
2. Anti-CCP (most SPECIFIC Ab!, CCP and RF have the same sensitivity of 80%)
3. ANA (increases uveitis risk in JIA)
What test should patients with Kawasaki disease have and when should it be done?
DO AN ECG bc of the risk for coronary aneurysms and pancarditis!
Do an ECG at diagnosis, 2 weeks in, and at 6-8 weeks
3 main pathologies in scleroderma?
Buzzword for scleroderma?
C.A.B. = Collagen overproduction, Autoimmune process, Blood vessel damage
"Salt n pepper vitiligo"
Sjogren's has Anti-SSA/SSB antibodies, what two other conditions also present with these antibodies?
What are the other names for SSA and SSB?
Sjogren's, Neonatal Lupus, SLE
Anti SSA is also Anti-Ro
Anti-SSB is also Anti-La
What is the diagnostic criteria for SLE?
4 criteria total with at least 1 Clinical AND 1 Serology confirmation
ex. 3 clinical and 1 serology, 2 clinical and 2 serology, 4 clinical and 1 serology etc.
What are 3 main characteristics that individuals with JIA present with that differs from RA?
1. Uveitis
2. Micrognathia (underdeveloped jaw)
3. Limb Length Discrepancy
RAPID FIRE
1. Which vasculitis presents with a possible Hep B infection, foot or wrist drop, and a string of beads sign?
2. Which vasculitis presents with a unilateral headache, visual changes, and jaw discomfort?
3. Which vasculitis presents with bruits, a fever, and different blood pressures in the upper and lower halves of your body?
4. Which vasculitis presents with glomerulonephritis and has cANCA?
1. Polyarteritis Nodosa
2. GCA (Giant Cell or Temporal Arteritis)
3. Takayasu Arteritis
4. GPA/Wegener's
Name some mimic conditions for scleroderma!
CREST is associated with what SLE subtype?
CREST is limited, systemic scleroderma!
Mimics: BENDS (2 E's and 2 S's):
Bleomycin reaction, Eosinophilic Myalgia, Eosinophilic fasciitis, Nephrogenic Systemic Fibrosis, Diabetic Cheiroarthropathy, SclerEDEMA, Scleromyxedema
Pts with MCTD are mostly at risk of developing...?
Pts with Sjogren's are mostly at risk for developing what 4 conditions?
MCTD - PAH
Sjogren's - Autoimmune hepatitis, autoimmune thyroiditis, PBC, and celiac disease!
What are some lupus-specific complications?
Shrinking Lung
Lupus Nephritis (renal disease #1 cause of death)
Libman-Sacks Endocarditis
Malignancies: Hodgkin's Lymphoma, Lung Cancer, Breast Cancer
SpA RAPID FIRE
1. SpA that has highest chance of uveitis?
2. SpA that has sausage fingers?
3. SpA associated with STDs?
4. SpA associated with IBD and gangrenosum?
1. SpA that has the highest chance of uveitis? Ankylosing Spondylitis
2. SpA that has sausage fingers? Psoriatic Arthritis
3. SpA associated with STDs? Reactive Arthritis
4. SpA associated with IBD and gangrenosum? Enteropathic Arthritis
A 5 year old boy comes in with a fever for 3 days now, a rash on his hands and feet and enlarged cervical lymph nodes. What could be a diagnosis for this patient?
If it's what you think it is, how do we treat it?
You thought it's Kawasaki's right? ITS NOT!
Kawasaki to dx needs a fever for at least 5 days (this pt's fever is only 3 days) OR 4/5 clinical symptoms (this pt only has 3).
Remember: CRASH n BURN
If it WAS Kawasaki, we treat it with IVIG AND high-dose aspirin.
Leading cause of death for scleroderma? Because of this condition, what screening should you do for patients?
Leading cause of death: PAH or pulmonary arterial hypertension
Pts should have an FVG and a DLCO done to assess pulmonary function!
HLA association for Sjogren's?
Sjogren's is two main components: keratoconjunctivitis sicca and xerostomia. What two test are needed to diagnose dry eyes (keratoconjunctivitis sicca)?
HLA-DR2/3
1. NEEDED - Rose Bengal test - assess for corneal abrasion/damage
2. NEEDED - Schirmer Test - assess tear production
There are 6 classes of lupus nephritis! Which class(es) are associated with an increased risk of ESRD?
Which class has focal vs diffuse proliferative glomerulonephritis?
Class 3 (focal) and Class 4 (diffuse)