IMID/CTD Flashcards
Another term for Progressive Systemic Sclerosis?
Scleroderma
What are the types of Scleroderma?
- Systemic - Diffuse cutaneous, Limited cutaneous (CREST), Overlap.
- Localized - Morphea or Linear Scleroderma.
- Not Systemic.
What is the difference b/t Diffuse and Localized Cutaneous Scleroderma?
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.
What is Overlap type Scleroderma?
diffuse or limited scleroderma w/features of another CTD (SLE, PM, DM).
Rare (autoimmune) chronic connective tissue disease that causes hardening and tightening of she skin and connective tissues?
Scleroderma.
What is another name for Limited Scleroderma and what does it stand for?
“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.
What is Sjogren’s (Show-grins) Syndrome?
An autoimmune disorder characterized by infiltration of lymphocytes into the exocrine glands.
What is the pathogenesis of Sjogren’s Syndrome?
Lymphocytes infiltrate and destroy exocrine glands, especially the lacrimal and salivary glands.
What are the main manifestations of Sjogren’s Syndrome?
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.
Epidemiology of Scleroderma?
- 1/10,000.
- peak onset 45-65 y/o.
- F:M of 3:1.
- More severe in AAs.
What is Polymyositis?
Inflammatory muscle disease characterized by PAINLESS, symmetrical proximal muscle weakness.
Common features of Polymyositis?
- Insidious onset.
- Painless.
- Dysphagia (30%).
- Arthralgias.
- LE weakness usually >UE.
- Constitutional Symptoms.
Who gets Polymyositis?
F:M of 2:1, AA:White of 5:1, usually >20 y/o (most often Dx at 46-60), associated w/cancers.
What do pt’s w/Polymyositis have difficulty with?
Kneeling, climbing stairs, lifting arms overhead, arising from a seated position, holding head up (severe).
What is Vasculitis?
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.
What happens when a blood vessel becomes inflamed?
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).
What are the classifications of Vasculitis?
- 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.
What is the most common large-vessel vasculitis in humans?
Giant Cell Arteritis (mean age 74).
Typical Signs of Systemic Vasculitis of large vessels?
Limb claudication, asymmetric blood pressures, absence of pulses, bruits, aortic dilatation.
Typical Signs of Systemic Vasculitis of medium vessels?
Cutaneous nodules, ulcers, livedo reticularis, digital gangrene, mononeuritis multiplex, microaneurysms.
Typical Signs of Systemic Vasculitis of small vessels?
Purpura, vesiculobullous lesions, urticaria, glomerulonephritis, alveolar hemorrhage, cutaneous extravascular necrotizing granulomas, splinter hemorrhages, scleritis/episcleritis/uveitis.
When would you suspect vasculitis?
Constitutional Symptoms, arthralgias/myalgias, uncontrollable asthma/sinus issues for years, palpable purpura (small vessel), non-palpable purpura, hemorrhagic bulla, ulcers/erosions, urticaria (minimal pruritus).
What does IMID/CTD stand for?
Immune-mediated Inflammatory Disorders/Connective Tissue Disorders
What are the common IMID/CTD disorders?
- 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.
Common risk factors of IMID/CTD?
FH, Personal History of autoimmune dz, Female, infection, trauma.
What are autoreactive B cells?
The ‘bad’ version of B cells that make the harmful autoantibodies.
What are autoantibodies?
Produced by autoreactive B cells that work against the body’s healthy tissues.
Unlike antibodies that protect and defend the body.
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.
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.
RA risk factors?
Genetics, Female, age, smoking, other autoimmune diseases (IBD, Hashimotos, Graves, DM1, etc).
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.
What can chronic joint inflammation cause?
Erosions in the articular cartilage and bone leading to bone deformities.
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.
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.
Which joint is spared in RA?
DIPs.
What is important to inspect with RA?
The Feet!
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.
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.
What will radiographs show in RA?
Periarticular Osteopenia, Joint Space Narrowing, Marginal Joint Erosions.
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.
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.
Treatment for RA is a Stepwise Approach…
- NSAIDs:
- -Do not prevent joint damage.
- -Do not change disease course.
- -Reduces inflammation and pain. - Corticosteroids:
- -Good short term until DMARDs are start working.
- -Many side effects. - DMARDs:
- -Plaquenil, Azulfidine, MTX, Imuran, Leflunomide.
- -Lab monitoring before/after starting (CBC, CMP, Hepatitis, Quant Gold). - 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.
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.
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).
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.
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.
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.
What is the former name of Spondyloarthritis?
Seronegative arthritis or Spondyloarthropathy.
Common characteristics of Spondyloarthritis?
- Seronegative; sometimes HLA-B27+
- Inflammatory Axial Arthritis (Sacroiliitis, Spondylitis).
- Asymmetrical Oligoarthritis (<4 joints involved).
- ENTHESITIS: inflammation of the sites where tendon/ligaments insert into the bone.
- -Plantar fasciitis, achilles tendonitis, costochondritis. - Familial Association.
- May have DACTYLITIS (sausage finger/toe).
- Extra-articular features: skin, GU tract, AR, eyes (anterior uveitis).
- Overlap is likely b/t several of the causative conditions.
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.
What are the common characteristics of Psoriatic Arthritis?
- Arthritis may start before Psoriasis (15-20%) and vice versa.
- Arthritis occurs more often w/worse Psoriasis; but the skin involvement can also be mild.
- Nail pitting is predictive and there are many forms of arthritis.
Etiology of Psoriatic Arthritis?
- unknown.
- 40% have a Family History.
- HLA-B27 association.
Common Presentation of Psoriatic Arthritis?
- Asymmetrical.
- 1/3 may develop EYE inflammation.
- Dactylitis and Sausage digits common.
- Unlike RA, PsA can target DIP Joints.
Diagnostics for Psoriatic Arthritis?
- Labs:
- -HLA-B27.
- -ESR/CRP.
- -ANA/RF to r/o RA. - Radiographs:
- -“Pencil in cup deformity.”
Treatment for Psoriatic Arthritis?
- Same meds as RA; some work better for skin.
- Topical Steroids.
- PUVA therapy.
Complication of Psoriatic Arthritis?
Arthritis Mutilans - a severe form of chronic RA or Psoriatic arthritis characterized by resorption of bones and the consequent collapse.