Rheumatology Flashcards
What are the labs like on scleroderma/systemic sclerosis?
Anti-centromere antibodes
Anti-SCL-70 (scleroderma antibody aka anti-topoisomerase)
CBC: Mild anemia
UA: proteinurua if renal involvement
ANA+ in 95%
Who is most likely to get PAN?
50yo men
What are the main features of Sjogrens?
Keratoconjunctivitis sicca (dry eyes)
Xerostomia
Arthralgia/arthritis*****
Fatigue
Parotid gland enlargement
Dryness of other surfaces
Tons of other extraglandular shit - raynaud, lymphadenopathy, pulmonary disease, vasculitis, nephritis, lymphoma
Do you have to order tons of different labs for SLE/
Yes because it can affect ANY tissue/organ
What is dermatomyositis?
It is polymyositis with cutaneous eruptions.
Frequently associated with occult malignancy
7-10% of patients with dermatomyositis also have what?
Occult malignancy!!
cervix, lung, ovary, stomach.
Ask them when their last PAP was
What other conditions are associated with polymyositis?
Interstitial Lung Disease***
Raynaud phenomenon
Are antinuclear antibodies associated with lupus?
Yes
How does lupus affect the kidney?
Nephritis with proteinuria (one of the things that kills SLE pts)
What are the specific ANA antibodies that will be present in primary Sjögren’s syndrome?**
Anti-sjogren’s syndrome:
Anti-Ro
Anti-La
Name it:
May involve interstitial lung disease
Polymyositis/dermatomyositis
Which antibody do you see with drug=induced SLE? Which ones don’t you see?
Positive Antihistone antibody*****
Negative anti-dsDNA, anti-Sm ab
What is the first line treatment for SLE?
daily Hydroxychloroquine (Plaquenil)
What is the test for tear production in Sjögren’s syndrome?
Schirmer test
What is the treatment for raynaud phenomenon
Calcium channel blocker
What will the labs look like for PAN?
Antinetirophil cytoplasmic antibodies (ANCA) will be NEGATIVE!!!!
Negative ANCA
If it’s positive, you don’t have PAN. You have another type of vasculitis
How will a pt with PAN present?
Cutaneous nodules, ulcers, and purpura
Renal manifestations
Malaise, neuropathy, arthralgia
Name it:
Autoimmune disorder that causes diffuse fibrosis (tightening and thickening) of the skin and internal organs
Systemic sclerosis (scleroderma)
What is the treatment for PAN
Steroids
Hepatitis treatment if they also have that
“I have difficulty arising from a chair” or “I have trouble reaching a high shelf”
Polymyositis
What is the universal symptom of lupus?
Fatigue
Name it:
Chronic dysfunction of exocrine glands, affecting the lacrimal and salivary glands.
Extra-glandular sx: a ton of other vague shit
Sjogrens
What are the cutaneous eruptions seen with dermatomyositis?
Heliotrope rash
Gottron’s papules
Shawl sign
What is the discoid rash?
Erythematous patches w/ keratotic scaling in sun exposed areas
If you get a positive ANCA, does that mean you have PAN?
No. Never. ANCA is always negative in PAN.
Name it:
Idiopathic, progressive, inflammatory condition causing symmetric proximal muscle weakness
(Weakness not pain)
Polymyositis
Does an ANA result over 40 mean you have lupus?
No, lots of healthy people have this, but 95% of SLE patients have an ANA over 40.
Can you see the calcifications of the skin on an x ray in a scleroderma patient?
Yes
Who usually gets systemic sclerosis (scleroderma)
20-50yo females
Name it: Chronic multi-organ autoimmune disorder Autoantibodies to nuclear antigens (ANA) Anti-dsDNA, anti-Sm ab Malar butterfly rash Discoid rash Can affect any organ Hydroxychloroquine 1st line tx
SLE
Name it:
Transmural inflammation of muscular arteries, causing a narrowing of the lumen.
Veins unaffected.
Results in thrombosis, ischemia, or infarct*
Polyarteritis Nodosa (PAN)
Name it:
Anti-Ro/SSA, Anti-La/SSB
Schirmer’s test
Sicca complex
Sjögren’s syndrome
How will lupus present?
Fatigue
Butterfly rash 50%
Discoid lupus in sun exposed areas
Painless** ulcers in mouth or nose
Alopecia
Raynaud phenomenon 30%
How is SLE diagnosed?
Clinical judgement
The American College of Rheum. has identified 11 criteria, and most pts will have 4 or more
What is the progression of the sclerodactyly that comes along with scleroderma
Hands and fingers start puffy
Then get shiny as skin thickens
What labs will be elevated with polymyositis and dermatomyositis?
Muscle enzymes: CK and aldolase***
ANA will be positive in 80%
What are the 11 criteria of SLE that the typical patient has 4 or more of?
(Just look at it)
Photosensitivity
Malar rash
Discoid rash
Mucosal ulcers
+ANA (anti nuclear antibody)
+Anti ds-DNA, +anti-sm +anti phospholipid abs
Polyarthritis
Serositis
Renal disorders
Neuro disorders
Hematologic disorders
What is the difference between limited and diffuse scleroderma?
Diffuse has all the same stuff as limited, but the sclerotic skin is also on chest, trunk, arms, and shoulders. They also have an increased risk of internal organ involvement.
What will you find if you biopsy skin lesions of a PAN patient?
Leukocytoclastic vasculitis*****
“WBC debris”
What are the 3 ANA subtypes that are associated with lupus?
Anti-dsDNA
Anti-Sm
Antiphospholipid
Which muscles are commonly affected by polymyositis?
Deltoids
Hip flexors
What is sicca complex?
Combo of xeropthalmia and xerostomia
(Dry eyes and mouth)
What drugs commonly cause drug-induced SLE?
Procainamide
Isoniazid
Hydralazine
***
Is polymyositis painful?
No its usually just weakness
What illness can sometimes trigger PAN?
Hepatitis B and C
Test them for hepatitis
Name it:
ACA (anticerntromere Ab)
Anti-SCL-70 (anti-topoisomerase Ab)
Systemic sclerosis (scleroderma)
What is an autoimmune disorder causing diffuse fibrosis?
Systemic sclerosis
Does PAN represent a spectrum of disease rather than a single entity?
Yes
Name it:
ANCA negative
Polyarteritis Nodosa (PAN)
What is CREST syndrome?
Calcinosis- Ca+ deposits in skin
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly- tightening of fingers
Telangiectasias
Do low complement levels indicate active or latent lupus?
Active
What is really important for your SLE patient to do if you give them hydroxychloroquine?
Regular ophthalmology follow-ups!!****
Retinal toxicity possible
What is it:
Fixed erythema over nasal bridge that spares nasolabial folds
Malar rash
What are the treatments for Sjogren’s syndrome?
See the dentist and eye doctor
Eye drops
Saliva substitute
Drink water
Sugarless candy
NSAIDs
Steroids for severe
Refer to rheumatology
What are some of the main treatments (non pharm) for SLE
Sun protection
Stop smoking**
Diet/exercise
Immunizations
Contraception- anti-phospholipid ab associated with fetal loss
What happens to the lumen of the arteries affected by PAN?
Narrowed due to inflammation
What is the first line treatment for polymyositis and dermatomyositis?
Steroids
What is so important to do if your pt has dermatomyositis?
Rule out occult malignancy
What is the treatment for systemic sclerosis?
Symptomatic care, no effective therapy for underlying process:
Warm clothing, stop smoking, Ca+ channel blockers for raynauds GERD drugs for esophagus ACE-inhibitors for renal involvement Immunosuppressive drugs for severe cases
Name it:
Heliotrope rash, shawl sign, gottrons papules
Dermatomyositis
What will the initial ANA report tell you?
- Titer dilution
- Staining pattern
(Not specific. You need to order the 3 subtypes separately)
What is the risk of plaquenil/hydroxychloroquine?
Retinal toxicity
What is raynaud phenomenon?
Episodic vasospastic** disease of the extremities
Fingers turn red white and blue in cold weather
Who is most likely to get lupus?
Black females in childbearing years
What are the two types of systemic sclerosis?
- Limited cutaneous-80%
2. Diffuse cutaneous- 20%
What mediates clinical manifestations of lupus?
Antibody formation and creation of immune complexes
**
What is the treatment for drug-induced SLE?
Stop the offending drug
Procainamide, isoniazid, hydralazine
Name it:
Anti-histone antibody
Drug induced SLE
What is a cause of chronic cough to consider in a pt with polymyositis?
Interstitial lung disease
Who is most likely to get Sjogrens Syndrome?
40-60yo women
Where are Gottron’s papules seen?
On the dorsal aspect of the hand joints
