Lecture 13: Rheumatologic Disorders Flashcards
SNOUT and SPPIN refer to testing sens and spec.
What do they stand for?
- SNOUT = a highly sensitive test that comes back negative means you can confidently rule OUT the disease.
- SPPIN = a highly specific test that comes back positive means you can confidently rule IN the disease.
The starting test for autoimmune disorders is a () test, which has high sens but low spec. It is positive if the titer is >= 1:160.
ANA: antinuclear antibody
You just need a positive and then you move onto SPECIFIC testing.
The only autoimmune disease that ALWAYS has ANA present is…
Drug induced lupus
SLE is 95%
Anti-Mi-2, Anti-Jo-1, and Anti-SRP correspond to what autoimmune diseases?
Dermato and polymyositis.
Mi and Joe were married (derm), but Jo cheated with Sarah, which is poly
Anti-histone corresponds to what autoimmune disease?
Drug induced Lupus
HIStory of meds
Anti-centromere corresponds to what autoimmune disease?
CREST syndrome
Its like the only one that starts with a C
Anti-SSA (Ro) and Anti-SSB (La) correspond to what autoimmune disease?
SLE/Sjogren’s
The two S diseases = SS
Also L for lupus and Ro for Sjogren’s (pronounciation)
Anti-Scl-70 corresponds to what autoimmune disease?
Progressive systemic sclerosis
Scl = sclerosis
ENA labs are a () lab with a (+) ANA
Reflex lab
Usually if you suspect a CT disorder
MCTD, mixed connective tissue disorder, encompasses 3 overlapping diseases:
- SLE
- Systemic scleroderma
- Polymyositis
T/F: Besides rheumatoid arthritis, RF is useful.
False.
Found in cancers and autoimmune and HEALTHY
Besides RA, RF is found in what other disease with a similar sensitivity?
Sjogren’s
75-95%, technically a little higher than RA
Also increases with age
The most specific test for RA is…
Anti-CCP
Very specific for RA
CRP vs ESR. Which is better for acute inflammation vs chronic?
- CRP = acute
- ESR = Chronic (Extended)
A normal ESR can rule out what disease?
Temporal arteritis + polymyalgia rheumatica
Since they are a spectrum of the same dz
ANCA comes in two types: p and c.
Since c-ANCA is for less diseases, its easier to remember, bc it is only positive in two diseases: () and ()
- GPA/Wegener’s
- Churg-Strauss syndrome/EGPA
Eosinophilic GPA
Classic SLE pt is (gender) and (ethnicity)
Native American Female
SLE is a (flaring/consistent) disease precipitated primarily by ()
Flaring disease precipitated by stress
SLE has a classic triad:
- (systemic sign)
- () pain
- (be specific) rash
- Low grade fever
- Joint pain
- Malar/butterfly rash on the cheeks and nose.
SLE requires 4 out of 11 S/S to be diagnosed.
- Constitutional (sign)
- Cutaneous (name a few)
- Arthritis (how many joints minimum)
- Neurologic
- Serositis (effusion of…)
- Hematologic (2 penias)
- Renal (syndrome)
- Antiphospholipid
- Complement (low or high)
- Specific antibodies (2)
- Fever
- Alopecia/oral ulcers/discoid/acute malar rash
- 2+ joints for 30+ mins in the AM
- Neuro psychosis/delirium/seizure
- Either pleural or pericardial effusion or even pericarditis
- Leukopenia or thrombocytopenia
- Proteinuria
- APL
- Low complement C3/C4
- Anti-dsDNA or Anti-Smith
Highlighting what ive seen on anki cards pointed out
The two most specific antibodies for SLE are…
- Anti-dsDNA
- Anti-Smith
SSA and SSB overlap with Sjogren’s
A false positive syphilis test is associated with what antibody?
Antiphospholipid antibody
Overall, the system that shows the highest rate of manifestations in SLE is..
MSK
SLE is often associated with what cardiac condition and what pulmonary conditions? (2 and 2)
- Pericarditis
- Myocarditis
- ILD
- Pulmonary HTN
Lupus nephritis is proven/diagnosed via…
Biopsy
Tim’s way of remembering Lupus demographics
The 3 highest risk medications that can induce lupus are… and you should check Drug-induced lupus with what antibody???
- Procainamide
- Hydralazine
- Penicillamine
- Check with an anti-HIStone antibody (HIStory of med use)
Generally, a completely stable SLE pt requires follow up every () months, and () on a CBC is usually indicative of acute flare.
- Q6months
- Thrombocytopenia suggests acute flare
3-4 months or less for less stable.
A SLE patient should use sunscreen with () SPF and probably supplement with vitamin ()
- > = 55 SPF
- Vit D supplementation
Since UV exposure can trigger flares.
In a stable SLE patient, you can vaccinate safely with 4 vaccines:
- (annual)
- (older)
- (sex)
- (liver)
- Influenza
- Pneumococcal
- HPV
- HBV
Smoking () the efficacy of hydroxychloroquine for SLE tx.
Lowers
So tell em to quit smoking
For someone who already has SLE, the two meds to avoid are…
- Sulfa ABX (Bactrim)
- Minocycline
Your SLE patient wants to have a baby. You advise her to wait at least () months after a flare before attempting.
6 months
Mild SLE with just skin, joint, and mucosal involvement is treated first-line with…
Hydroxychloroquine.
Moderate SLE, with signficant manifestations but NO ORGAN threatening manifestations, is treated with…
Hydroxychloroquine + NSAID + daily prednisone + MTX or azathioprine
Severe SLE, with renal/CNS involvement, is treated with…
Hydroxychloroquine + NSAIDs + High dose IV prednisone + Belimumab/Rituximab/Cyclosporine
Mainly switching to IV prednisone + new immunosuppressants
The mainstay of treatment for the joint symptoms of SLE is what drug?
Hydroxychloroquine
Chloroquine toxicity appears as what sign?
Bull’s eye retinopathy
Do annual ophtho exams after 5 years!
On top of hydroxychloroquine for joint symptoms in SLE, you can add on (specific NSAID) next, and then ().
- Naproxen (might need PPI if long-term)
- Can use Celebrex to lower GI ulcer/CV risk.
- Prednisone if hydroxychloroquine and NSAIDs are insufficient.
Scleroderma is characterized by () of the skin, internal connective tissue, and organs
Hardening
know your greek!
Scleroderma is MC in (males/females)
Females
Scleroderma has 5 million manifestations of every organ system, but it has 4 main subtypes:
- Limited cutaneous systemic sclerosis: Characterized by manifestations distal to () and on the face/neck. () syndrome.
- Diffuse cutaneous systemic sclerosis: Disease progresses to include () and () manifestations.
- Systemic sclerosis sine scleroderma: Rarest kind. Specifically does NOT have () manifestations.
- Systemic sclerosis with overlap syndrome: overlaps with a diff rheumatologic disorder
- Distal to elbows
- CREST syndrome describes limited cutaneous systemic sclerosis
- Diffuse includes proximal and truncal manifestations.
- Ssstemic sclerosis SINE has no SKIN manifestations.
The MC subtype of scleroderma is…
Limited cutaneous systemic sclerosis
The two areas of the body CREST/limited scleroderma affects are…
Head and Hands
Up to 90% of scleroderma patients have (organ) symptoms
GI
The antibody most associated with diffuse cutaneous SSc and lung disease is…
Anti-DNA topoisomerase I, aka Anti-Scl-70
The antibody most associated with CREST is…
Anti-centromere
C for CREST
Anti-RNA polymerase III antibody is associated a great risk of () failure and cancer in scleroderma.
Diffuse disease + renal failure
You should have baseline testing of the lungs to check for () in scleroderma. What 3 tests?
Checking for ILD via PFTs, Echo, and CT Chest
The treatment for SSc is organ system based.
Raynaud’s = ()
GERD = ()
Arthritis = ()
RA = ()
Diffuse skin sclerosis/organ involvement = ()
- Raynaud’s = CCB
- GERD = PPI
- Arthritis = NSAIDs
- RA = see slide for stepwise
- Diffse skin = MTX
The top two characteristics for Sjogren’s is () of the eyes and decreased () in the mouth
- Dryness of the eyes/no lacrimation
- Decreased salivation in the mouth
T/F: Drinking more water will help Sjogren’s
Eh, no
The two antibodies for Sjogren’s are () and ().
- Anti-SSA (Anti-Ro)
- Anti-SSB (Anti-La)
Anyone with eye symptoms in suspected Sjogren’s should get a () test, and a negative test is when greater than () mm is seen.
Schirmer test, which is negative if more than 10mm of the strip is wet.
Also get a surface stain test
HLA-() is seen in 85% of Sjogren’s pts
HLA-DR52
Dry 52, MC age range is 50-60 and its a dry condition.
You should be sure to rule out (autoimmune condition), (Hep ?), and Vitamin () Deficiency, which can mimic Sjogren’s.
- HIV
- HCV
- Vitamin A deficiency
ESR and CRP values in Sjogren’s are often…
- ESR often elevated.
- CRP is NORMAL.
Specifically, women with Sjogrens and high titers of SSA/SSB are at high risk for associated congenital ()
Congenital Heart Block
What diet/supplement is good for eye health in Sjogren’s? (since its always dry)
Omega-3-fatty acids
Eat ur salmon
The pharm tx for Sjogren’s is…
Pilocarpine, a cholinergic
The shared features of polymyositis and dermatomyositis are () skeletal muscle weakness and () inflammation. (PM and DM)
- Proximal muscle weakness
- Muscle inflammation
PM usually peaks during ().
DM usually peaks during () or ()
- PM = adulthood
- DM = childhood or adulthood
PM is rarer
PM and DM have a () onset of symmetric, progressive, (pain/painless) proximal weakness that SPARES () and () muscles.
- Insidious onset
- Painless weakness
- Spares eye and facial muscles
Increased risk of cancer is primarily with PM vs DM?
DM
DM has skin manifestations on top of PM.
Raised, violaceous scaly eruptions on the knuckles are known as ()
A blue-purple rash over the face is known as ()
- Gottron’s Papules
- Heliotrope Eruption
A hyperkeratotic, dirty appearing hand is seen in () and is known as () hands
DM, mechanic hands
A hyperkeratotic macular rash appears on your patients upper back looking like this. You know this is a (), indicative of DM.
Shawl sign
There are 3 antibodies associated with DM/PM.
1 is only DM.
1 is associated with ILF and mechanic hands
1 is associated with mainly PM, muscle and cardiac manifestations.
- Anti-Mi-2: Just DM.
- Anti-Jo-1: ILF + Mechanic hands (both PM/DM)
- Anti-SRP: Mainly PM, muscle weakness and cardiac manifestations.
T/F: muscle biopsy is required to diagnose the myositis conditions.
False.
The two muscle enzymes you want to order in DM/PM are: () and (), which should show greater than () times the upper limit in active disease.
- Aldolase and CK.
- CK should be 10x ULN
The mainstay TOC for PM and DM is…
1mg/kg of Prednisone.
Cancer screenings, CT chest, abd, pelvis, TVUS, and mammogram should be done every () years for people with PM/DM.
Every 2 years.
