post midterm 3 rheum Flashcards
what does the ESR or sed rate measure
Distance that RBCs fall in 1 hour – cells aggregate and settle faster when inflammatory proteins are present
what does C reactive protein measure?
Acute-phase reactant, secreted by liver in response to inflammatory cytokines
High levels (>10) sensitive but nonspecific for inflammatory states
*Chronic low level elevation (1-10) associated with increased CV risk (high sensitivity or HS-CRP)
what is rheumatoid factor?
Antibodies (IgM) against Fc region of human IgG
*but can have false + or -
what does the ANA test measure?
Antibodies to nuclear antigens (DNA, RNA, histone and other proteins)
what is lupus anticog?
Antibody w/ prothrombotic effects in vivo but prolongs PTT in vitro
Observed in patients with lupus, but can occur in patients w/o lupus
what is the most common joint disease?
osteoarthritis
what is osteoarthritis?
what might you see on X ray?
*Non-inflammatory joint disease
*assoc w/ advancing age
X-ray findings:
–Loss of cartilage (joint space narrowing)
–Osteophytes (bone spurs)
Some genetics
Trauma / repetitive motion
osteoarthritis affects what aspect of body?
Limited to joint involvement:
Distal interphalangeal joints
Proximal interphalangeal joints
1st metacarpophalangeal joint (only)!!
get nodules in fingers
-also affects cervical spine, lumbar spine, hips and knees
primary tx for osteoarthritis?
tylenol (since NOT inflammatory!) and maybe physical therapy
what is Carpal Tunnel Syndrome?
what does it affect?
Pressure on median nerve in the carpal tunnel
-Digits 1, 2, 3 and ½ of 4th fingers
what can trigger carpal tunnel?
Repetitive use
Inflammation (e.g. rheumatoid arthritis)
Trauma
what is rheumatoid arthritis?
A systemic autoimmune disease of unknown etiology that includes chronic synovial inflammation, joint destruction, systemic manifestations and ocular inflammation
what kind of pop do you see RA in?
Usually presents in young women
Onset may be at any age, but most are 30-40
Some genetic predisposition
what are some clinical features of RA?
*Constitutional symptoms
(Fever, weight loss, fatigue)
- Arthritis: small joints
- Synovial thickening/pannus
- -Secondary carpal tunnel syndrome
- -Articular, periarticular destruction of joint destruction
*Rheumatoid nodules
Extensor surfaces (skin)
Heart, lungs, other organs
*osteoporosis
what are some Ocular Manifestations of Rheumatoid Arthritis?
1) Keratoconjunctivitis sicca (associated Sjogren’s syndrome)
2) Episcleritis (mild, transient)
- Pain, no visual impairment
3) Scleritis (edema, pain) – risk for systemic vascular disease*
–Nodular scleritis
–Scleromalacia perforans (choroid pigment visible)
–Brawny scleritis=
inflammation, tenderness or pain of the globe, history of collagen vascular disease
4) Ulcerative keratitis
5) Brown’s syndrome- Tendinitis of superior oblique → diplopia
what is still’s disease?
Systemic onset JRA w/
Prominent fever (daily spikes to > 103oF)
Rash, lymphadenopathy, hepatosplenomegaly
In Pauciarticular (or oligoarticular) onset JRA, where Less than 5 joints involved in first 6 months, no systemic features, what kind of ocular features can you see?
Chronic bilateral nongranulomatous iridocyclitis is common
–Appears insidiously over months, independent of arthritis, ANA usually (+)
*Significant threat to vision from complications:
posterior synechiae, band keratopathy, cataracts, secondary glaucoma
main tx forms for RA?
- *primarily just know DMARDs
- NSAID’s: for symptoms! not strong enough for anti-inflamm
- -Non-selective (aspirin, ibuprofen, naproxen, etc.)
- -COX-2-selective (celecoxib)
- Glucocorticoids
- Disease Modifying Anti-Rheumatic Drugs (DMARD’s
besides affecting GI tract, NSAIds can also cause what other kinds of problems?
- GI inflammation, erosion, ulceration
- *Decreased by concomitant use of PPI
- Renal dysfunction
- Cardiovascular deterioration (edema, HTN, CHF)
what are the side effects assoc with corticosteroids?
Cushing’s syndrome Diabetes Worsening joint instability Osteoporosis Increased cataract risk *Rarely used alone in modern therapy: Low dose can be added to DMARD Tx
what are some Disease-Modifying Anti-Rheumatic Drugs: DMARD’s? what are some side effects of each?
Methotrexate: can get Hepatotoxicity
Bone marrow suppression
Antimalarials (hydroxychloroquine, chloroquine):
Probably the least toxicity of the DMARD’s
Risk for toxic retinopathy requires monitoring of ocular exam
Asymptomatic initially; early Sx scotomata*
Ocular findings: Irregularity in macular pigmentation* progress to concentric hypopigmentation
describe the course of RA?
Course of disease is highly variable:
- Multiple exacerbations and remissions are common
- Most show progressive, destructive polyarthritis over years
what is lupus or Systemic Lupus Erythematosis?
A systemic autoimmune inflammatory disease of unknown etiology
lupus affects what type of pop? what causes it?
women
Onset usually between 15-25 years of age
cause: unknown, genetics does play some role
what kind of clinical symptoms do you see with lupus?
Constitutional symptoms *Arthritis, arthralgia (92%) Mucocutaneous lesions Serositis: affects lungs intest heart Cardiopulmonary-, nonbacterial endocarditits Renal Neurologic Hematologic Ocular
what kind of clinical symptoms do you see with lupus?
Constitutional symptoms *Arthritis, arthralgia (92%) Mucocutaneous lesions Serositis: affects lungs intest heart Cardiopulmonary Renal Neurologic Hematologic Ocular
what is the “malar rash” and what is it assoc with?
like a butterfly rash across cheeks, associatied with lupus
what kind of arthritis is associated with lupus?
Pain, stiffness, swelling of hands, wrists, elbows, knees, ankles
Polyarthritis with benign joint fluid, minimal deformity**
what are some possible ocular side effects of lupus?
-Conjunctivitis, keratitis, episcleritis, uveitis
-Retinopathy:
CWS, Retinal edema, Superficial hemes, Arteriolar narrowing
-Proptosis
-Keratoconjunctivitis sicca (20%)
-Ischemic optic neuropathy, optic atrophy (rare)
-Neuro-ophthalmologic complications of CNS involvement: cranial nerve palsies (20%) from mononeuritis,
what are some possible lab findings for lupus?
- Antinuclear antibodies (ANA) - positive in 90%, but nonspecific
- Lupus anticoagulant, thrombocytopenia, anemia
- Biopsy of skin, kidney, etc. revealing Ig/complement immune complex deposition
what are some possible tx forms for lupus?
1) Anti-inflammatory medications (NSAIDs, aspirin)
2) Glucocorticoids
Useful in treating renal failure, hemolytic anemia, severe psychosis
3) Hydroxychloroquine
4) Immunosuppressive drugs
what kind of course does lupus take in progression?
- Typically relapsing/remitting course over years
- Most do well, with skin/joint disease only
- Flares often during pregnancy, ↑ risks for miscarriage
- Can improve after menopause
- Fulminant downhill course may occur, with death from renal, neurologic, hematologic, infectious complications
- -Mortality varies but has improved over past decades
what is Antiphospholipid Antibody Syndrome?
Positive antiphospholipid antibodies PLUS:
- Arterial and/or venous thrombosis
- retinal artery occlusion
- Thrombocytopenia
- Spontaneous abortions (recurrent)
***can get secondarily w/ lupus
what is Sjogren’s?
A systemic autoimmune inflammatory disease of unknown cause
–Destruction of exocrine glands -> dry eyes, mouth, etc.
[Frequent association with other autoimmune disorders]
what pop do you see Sjogren’s in?
women
usually middle aged
what are some ocular clinical signs of sjogren’s?
Filamentary keratitis
Corneal ulcers
Lacrimal enlargement (unusual)
what are some other autoimmune disorders that can give secondary sjogrens?
Rheumatoid arthritis S.L.E (Sjogren’s occurs in 30% of patients with R.A. or S.L.E.) Scleroderma Polymyositis Autoimmune cirrhosis Hashimoto’s
how can you diagnose SJogren’s?
- Clinical features
- Exocrine gland, lip biopsy diagnostic (lymphocyte infiltration)
- ANA, rheumatoid factor often positive
what is Systemic Sclerosis - Scleroderma?
idiopathic autoimmune disorder causing fibrosis of skin, with or without internal organ involvement
-Fibrosis of dermis, with increased collagen deposition
Two clinical subsets of the disease:
Limited cutaneous scleroderma
Diffuse cutaneous scleroderma
-Often family Hx of rheumatologic disease (eg SLE, RA)
What is Limited Cutaneous Scleroderma“CREST” Syndrome?
Calcium deposits (calcinosis)
Raynaud’s phenomenon
Esophageal dysmotility (dysphagia, reflux - 50%)
Sclerodactyly: dermal scarring, thickening, tightening of skin
Telangiectasias
What is Raynaud’s DISEASE?
Raynaud’s phenomenon in young women (90%) without associated autoimmune disorder
you can see Raynaud’s with what other autoimmune disease?
Rheumatoid arthritis (< 5%)
Scleroderma (> 95%)
S.L.E., Sjogren’s syndrome (20-30%)
Polymyositis (25%)
what is Diffuse Cutaneous Scleroderma? what do you see clinically?
1) Constitutional symptoms and Raynaud’s initially
2) Rapidly progressive skin changes
- Edema and pruritis
- Telangiectasias, calcinosis, dermal fibrosis
- Destruction of skin appendages - sweat, hair, sebaceous
- Hyper-/hypo-pigmentation
3) Arthralgias, tendon involvement, bone destruction
4) Visceral involvement
- Diffuse GI involvement
- Pulmonary fibrosis
- Cardiac fibrosis
- Renal failure and hypertension
what are some ocular manifestations of scleroderma?
*Eyelids - lose elasticity Retraction Exposure keratitis *Retinopathy (cotton wool spots) *Keratitis *Cataracts *EOM palsy (nerve sheath compression
how would you diagnose scleroderma?
Characteristic clinical picture, especially skin changes and Raynaud’s
what is polymyositis?
An inflammatory myopathy causing skeletal muscle weakness
what is dermatomyositis?
Polymyositis with skin manifestations
what clinical features do you see with Polymyositis and Dermatomyositis? what is pathognomonic?
**Muscle weakness (85%) – proximal
[Difficulty: standing from chair w/o arms, getting out of car, reaching overhead, brushing hair]
**Skin lesions: Gottron’s papules -pathognomonic sign of dermatomyositis
***Association with underlying malignancy often-like cancer
--not as much of a focus-- Arthritis Raynaud’s phenomenon Associated autoimmune disorders S.L.E. Scleroderma Sjogren’s syndrome
what kind of ocular side effects do you see with Polymyositis and Dermatomyositis?
Periorbital edema Heliotropic rash on lids Retinopathy Cotton wool spots Hemorrhages EOM involvement (unusual) Episcleritis (unusual)
what kind of ocular side effects do you see with Polymyositis and Dermatomyositis?
Periorbital edema Heliotropic rash on lids Retinopathy Cotton wool spots Hemorrhages EOM involvement (unusual) Episcleritis (unusual)
what pop do you see GCA in?
Affects elderly population exclusively:
Peak incidence at 60-75 y.o., all are > 50 y.o.
Women : men = 3 : 1
more caucasians, genetics
what pop do you see GCA in?
Affects elderly population exclusively:
Peak incidence at 60-75 y.o., all are > 50 y.o.
Women : men = 3 : 1
more caucasians, genetics
what will GCA cause in eye?
ocular ischemia
Posterior ciliary artery - Ophthalmic artery – Internal carotid
-Anterior ischemic optic neuropathy
-Amaurosis fugax (10-12%) or sudden total blindness
Permanent visual loss in 8-23%
Unilateral visual symptoms in first month, bilateral by second
***Second eye involved 65% of untreated patients (why it’s so imptnt to tx)
(Onset transient or sudden, partial or complete)
**Findings: sludging, disk pallor, papillitis, optic atrophy
-EOM palsies (2-14%):
-Central retinal artery occlusion (unusual)
what are some Extraocular Neurologic Complications of Giant Cell Arteritis
- Vertigo, hearing loss
- Hemianesthesia or paralysis of tongue
- Facial pain (facial artery)
- Mononeuritis multiplex
- Cervical myelopathy (anterior spinal artery)
- Carotid, vertebrobasilar TIA or stroke
T/F: negative temporal artery biopsy excludes GCA
FALSE
how do you tx GCA?
Moderately high doses initially (60-80 mg/d prednisone)
Slow taper (5-10 mg less, q3-4 wks)
Maintenance = 3-5 mg/day, usually x 3-4 years
**also remember Prophylaxis for osteoporosis
with glucocort
what is Polymyalgia Rheumatica?
DO NOT CONFUSE with polymyositis, there is no muscle inflamm or weakness
- Bilateral, symmetrical muscle pain, without weakness
- -Increased with motion
- -Stiffness most severe after rest
- -Muscle tenderness typical, arthritis is not
what is Polymyalgia Rheumatica?
DO NOT CONFUSE with polymyositis, there is no muscle inflamm or weakness
what is Behcet’s Syndrome:
what kind of clinical features do you see
A rare multisystem inflammatory disorder of unknown cause
- Recurrent oral and genital ulcers
- Ocular inflammation
- Systemic features (don’t worry about what)
Describe the ocular inflamm you see with Behcet’s:
Ocular inflammation (25 - 75%); recurrent → visual loss (25%)
- Uveitis (anterior, posterior, or panuveitis)
- Conjunctivitis, scleritis
- Retinal vasculitis (arterial/venous occlusion)
- Optic neuritis
