IMID/CTD Flashcards
(141 cards)
1
Q
Another term for Progressive Systemic Sclerosis?
A
Scleroderma
2
Q
What are the types of Scleroderma?
A
- Systemic - Diffuse cutaneous, Limited cutaneous (CREST), Overlap.
- Localized - Morphea or Linear Scleroderma.
- Not Systemic.
3
Q
What is the difference b/t Diffuse and Localized Cutaneous Scleroderma?
A
Localized often only affects the skin and not other major organs, it is also non-progressive thickening; whereas, diffuse affects the skin, tissue underneath incl. blood vessels and major organs.
4
Q
What is Overlap type Scleroderma?
A
diffuse or limited scleroderma w/features of another CTD (SLE, PM, DM).
5
Q
Rare (autoimmune) chronic connective tissue disease that causes hardening and tightening of she skin and connective tissues?
A
Scleroderma.
6
Q
What is another name for Limited Scleroderma and what does it stand for?
A
“CREST” - mnemonic for Signs/Sx.
- Calcinosis - calcium deposits in the skin.
- Raynaud’s Phenomenon.
- Esophageal Dysfunction - acid reflux and decrease in motility of esophagus.
- Slcerodactyly - thickening and tightening of the skin on the fingers and hands.
- Telangiectasias - dilation of capillaries causing red marks on surface of skin.
7
Q
What is Sjogren’s (Show-grins) Syndrome?
A
An autoimmune disorder characterized by infiltration of lymphocytes into the exocrine glands.
8
Q
What is the pathogenesis of Sjogren’s Syndrome?
A
Lymphocytes infiltrate and destroy exocrine glands, especially the lacrimal and salivary glands.
9
Q
What are the main manifestations of Sjogren’s Syndrome?
A
Dry eyes/gritty sensation in eyes and dry mouth.
- Sx must be ongoing for at least 3 months.
- Other possible Sx – many:
- -dry skin/rashes.
- -joint pain, swelling.
- -persistent dry cough.
- -Lymphadenopathy.
- -Fatigue.
10
Q
Epidemiology of Scleroderma?
A
- 1/10,000.
- peak onset 45-65 y/o.
- F:M of 3:1.
- More severe in AAs.
11
Q
What is Polymyositis?
A
Inflammatory muscle disease characterized by PAINLESS, symmetrical proximal muscle weakness.
12
Q
Common features of Polymyositis?
A
- Insidious onset.
- Painless.
- Dysphagia (30%).
- Arthralgias.
- LE weakness usually >UE.
- Constitutional Symptoms.
13
Q
Who gets Polymyositis?
A
F:M of 2:1, AA:White of 5:1, usually >20 y/o (most often Dx at 46-60), associated w/cancers.
14
Q
What do pt’s w/Polymyositis have difficulty with?
A
Kneeling, climbing stairs, lifting arms overhead, arising from a seated position, holding head up (severe).
15
Q
What is Vasculitis?
A
An inflammation of the blood vessels in the body where the immune system mistakenly attacks the body’s own blood vessels, causing them to become inflamed.
16
Q
What happens when a blood vessel becomes inflamed?
A
The blood vessels will narrow making it more difficult for blood to get through, they can close off completely (occlusion); in rare cases, stretch or weaken so much that they bulge (aneurysm) and possibly burst (aneurysm rupture).
17
Q
What are the classifications of Vasculitis?
A
- Large-vessel: involve the aorta and it’s branches.
- Medium-vessel: affect medium-size and small arteries of the kidneys, liver, heart, etc.
- Small-vessel: predominantly targets capillaries and post-capillary venules.
18
Q
What is the most common large-vessel vasculitis in humans?
A
Giant Cell Arteritis (mean age 74).
19
Q
Typical Signs of Systemic Vasculitis of large vessels?
A
Limb claudication, asymmetric blood pressures, absence of pulses, bruits, aortic dilatation.
20
Q
Typical Signs of Systemic Vasculitis of medium vessels?
A
Cutaneous nodules, ulcers, livedo reticularis, digital gangrene, mononeuritis multiplex, microaneurysms.
21
Q
Typical Signs of Systemic Vasculitis of small vessels?
A
Purpura, vesiculobullous lesions, urticaria, glomerulonephritis, alveolar hemorrhage, cutaneous extravascular necrotizing granulomas, splinter hemorrhages, scleritis/episcleritis/uveitis.
22
Q
When would you suspect vasculitis?
A
Constitutional Symptoms, arthralgias/myalgias, uncontrollable asthma/sinus issues for years, palpable purpura (small vessel), non-palpable purpura, hemorrhagic bulla, ulcers/erosions, urticaria (minimal pruritus).
23
Q
What does IMID/CTD stand for?
A
Immune-mediated Inflammatory Disorders/Connective Tissue Disorders
24
Q
What are the common IMID/CTD disorders?
A
- Rheumatoid Arthritis (RA).
- Spondyloarthritis
- -Psoriatic Arthritis.
- -Ankylosing Spondylitis.
- -Reactive (Reiter’s Arthritis).
- -IBD-arthritis. - PMR – Polymyalgia Rheumatica.
- SLE.
- Scleroderma.
- Sjogren’s Syndrome.
- Polymyositis/Dermatomyositis.
- Vasculitis - GCA, etc.
25
Common risk factors of IMID/CTD?
FH, Personal History of autoimmune dz, Female, infection, trauma.
26
What are autoreactive B cells?
The 'bad' version of B cells that make the harmful autoantibodies.
27
What are autoantibodies?
Produced by autoreactive B cells that work against the body's healthy tissues.
Unlike antibodies that protect and defend the body.
28
What is Rheumatoid Arthritis?
A chronic, systemic, inflammatory disorder of unknown cause that is characterized by symmetrical, polyarticular pain and swelling, morning stiffness, and fatigue.
**It is the most common inflammatory arthritis and the 2nd most common arthritis.
29
Who does RA affect?
- 1% of the world's population.
- 3:1 ratio, female to male.
- peak age 35-50.
- Causes a high incidence of disability and decreased ADLs.
30
RA risk factors?
Genetics, Female, age, smoking, other autoimmune diseases (IBD, Hashimotos, Graves, DM1, etc).
31
Pathogenesis of RA?
Genetic predisposition -- external trigger sets off autoimmune reaction (environmental or infection) -- T Cells perceive something in the synovium as an antigen -- inflammatory cascade -- excessive amounts of pro-inflammatory cytokines (TNF-alpha, IL1, IL6, etc), which bind to the joints leading to synovial membrane hypertrophy (PANNUS), joint swelling and chronic joint inflammation along w/ potential for extra-articular manifestations.
32
What can chronic joint inflammation cause?
Erosions in the articular cartilage and bone leading to bone deformities.
33
What are the CLINICAL features of RA?
* Systemic inflammation.
* *Morning stiffness >1 hr!!
* Polyarthritis.
* Symmetrical distribution.
* GELLING phenomenon.
* Decreased ability to perform ADLs.
* ERYTHEMA, WARMTH OR SWELLING OF JOINTS (swelling of joints feels 'doughy').
* Fatigue.
* Constitutional symptoms.
34
What are the most commonly affected joints in RA?
**MCP, Wrist, PIP.
- -MCP will have a loss of definition as a fist is made.
- -Bouchard's nodes of the PIP, feel hard and bony.
-Also, Knee, MTP, Shoulder, Ankle, C-Spine, Hip, Elbow, TMJ.
35
Which joint is spared in RA?
DIPs.
36
What is important to inspect with RA?
The Feet!
37
Extra-articular manifestations of RA?
* Eye inflammation - scleritis, episcleritis, uveitis, glaucoma, cataracts, corneal ulcers, blindness.
* Serositis - pericarditis, pleuritis.
* Vasculitis - can lead to amputation, nerve damage (mononeuritis multiplex).
* Sicca Complex - dry eyes, dry mouth.
* Rheumatoid Nodules - feel like a lump under the skin.
38
Laboratory Studies for RA?
**RA is a clinical diagnosis but...
- RF (Rheumatoid Factor), +80% of pt's.
- Anti-CCP (Cyclic Citrullinated Peptide)
- --more specific than RF (98%), can be an indicator of more severe, aggressive forms of RA.
- Acute Phase reactants: ESR, CRP, Platelets.
**Seronegative RA exists.
39
What will radiographs show in RA?
Periarticular Osteopenia, Joint Space Narrowing, Marginal Joint Erosions.
40
Other Diagnostic Imaging for RA?
* Ultrasound:
- -Superior to radiographs at picking up erosions in early RA.
- -Requires special training.
* MRI:
- -Sensitive at picking up early joint damage.
- -Expensive; sometimes not covered by insurance.
41
Fluid analysis of joint aspiration in RA?
* Cell count and crystal analysis.
* Culture and Gram Stain.
* *ELEVATED WBCs = inflammatory but >100,000 is considered septic.
* *Synovial fluid will be darker/more turbid, in inflammatory arthritis.
42
Treatment for RA is a Stepwise Approach...
1. NSAIDs:
- -Do not prevent joint damage.
- -Do not change disease course.
- -Reduces inflammation and pain.
2. Corticosteroids:
- -Good short term until DMARDs are start working.
- -Many side effects.
3. DMARDs:
- -Plaquenil, Azulfidine, MTX, Imuran, Leflunomide.
- -Lab monitoring before/after starting (CBC, CMP, Hepatitis, Quant Gold).
4. Biologics (e.g, Humira, Enbrel, Xeljanz):
- -TNF-alpha inhibitors, IL inhibitors, JAk inhibitors, T/B cell targets.
- -Calm down inflammation.
- -Increased risk of infections.
- -Lab monitoring before and after.
43
What are some of the many AEs of Corticosteroids?
Weight gain, DM, poor healing, cataracts, glaucoma, thinning of skin/STRIAE, moon facies, Increased BP, Osteoporosis.
44
What is an important treatment consideration for RA patients?
**They are IMMUNOCOMPROMISED!
- Diligent treatment of infections.
- D/C biologics while active infection.
- Vax up-to-date; no live vax.
- TB testing prior to biologic initiation.
- Pt's at increased risk of heart disease (monitor cholesterol, assess other cardiac risk factors).
45
What if your patient chooses NO treatment of their RA?
Having RA...
- CVD 10 yrs earlier than others.
- 2x increased risk of malignancy.
- Increased GI Bleeds.
- Increased risk of pulmonary disease.
- 6-8x increased risk of infections.
46
What is Spondyloarthritis?
An umbrella term for a group of related inflammatory disorders that involve the axial skeleton and having negative serostatus; it may also involve the appendicular skeleton.
47
What are the main types of Spondyloarthritis?
Ankylosing Spondylitis, Reactive Arthritis, IBD-Arthritis, Psoriatic Arthritis (PsA).
**Ankylosing Spondylitis affects the axial joints (spine, SI joints).
**PsA, Reactive Arthritis, IBD-arthritis affects the peripheral joints.
48
What is the former name of Spondyloarthritis?
Seronegative arthritis or Spondyloarthropathy.
49
Common characteristics of Spondyloarthritis?
1. Seronegative; sometimes HLA-B27+
2. Inflammatory Axial Arthritis (Sacroiliitis, Spondylitis).
3. Asymmetrical Oligoarthritis (<4 joints involved).
4. ENTHESITIS: inflammation of the sites where tendon/ligaments insert into the bone.
- -Plantar fasciitis, achilles tendonitis, costochondritis.
5. Familial Association.
6. May have DACTYLITIS (sausage finger/toe).
7. Extra-articular features: skin, GU tract, AR, eyes (anterior uveitis).
8. Overlap is likely b/t several of the causative conditions.
50
An autoimmune and inflammatory arthritis that usually occurs in combination with the skin condition Psoriasis? Prevalence?
Psoriatic arthritis; affects 5% of Psoriasis patients.
- More prevalent in Caucasian pt's.
- M:F ratio of 1:1.
- Presents in ages 35-55.
51
What are the common characteristics of Psoriatic Arthritis?
1. Arthritis may start before Psoriasis (15-20%) and vice versa.
2. Arthritis occurs more often w/worse Psoriasis; but the skin involvement can also be mild.
3. Nail pitting is predictive and there are many forms of arthritis.
52
Etiology of Psoriatic Arthritis?
1. unknown.
2. 40% have a Family History.
3. HLA-B27 association.
53
Common Presentation of Psoriatic Arthritis?
1. Asymmetrical.
2. 1/3 may develop EYE inflammation.
3. Dactylitis and Sausage digits common.
4. Unlike RA, PsA can target DIP Joints.
54
Diagnostics for Psoriatic Arthritis?
1. Labs:
- -HLA-B27.
- -ESR/CRP.
- -ANA/RF to r/o RA.
2. Radiographs:
- -"Pencil in cup deformity."
55
Treatment for Psoriatic Arthritis?
1. Same meds as RA; some work better for skin.
2. Topical Steroids.
3. PUVA therapy.
56
Complication of Psoriatic Arthritis?
Arthritis Mutilans - a severe form of chronic RA or Psoriatic arthritis characterized by resorption of bones and the consequent collapse.
57
A chronic, inflammatory arthritis that primarily affects the Sacroiliac (SI) joints and the axial skeleton?
Ankylosing Spondylitis (AS).
*Over time, the vertebrae can fuse causing the spine to be less flexible resulting in a hunched-forward position.
58
What are the Hallmarks of Ankylosing Spondylitis?
1. Inflammatory back pain.
2. Bony union of joints in the back (fused together).
3. Uveitis.
4. Tendon inflammation.
5. Asymmetric and axial joint inflammation.
59
Epidemiology and prevalence of AS?
1. Male > Female; 5:1 male.
2. Ave. age of onset:
- -teenagers to early adulthood.
- -mean onset 3rd decade.
60
What is 90% positive in AS?
HLA-B27 (blood test).
61
What is HLA-B27?
Blood test; protein on the surface of WBC.
62
Pathophysiology of AS?
Completely unknown -- clear genetic predisposition; HLA-B27+ -- environmental factor triggers or possible infection.
63
Some x-ray findings of AS?
Sacroiliitis and bamboo spine.
64
What is the classification criteria for Ankylosing Spondylitis?
1. Inflammatory Spinal Pain:
* Age less than 40-45.
* Back pain that wakes the pt up in the early morning.
* Improved by exercise.
2. Asymmetric or mostly LE Synovitis.
3. And 1 or more of the following:
- FH.
- Enthesopathy = inflammation where the tendons or ligaments insert.
- Sacroiliitis.
- Buttock pain alt. left to right.
- Assoc. condition like Psoriasis, IBD, Urethritis.
65
Treatment goals for AS?
1. Ideally, diagnose early and begin disease modifying therapy before FUSION occurs.
2. May treat spinal and peripheral manifestations differently.
3. Assess need for local or systemic therapy.
66
Local treatments for Ankylosing Spondylitis?
1. PT is very important.
2. Heat often helps locally.
3. Intra-articular injections.
4. Emphasize posture, sleeping positions, very thin pillows (vertebrae tend to fuse in functional positions).
67
Systemic therapy for Ankylosing Spondylitis?
1. Spinal Activity:
- Biologics (TNF and IL-7 inhibitors proven to prevent progression).
- NSAIDs can help with Sx.
2. Peripheral Manifestations:
- NSAIDs.
- Sulfasalazine.
- Prednisone, particularly for flares.
- MTX.
68
"Can't pee, can't see, can't climb a tree."
Reactive Arthritis.
69
Previous name for Reactive Arthritis?
Reiter's Syndrome.
70
What is the TRIAD for Reactive Arthritis?
* Urethritis.
* Conjunctivitis.
* Arthritis.
71
What is the etiology behind Reactive Arthritis?
1. Associated w/GI and GU infections:
- C. Diff, Shigella, Salmonella, Campylobacter, Chlamydia/Gonorrhea, Cervicitis, Prostatitis.
* *Sx develop 2-6 weeks after infection.
72
Prevalence of Reactive Arthritis?
1. most pt's 20-40 y/o.
2. GI cases: M:F ratio of 1:1.
3. GU cases: M:F ratio of 9:1.
73
Define the arthritis in Reactive Arthritis?
1. Asymmetrical.
2. Extremity or low back pain.
3. Oligoarthritis or Pauciarticular (2-4 joints).
74
Name some skin manifestations involved with Reactive Arthritis?
1. Keratoderma Blennorrhagica -- skin lesions that commonly manifest on the palms and soles.
2. Circinate Balanitis -- serpiginous ring-shaped dermatitis of the glans penis.
75
Diagnostics for Reactive Arthritis?
1. Labs:
- -ESR/CRP, HLA-B27, CBC, Arthrocentesis, Cultures.
2. XRs:
- -Negative in early Dz.
- -Periosteal Rxn at tendon attachments (Enthesitis).
- -Plantar heel spurs.
- -Erosions.
- -Sacroiliitis.
- -Ankylosing Spondylitis.
76
Treatment for Reactive Arthritis?
1. Abx.
2. NSAIDs.
3. Corticosteroids.
4. DMARDS -- MTX, Azulfidine.
*Typically, self-limited but may take a long time.
77
How often will pt's with Reactive Arthritis develop long standing arthritis?
15% of pt's.
78
Occurs about 7-20% in pt's with Crohn's or UC and largely affects the lower extremities?
IBD associated Arthritis.
79
Characteristics of IBD associated Arthritis?
1. M = F.
2. Sx are worse w/ UNCONTROLLED IBD.
3. Treat the IBD adequately and Sx will most likely resolve.
80
Pharmacologic treatment for IBD?
- Sulfasalazine (DMARD).
- Corticosteroids.
- TNF-alpha blockers (Humira, Remicade).
81
What does PMR stand for?
Polymyalgia Rheumatica.
82
A symmetrical inflammatory disorder causing muscle pain and stiffness of the proximal muscles, especially the shoulder girdle and hip girdle?
Polymyalgia Rheumatica (PMR).
*Has an immediate onset and Systemic Sx.
83
Prevalence of PMR?
1. always >50 y/o (70+ is most common).
2. Caucasian > others.
3. Women > Men.
84
Physical Exam findings in PMR.
1. AROM = decreased due to pain.
2. PROM = may be normal.
3. Strength = may be normal.
4. SWELLING is possible; hands, wrists, knees.
5. Deep Knee Bend -- can't do!
85
Diagnosis of Polymyalgia Rheumatica?
- Elevated ESR/CRP!!!
| - CK (muscle enzyme) is NOT elevated.
86
Treatment of PMR?
1. STEROIDS (Prednisone) 15-20mg Qday, relief within days. If NOT...think about DDx.
2. Treat for 6-18 months.
3. CRP/ESR will normalize w/steroid treatment.
87
What will 10% of PMR patients develop?
Giant Cell Arteritis (GCA); vasculitis.
88
What is the most common finding in SLE?
Malar rash (aka butterfly rash) of the face that spares the nasolabial fold.
89
A highly variable and complex systemic inflammatory autoimmune disease that can target many organ systems?
Systemic Lupus Erythematosus.
- Mild to Life-threatening.
- each pt. has a unique disease presentation.
90
What organ systems does SLE target?
Skin, joints, mucosa, heart, lungs, kidneys, brain.
91
Epidemiology of SLE?
1. Affects 1:1000 people.
2. F:M ratio of 9:1 (men have more severe Sx).
3. AAs > Asians > Caucasians.
4. AGE: women of childbearing age (often 15-40), but varies.
5. Genetics:
- 5% chance if 1st degree relative affected.
- 25-50% chance if identical twin affected.
92
Pathophysiology of Systemic Lupus Erythematosus?
Genetic factors -- unknown antigen starts process -- autoantibodies formed (ANA) -- Abs may contribute to patho by forming "immune complexes," esp in kidney -- B-Cells (Ab producers) that react to self-antigens abnormally activated -- Inflammatory cascade/response.
93
Lupus Characteristics?
- The "Great Imitator."
- On ave., takes about 6 yrs before pt's are correctly diagnosed.
- Combine HPI, PE, Labs.
- Hx is key: Sx may not present simultaneously.
- Many pt's develop "OVERLAP DISEASES." (overlap of autoimmune diseases).
94
What is the most sensitive and accurate test for SLE?
ANA - antinuclear autoantibodies via IMMUNOFLUORESCENCE.
* Will pick up >95% of people with SLE.
* A negative ANA is strong evidence AGAINST the presence of SLE.
* +ANA does not always equal lupus; +in variety of other inflammatory and autoimmune diseases.
95
What other diseases is an ANA blood test positive?
1. SLE 95%.
2. Scleroderma 60-70%.
3. RA 25-30%.
4. Sjogrens Syndrome 50-60%.
5. Dermatomyositis 10-50%.
6. Polyarteritis 10%.
96
Classification Criteria for SLE?
1. +ANA, >180 titer (+ANA does not = lupus).
2. Other criteria...weighted after r/o other causes:
- Fever, Serositis, mucocutaneous, Neuropsych.
3. Other criteria:
- Renal (proteinuria, renal Bx showing lupus nephritis).
- MSK (joint pain, swelling).
- Hematologic (thrombocytopenia, leukopenia).
- Antiphospholipid antibodies.
- Complement proteins.
- Specific antibodies (Anti-dsDNA or anti-smith).
97
What to complement proteins tell us?
How well the immune system is working.
98
Weighted criteria must add up to what to be classified as lupus?
10 or more.
-more points are given to more serious complications (eg. renal biopsy showing lupus nephritis - 10 pts vs oral ulcers - 2 pts).
99
What are some generalized symptoms associated with SLE?
**Weight loss, fever, fatigue, aching, weakness.
100
Other common symptoms associated with SLE?
Various types of rashes (MC malar rash), photosensitivity, alopecia, oral ulcers (painless), arthralgias/myalgias/arthritis, GI distress, anorexia, etc.
101
Common hematologic/vascular manifestations associated with SLE?
- Leukopenia.
- Lymphopenia.
- Hemolytic anemia (anemia due to shortened survival of circulating RBCs due to premature destruction).
- Thrombocytopenia.
- Lupus anticoagulant.
- Raynaud's Phenomenon.
- Antibodies to coagulation factors.
102
What is the Triphasic color change associated w/Raynaud's Phenomenon?
1. White -- blanching in response to temp change or emotional stress; due to arterial vasospasm.
2. Blue -- cyanosis due to venous stasis.
3. Red -- vasodilation, due to arterial hyperemia (excess of blood in the vessels supplying an organ).
103
Pulmonary and cardiac manifestations associated with SLE?
1. Fibrosis/Pneumonitis.
2. Pulmonary HTN.
3. Myocarditis.
4. Premature CAD.
5. LIBMAN SACKS LESIONS!
6. Serositis:
- Pleuritis.
- Pericarditis.
- Pericardial effusion.
- Pleural effusion.
104
What is the most common cardiac manifestation associated with SLE?
Libman Sacks Lesions -- fibrinous vegetations that may develop anywhere on the endocardial surface of the heart w/ a propensity for the left heart valves; particularly the ventricular surface of the mitral valve.
105
Common neurological manifestations of SLE?
-Depression, psychosis, seizures, coma, myelitis, stroke, lupus cerebritis.
106
Once symptoms + ANA test, what do we do next?
1. dsDNA antibodies -- go up in active lupus.
2. C3/C4 -- go down in active lupus.
3. Anti-Smith Antibody -- more specific for lupus.
4. Anti-histone antibody -- specific for drug-induced lupus.
5. CBC, Renal function tests -- UA, BUN/Cr.
107
X-ray in SLE?
-No erosions and joint spaces preserved.
**Ligaments have become lax causing the ulnar deviation.
108
Treatment of SLE?
1. Treat skin and joint disease w/milder agents.
2. Plaquenil (Hydroxychloroquine) in the water!
- -Mildest agent, safe in pregnancy.
- -Lessens severity and frequency of flares.
- -Usually dose is 200mg BID (weight-dependent).
- -Eye exams w/visual field Q6-12 months.
3. NSAIDs.
4. Corticosteroids used as a bridging agent for acute flares; watch side effects.
5. Internal organ involvement requires immunosuppression beyond Hydroxychloroquine.
- -Drug choice is dependent on disease manifestations (MTX, Azathioprine, Imuran, CellCept, Cytoxan).
109
Why is drug monitoring important in the treatment of SLE?
1. The stronger drugs require lab monitoring Q2-3 months to ensure not anemia or liver toxicity.
2. Cytoxan has added AEs of potentially affecting fertility (premature ovarian failure).
110
Guidelines for Prophylactic treatment in SLE?
- Anyone on prednisone should be taking Calcium and Vitamin D to prevent bone loss.
- Baseline DXA scan.
- Bisphosphonate use is possible.
- Generally...all lupus pt's should be taking ASA because of accelerated atherosclerotic risk.
111
Define DIFFUSE Scleroderma?
Thickening/Tightening of the skin w/WIDESPREAD AND RAPID PROGRESSION.
* *Early internal organ involvement:
- Skin 98%, GI tract 90%, Lungs 65%, Heart 25%, Kidney 15%.
**SIGNIFICANT MORTALITY.
*20% will have +Scl70 Antibody.
112
Common manifestations found on Physical Exam of Slceroderma?
- Loss of skin lines, waxy tight skin.
- Exophthalmos.
- Decreased oral aperture (Microstomia or mouse facies, protruding teeth).
- Inability to close the mouth, protruding teeth.
- Telangiectasias.
- Sclerodactyly (localized thickening and tightening of the fingers and toes).
- Acral ulcers (acral = peripheral parts of extremities).
- Raynaud's.
- Claw like deformities of the hands.
- Calcinosis (calcium deposits of the soft tissue).
- Loss of ROM of hands and fingers.
- Decreased strength.
- Abnormal nail folds.
- Salt and pepper skin.
113
Treatment of Systemic Sclerosis/Scleroderma?
1. PPIs (esophageal dysmotility).
2. CCBs -- Raynaud's.
3. Tracleer (Pulmonary HTN).
4. Pain meds -- ulcerations.
5. Other -- d/c smoking, keep warm, gentle exercise.
114
Restricted (distal), NON-PROGRESSIVE skin thickening?
Limited Scleroderma (CREST).
* *Prognosis is good.
* *Anti-centromere antibody.
115
Organ involvement in Limited Scleroderma?
- Renal and lung involvement is rare.
| - Organ involvement occurs later, except for PAH.
116
What are the serious complications with Limited Scleroderma (CREST)?
1. Pulmonary HTN.
| 2. Interstitial Lung Disease (ILD) 40%.
117
What are the serious complications with Diffuse Scleroderma?
1. Scleroderma Renal Crisis, which can present as CHF, HTN Encephalopathy -- monitor BP.
2. Pulmonary Complications (ILD).
- -check PFTs and ECHO yearly.
3. Cardiac Disease (conduction abnormalities).
- -pericarditis or large pericardial effusion/tamponade; treat w/NSAIDs or steroids but care w/steroids, which can increase risk of renal crisis.
4. Significant mortality.
118
Specific aspects of the PE that should be completed in Sjogren's Disease?
1. Salivary Pooling for dry mouth.
2. Schirmer's test for dry eye.
3. Lymph node exam - Lymphoma is common.
4. Heart and Lung Exam (pericardial friction rub, bibasilar crackles are common).
119
Diagnosis of Sjogren's Syndrome?
1. SS-A and/or SS-B antibodies.
2. Schirmer's Test.
3. Lip/Salivary Gland biopsy.
120
Sjogren's Syndrome Complications?
1. Dry mouth -- dental caries, thrush 30-70% (yeast infx), dysphagia.
- -Dental caries due to lack of clearance of acid, lack of saliva and lack of proteins to counter decay.
2. Dry eyes -- light sensitivity, blurred vision, corneal ulcers.
3. Inflammation of lungs, kidneys, heart, GI -- Pneumonitis, interstitial nephritis, pericarditis (
121
Treatment for Sjogren's Syndrome?
**Treatment is guided by Symptoms.
1. OTC mouth and eye moisturizers.
2. Increased hydration.
3. Rx meds for increased saliva production.
4. NSAIDs for joint pain.
5. Plaquenil or MTX for fatigue, joint complaints, Extraocular/Extraoral Sx.
6. Rx eye drops available.
7. REGULAR DENTAL CHECK-UPs!
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Polymyositis Complications?
1. Aspiration Pneumonia.
2. ILD 5-30%.
3. Pericarditis/Myocarditis -- rare.
4. Arthritis or arthralgias.
5. Sometimes...CANCER -- ensure pt is uptodate on Colonoscopy, mammogram, etc.
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Labs that are elevated in Polymyositis?
1. Creatine Kinase (CK), Aldolase, LFTs -- MC.
2. CBC.
3. ESR/CRP.
4. Anti-Jo Antibody 20% = poor prognosis.
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Other testing involved in diagnosing Polymyositis?
1. Muscle biopsy (degeneration, necrosis) -- very important to get because of other causes of weakness, including drugs (alcohol, colchicine, statins, thyroid, steroids) -- Bx will differentiate.
2. Electromyography (fibrillations, repetitive discharges).
3. CT Scanning.
4. MRI.
5. Cancer screenings.
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What is the difference between Polymyositis and Polymyalgia Rheumatica?
Polymyositis is usually painless, muscle weakness and possible muscle destruction.
Polymyalgia Rheumatica involves muscle pain.
**Both affect the proximal muscles, shoulder girdle and hip girdle.
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What can Polymyositis be a sign of?
*Existing cancer or increase risk of cancer.
MC = breast, lung, ovarian, stomach, intestine, throat, pancreatic, bladder and hodgkins.
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Treatment for Polymyositis?
1. Corticosteroids are mainstay (Prednisone).
2. If recurrent:
- -MTX, Imuran (DMARDs).
3. If still recurs:
- -TNF-alpha blockers (Immmunosuppressant).
- -Other immunosuppressants (Mycophenolate, Mofetil, Cyclophosphamide, IVIG).
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A rare Connective Tissue Disease that includes Polymyositis + Rashes...
Dermatomyositis.
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What is the presenting feature of Dermatomyositis?
**Rash in 40%. Types:
- Heliotrope rash.
- GOTTRON PAPULES (Pathognomic).
- Nailbed Telangiectasias.
- Shawl sign (shoulders) and/or Holster Sign (thigh).
- Poikiloderma Photodistribution.
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Treatment and considerations of Dermatomyositis?
1. Treatment - same as Polymyositis (Prednisone).
2. Polymyositis and Dermatomyositis are BOTH associated w/increased cancer risk or sign of cancer (DM>PM).
**Cancer Screening**
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What does MCTD and UCTD stand for?
* Mixed Connective Tissue Disease.
| * Undifferentiated Connective Tissue Disease.
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Has features of more than one autoimmune inflammatory disorders...the pt's symptoms do not fit into one category?
MCTD or UCTD.
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Overlap diseases have features of?
RA, SLE, Scleroderma, Sjogren's and Polymyositis.
**Etiology unknown.
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What is the difference between MCTD vs. UCTD?
1. MCTD:
- Often has develop defined features of one of the overlap diseases (SLE, Polymyositis, Scleroderma).
- ANA+, Centromere pattern and +RNP antibody present in 95-100%; usually presence of Raynaud's.
2. UCTD:
- Less defined features.
- ANA+, but no specific antibody other than +ANA.
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Etiology of Vasculitis?
1. Infections (Hepatitis, HIV, CMV, EBV, Parvovirus).
2. Malignancies.
3. Drugs (Cocaine).
4. IMID/CTD (SLE, RA).
5. Cryoproteins.
6. Idiopathic.
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Inflammation of extracranial branches of carotid arteries, branches of aorta aka Large Vessel?
Vasculitis.
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40% of pt's with GCA will have what?
Polymyalgia Rheumatica.
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Clinical presentation of Vasculitis?
1. Sudden Onset.
2. Headache.
3. Vision changes that can lead to vision loss.
4. Jaw claudication.
5. Arm/Leg claudication.
6. Abnormal temporal artery.
7. Bruits.
8. Asymmetric blood pressures.
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Gold Standard diagnostic of GCA?
Temporal Artery Biopsy.
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Diagnostics of GCA?
- ESR/CRP, Alk Phos, Platelets will be high.
- Temporal Artery Bx.
- MRA to look for large vessel involvement.
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Treatment of Giant Cell Arteritis?
- Prednisone (40-60 mg Qday x 4 wks, then taper).
- Usual course of 2-4 yrs.
- ASA to reduce risk of stroke and blindness.
- Annual monitoring for Aneurysm development.
