Week 12 Rheumatologic Disorders Flashcards
Osteoarthritis
* Common cause of joint pain and disability
* “Wear and tear” arthritis
* Cartilage breakdown, bony overgrowth
* Mild inflammatory component, Genetics, Prior Injuries
* Commonly affects the hands, CMC joint (base of thumb), knees, spine, hips
Osteoarthritis presentation
Increasing pain/aching with activity
Overuse
Morning stiffness < 30 minutes
GONA
1.Pain with activity
2.Swelling
3.Stiffness
4.Deformity
5.Decreased range of motion, creaking joints (crepitus)
6.Instability
7.Loss of function
OA diagnostics
X-ray films: joint space narrowing d/t cartilage loss, osteophytes
Mostly clinical diagnosis
OA treatment
- PT: cannot rebuild cartilage, so we buildup the muscles surrounding the joint to compensate and strengthen
- OT: Can help with building hand strength and find tools to help with ADLs, can help with splinting
Analegsics
* Tylenol (1st line) 3g or < per day
* NSAIDs (If not Contraind., may need GI protection - PPI, check GFR)
* Careful w/comorbid. cardiac disease, kidney disease
* Steroids- may help with IA joint injections. There is a low-grade inflammatory component of OA.
* U/S guided, Synvisc (cushion support)
* Limit to Q 3-4 months for next injection to reduce risks of bone damage
* Conservative management until it is unbearable pain, then replacements. Want to send to ortho before they become too old or ill to be a good candidate for replacement
* Joint replacement: Hips, knees, ankles, shoulders, etc.
Rheumatoid Arthritis
- Autoimmune condition affecting the synovial membranes leading to synovial thickening and inflammatory joint effusions – hot fluid in joints
- Leads to symmetrical, peripheral, joint swelling, pain, and stiffness
- Untreated may lead to irreversible joint damage through destruction of ligaments, cartilage, and bone leading to loss of function - Inflammation in the joint leads to destruction of ligaments, causing sublocation of joints
- Cartilage and bone destruction causes bony deformities that are irreversible, sometimes require joint replacement.
- You can have irreversible damage within the first few months of onset.
- Chronic inflammation leads to bony joint damage, cartilage breakdown, laxity of the tendons and ligaments leading to subluxation of the joints
Women > Men
40-60y/o
Key Features of RA + PE
Clinical DX?
Symptoms > 6 weeks’ duration
* Lifelong condition (usually)
Swollen Inflamed Joints
* Palpable synovial swelling leading to pain, stiffness, tenderness, hot, sometimes erythematous joints
* Morning stiffness >1 hour, stiffness with immobility that improves with activity
Symmetrical and polyarticular
* Typically involves wrists, MCP, PIP, & MTP joints, but can also involve the elbows, ankles, knees, shoulders, hips
* Typically spares certain joints
* Thoracolumbar spine
* DIPs of the fingers and IPs of the toes
DX CLINICALLY
PE
- Joint swelling w/fluid, joint tenderness (tender,warm,swollen)
- Late stages: classic deformities, nodules in skin
RA things to ask
- Recent illness? Fevers, chills? Sick contacts?
- R/o ifxn arthritis
- How long does morning stiffness last?
- Family history of arthritis?
- History of rashes? Psoriasis?
- Respond to NSAIDs, Tylenol?
When thinking about a possible inflammatory arthritis…get a comprehensive hx, ruling out possibility of infectious disease as well (Lyme, Parvovirus)
Positive response to NSAIDs? → May point in the direction of an inflammatory disorder.
RA diagnostics
Best lab test?
X-ray - see bony erosions - help to monitor disease down line (aggressive tx of erosions present)
RF (poor specificity) high titer early = BAD
Anti-CCP antibody - diagnostic + prognostic tool
ESR/CRP
Synovial fluid analysis
Can do this workup prior to sending them to Rheumatology
Key points for RA overall
- The sicker they are and the faster they get that way, the worse prognosis
- Early intervention can make big a difference in long term outcomes
- Essential to establish a treatment plan early in the disease
- Quick referral is key. Better to send someone who doesn’t have RA, than to not send someone who does.
- Swelling is confined to areas of joint capsule
- Synovial thickening feels like a firm sponge: “Boggy” Joints, Swan neck and Boutonniere Deformities
People rarely end up with hands like these now because of the treatments we have
Systemic manifestations a/w RA
- Fatigue
- Raynaud’s Phenomenon
- dry eyes, dry mouth (secondary Sjogren’s Syndrome)
- Interstitial Lung Disease
- Pleuritis, pericarditis
- Vasculitis
At RISK for:
* CAD
* HTN
* MI
* Vascular disease
* Lymphoma
RA treatment principles
- REFER to Rheumatology
- Determine where the patient stands in the spectrum of disease
- When damage begins early, start aggressive treatment early
- Use the safest treatment plan that matches the aggressiveness of the disease
- Monitor treatment for toxicity
- Monitor disease activity, revise Rx as needed
- What we are thinking about when beginning treatments…we want to get the best clinical results with the least amount of medication risk/toxicity
Pharm RA treatment
NSAIDs
* Symptomatic relief, No change in disease progression
Prednisone/Medrol (low dose long term)
* May substitute if NSAID not efficacious
* Also used as bridge therapy
Intra-articular steroids
* Useful for flares in individual joints
DMARDS (May be on 1-3 at one time) Disease-modifying-antirheumatic-drugs
* Plaquenil (Hydroxychloroquine)
* Sulfasalazine
* Methotrexate
* Leflunomide
* Azathioprine
* Biologics (often in combination with DMARD or alone)
- Prednisone works for everyone, but the goal is to get them off the prednisone because of side effects.
- In primary care, prior to their referral, you might try for symptom control NSAIDs for mild inflammation or Prednisone for Moderate to Severe inflammation.
- Avoid “Bursts” if they have diffuse swelling, and try a longer taper (3-4 weeks starting with 15-20 mg and dropping by 5 mg each week). If only 1-2 joints, may do a burst folowed by NSAIDS (not at the same time)
Bioloigics for RA tx (unsure if need to know for PC)
INHIBIT INFLAM RESPONSE
TNF inhibitors
* Remicade IV
* Enbrel SC
* Humira SC
* Cimzia SC (2x/mo)
* Simponi SC (1X/mo)
Costimulation Modulator
* Orencia SC & IV
B Cell Depletion
* Rituxan I
IL1 inhibitor
* Anikinra SC (not so effective in RA but works well in auto-inflammatory diseases like FMF)
* Kevzara SC 200mg (2X/month)
IL-6 inhibitor
* Actemra SC & IV (q 4 wks.)
“Jak” Kinase Inhibitor
* Tofacitinib (Xeljanz), PO daily medication. JAK’s play a role in inflammation of the joints, if inhibited: less inflammation, pain and stiffness.
PDE4 Inhibitor (Ps Arth)
* Otezla (Apremilast) 30 mg BID maintenance
IL 17 Inhibitor (Ps Arth)
* Taltz 80 mg SC once a month
IL 12/23 antibody Ps Arth, Crohns
* Stelara (Ustekinumab) 90 mg every 8 weeks
When to refer for RA
- Multiple inflamed joints
- Isolated inflamed joint not explained by something else (gout, infection, injury)
- Polyarthralgias not explained by OA (can be subtle)
- In interim for joint pain/swelling:
o Mild- moderate symptoms: NSAIDs (Ex. Naproxen 500 mg Q 12 hours) OR low dose pred (5-10 mg)
o Mod-severe symptoms: Prednisone taper (Ex. 20 mg X 7 days, drop by 5 mg weekly)
ACR/ELAR Classification criteria
A score of 6 points or more indicates a high likelihood of rheumatoid arthritis with a sensitivity of 82% and specificity of 61%. CRP, C-reactive protein; ESR, erythrocyte sedimentation rate.
Role of PC in RA
NSAIDS & Steroids until rheumatology appointment. ACR – patients should be seen within 6 weeks.
In a patient with inflammatory arthritis, the presence of a rheumatoid factor and/or anti-citrullinated protein antibody, elevated C-reactive protein level, or elevated erythrocyte sedimentation rate is consistent with a diagnosis of rheumatoid arthritis
Psoriatic Arthritis and manifestations
- Associated with the skin/nail condition
- Usually seronegative
- Asymmetrical oligoarthritis or symmetrical polyarthritis (similar to RA)
- Enthesitis, Dactylitis, & Tenosynovitis
- DIPs often affected,
- May affect the spine (sacrolitis/spondylitis)
- See inflammation in the surrounding joint structures (tendons, ligaments, attachment sites, bursa) more so than with Rheumatoid Arthritis
- Characteristic “sausage digits” associated with psoriatic. DIPs rarely involved in other arthopathies
Dactylitis
Psoriatic Arthritis
When to refer?
- Patient with hx or possible hx of psoriasis and new joint symptoms (including low back pain/stiffness)
- Patient with any clear inflammatory joint symptoms
Ankylosing Spondylitis manifestations, joints involved, lab, and treatment
4/5 following features:
* < 40 yrs at onset
* Insidious onset
* Improves with exercise
* No improvement with rest
* Pain at night, improves with awakening
- RA does not affect the back (occasionally the C-spine)
- Inflammatory back arthritis, with extraspinal involvement as well (peripheral joints, periarticular inflammation) causing bones in spine to fuse
- May not see in person w/hx of arthritis/normal ROS
- HLA B 27 +
- Eye involvement
- IBD/IBS
- Treated with biologics, NSAIDs
Polymalgia Rheumatica definition
- Inflammatory rheumatic condition causing aching/stiffness in the neck, shoulders, hip girdle.
- Areas of pain usually in upper body from BL shoulders down to nipple line and area over shorts
- Cause is unknown.
PMR symptoms and RFs
- Symptoms arise from G-H joint synovitis, biceps tenosynovitis, subacrombial bursitis, along with hip synovitis/bursitis.
- Affects joint bursa, not muscle.
- Age at least 50 (usually >70 years old)
- F > M
PMR Pt Hx
- Acute onset of pain lasting weeks in 2 or more “axial” areas, including neck, shoulder, pelvic girdle
- Morning stiffness for 1+ hours
PMR red flag?
What to ask?
Tx?
GCA
* Ask the questions: visual changes? jaw claudication (pain with chewing)? scalp tenderness? H/A?
* Treat with high dose steroids required to prevent blindness if GCA is suspected, may need temporal artery biopsy to diagnose
* ½ of ppl with GCA get PMR (~ 10 % of those with PMR thought to develop GCA)
* They are different manifestations of the same disease
PMR treatment
- Management: Prednisone, 15-20 mg/day, taper to lowest tolerated dose, ideal duration uncertain (avg: 2.5 yrs) but discontinue as soon as possible
- Common taper schedule: drop by 2.5 mg Q 2-4 weeks, then once they reach 10 mg QD, taper by 1 mg Q 2-4 weeks
- Change as tolerated by patient
- Prednisone not helping, it’s something else
- Failed taper? Methotrexate or Plaquenil may be used if a patient has failed 2 attempts to taper so increasing to lowest effective dose, then add DMARD before performing a rapid taper (refer if this occurs)
- Monitor ESR/CRP along with symptoms during tapering
- Relapses do occur
PMR when to refer?
- A PCP could manage this
- BUT many refer, because of the risk for relapse, risk for GCA development, the need for follow up on bone health, the possibility of misdiagnosis
- Sometimes it appears to be PMR, but can be a seronegative RA
What implications to think of when prescribing prednisone?
- Diabetes
- Osteoporosis
- HTN
- Infection Risks
- Weight gain
- Risk for GI bleeds
- NSAID avoidance
Monitor and address!
Systemic Lupus Erythematosus
- Multi system autoimmune disease, can affect any organ
- Diverse signs and symptoms which change over time, vary from person to person
- Clinical manifestations mediated by antibody formation and the creation of immune complexes, which can destroy tissue
ACR SLE criteria
How many to DX?
Need 4 out of 11 of these to DX
* Malar Rash
* Photosensitivity
* Discoid Rash
* Oral Ulcers
* Arthritis
* Serositis (Pleuritis or Pericarditis)
* Renal Disease (proteinuria, cell casts) serious complication
* Neurologic disorder (seizures or psychosis)
* Hematologic disorder (Hemolytic anemia, Lymphopenia, Leukopenia, Thrombocytopenia)
* ANA (+)
* Antibodies (Anti-DNA, Anti-Sm, Anti-Phospholipids)
SLE characteristics and symptoms
Constitutional s/s + Demographics
Constitutional Symptoms:
* Fatigue, fevers, and weight loss may dominate the clinical picture
Demographic Characteristics:
* Overwhelming female predominance
* Approx. 9F : 1M
* Typical onset during the reproductive years
* Strong minority representation
SLE triggers
- Recent sun exposure
- Emotional stress
- Infection
- Certain drug i.e. sulfonamides
- Surgery
Autoantibody labs and clincal features
All pts w/Lupus will have ANA (+)
SLE treatments
- SUN BLOCK! – Photosensitivity
- NSAID’s - Pain relief for arthralgias & serositis
- Caution with kidney involvement
- Plaquenil (Hydroxychloroquine) – Skin and joint manifestations (anyone w/lupus most likely on this)
- Well tolerated good safety profile, retinal toxicity
- Corticosteroids – Topical
- For skin rashes, but should not be used on the face because of the risk of skin atrophy
- Corticosteroids – Systemic Steroids
- Low dose – skin rashes and joint involvement
- Moderate dose – more aggressive skin disease, serositis and hematologic abnormalities
- High dose – Nephritis, Cerebritis, Vasculitis and life threatening hematologic abnormalities
- Immunosuppressives:
- Mycophenalate Mofetil (Cellcept)
- Methotrexate
- Azathioprine (Imuran)
- Cytoxan
- Benlysta (relatively new treatment)- biologic
Undifferentiated CT disease
What is it, DX, tx
- Some serologic and clinical characteristics of a systemic inflammatory disease, without meeting the criteria for a single disease
- May develop into a clear-cut diagnosis, or not
- May still treat with Plaquenil, treat symptoms
- Important to have routine monitoring of signs, symptoms, bloodwork
When to order ANA (guideline)
- Don’t order unless true suspicion, because it can point you in the wrong direction diagnostically
- A + ANA of 1:40 or 1:80 with otherwise benign workup is not an indication for a rheumatology referral…this can make patients panic.
Other systemic Autoimmune diseases
* Scleroderma/Systemic Sclerosis
* Sjodren’s disease
* Relapsing Polychrondritis
Scleroderma/Systemic Sclerosis
* May be limited to skin or systemic
* Thickening and fibrosis of the skin and other soft tissues
Sjogren’s Disease
* + sicca symptoms
* High incidence of lymphoma
Relapsing Polychondritis
* Inflammation affecting the cartilage
* Can affect respiratory tract, ears, eyes, joints
* May be misdiagnosed initially as cellulitis
Infectious arthritis
What is it
What to ask in Hx/HPI
Examples
- New onset mono or poly-arthritis. Condiser infx.
- Ask: Fever, chills, Immunosuppressed, sick contacts, hx of infection, travel, exposures, IV drug use, occupation, STI risk
Examples:
* Parvovirus (5ths disease)
* Lyme Disease
* Gonoccocal Arthritis
* Reiter’s Syndrome (reactive arthritis)
* Hepatitis B/C
* Septic Joint
Fibromyalgia
- Chronic, widespread pain accompanied often by sleep disturbance, fatigue, psychiatric symptoms, and other somatic complaints (GI)
- Chronic pain and fatigue – can often be labeled as a “fake disease”
- A disorder of pain regulation, sensitization (processing)
- Many rheum patients have fibromyalgia as well
- We often refer back to PCP for care, or not, depending on the patient/provider
NOT CURABLE
Diagnostic algorithm for fibromyalgia
Who is fibromyalgia more common in?
Six times more common in women in reports from specialty clinics, although the female predominance is not as striking in the community and when using survey criteria that do not require a tender point examination
Fibromyalgia symptoms and goal of management
- Fatigue and disrupted sleep
- Cognitive disturbance
- Multiple somatic and psychiatric symptoms
- reducing the major symptoms of this disorder and employs a variety of both nonpharmacologic and pharmacologic therapies, which are often provided in combination in a stepwise approach
Fibromyalgia Patient treatment
- Reassuring them that their pain is REAL
- Sleep Hygiene
- Daily Exercise
- Medications
- Gabapentin/Lyrica
- SSRI/SNRI (Duloxetine)
- Sleep aids: Cyclobenzaprine
- I often describe it to the patient as what is thought to be a dysregulation in how their body perceives pain signals, and so the pain is 100% real, but they are not in any danger of joint damage, etc.
- Exercise is most effective – 1st line over
- Avoid opioids!
- Amitriptyline or cyclobenzaprine followed by duloxetine or milnaciprin, pregabalin or a combination of the above
- Amitriptyline 5-10 mg at QHS, on beers list
- Cyclobenzaprine
Primary Care for Rheum patients
Immunosuppressed patients:
* At risk for infections, consult rheum when they do have an infection (may hold drug)
* Up to date on vaccines (no live vaccines while on biologic therapy)
Long term prednisone patients:
* Diabetes control/HTN control
* Bone health, osteoporosis (may need endocrine referral)
When in doubt, contact rheumatologist
We always try to address these things during out visits as well, but often the PCP is already ahead of us.
Differential diagnosis of fibromyalgia
Fibromyalgia patient education
- Educate on symptom management
- Reassurance that fibromyalgia is a real disease
- There is no cure for fibromyalgia; chronic but possible to live a functional life
- The most effective treatments are non-pharmacologic (sleep, exercise, stress reduction)
- There is a lot of misinformation on the internet; use caution
- Acknowledge patients’ beliefs (e.g. underlying persistent infection)
- Fibromyalgia as a central pain syndrome
- Important to treat comorbid conditions (IBS, headache, psychiatric or mood disorders)
Stepwise fibroyalgia therapy
Overall PC referral guidelines for rheumatic diseases
- Rheumatologists provide care for patients with rheumatic disease in a cost-efficient and evidence-based approach that is tailored to a patient’s circumstances and preferences.
- According to the CDC, rheumatic diseases remain the number one cause of disability in adults in the United States
- Early diagnosis and intervention as well as prevention efforts are important to minimize the impact of short- and long-term morbidity and mortality.
- Rheumatologists provide a key role in the non-surgical treatment of osteoarthritis, soft tissue rheumatism, back pain, and other aspects of musculoskeletal health.
- Particularly, attention is paid to care of the geriatric patient who is may be unable or does not wish to have extensive surgical procedures for treatment of osteoarthritis, spinal stenosis as well as other conditions
Gout
- Chronic inflammatory arthritis characterized by sudden, severe attacks of pain, redness, and tenderness in joints. Big toe most common site - known as Podagra.
- Common pain areas: in joints, ankle, foot, knee, or toe
- Pain and inflammation occur when too much uric acid crystallizes and deposits in the joints.
- M > W until after menopause
Gout clinical manifestations + PE
- Attacks can come suddenly, often at night.
Case notes
- The redness now is spreading over the top of his foot
- Has tried heat, ice, elevation, OTC NSAIDS, Tylenol, and his wife’s Keflex that she had left over
- He has this same issue 10 years ago in the same toe. He was put on medication at that time but he does not remember what he was given
- He has not been able to wear a shoe for the last 3 days, any touch makes the pain much worse
- Very fatigued due to lack of sleep
PE
* Exam unremarkable except for red, inflamed left great toe focused at MTP joint, with redness extending across top of foot.
* Redness does not extend up the leg.
* Patient will not allow you to touch the foot given his degree of discomfort
Gout diagnostics
What would you see?
CBC, CMP, Lipid profile, ESR/CRP, Lyme, Hgb A1c, BUN/Cr, Urate level
- elevated WBC, ESR, CRP during a flare, Urate can be high, normal or low.
- Should repeat the urate level 2 weeks after symptom resolution - most accurate time to assess baseline
- Elevated serum urate >6.8 mg/dL can help to support the diagnosis.
- Hyperuricemia (> 6.8 mg/dL) is helpful in the clinical diagnosis, but does not alone confirm the diagnosis.
Gout lifestyle modifications
What kind of diet?
- Consider modifications of diet, exercise, and decreased intake of alcohol and fatty foods to help minimize the frequency of attacks.
- Avoid fasting or rapid weight loss diets as this can increase uric acid levels in the blood
- Avoid low-carb diets that are high in protein and fat -> increase the risk of hyperuricemia and exacerbate gout
Gout tx in acute attack and onset
- anti-inflammatory medications can help relieve pain and shorten the length of the attack. Important to begin treatment as soon as symptoms begin. Treatment can range from 5 days to several weeks.
- When patients first develop gout there may be months or years between episodes. When uric acid levels are not controlled, episodes become more frequent and severe.
Gout pharm tx and considerations
- Acetaminophen - Pain management
- Allopurinol (Aloprim, Zyloprim) - Helps reduce production or increase elimination of uric acid.
- Mild - 100 mg/day - increase to 200-300 mg/day
- Doses over 300 mg/day - divide doses
- Max 600-800 mg/day in divided doses
- ***Avoid in patient with dehydration, anemia, low WBC, low platelets, elevated LFTs, CKD stage 4/5
Naproxen, Ibuprofen, Indocin - Relieves pain, decreases inflammation, and reduces fever.
o NSAIDS- most effective when therapy is started w/in 48 hours of symptom onset. Using indocin 50 mg TID or Naproxen 500 mg BID is a reasonable alternative to glucocorticoids Treatment for 5-7 days
- Colchicine - Prevents or counteracts inflammation in joints and tissues.
- 1.2 mg at onset, repeat 0.6 mg 1 hour later (max 1.8mg in 1 hour)
- Prednisolone taper when NSAIDS/Colchicine contraindicated
- ORAL Glucocorticoids - recommended for those patients who cannot take NSAIDS or chochicine or with patients with renal insufficiency
- PO - 30-40 mg QD until flare resolution begins then taper over 7-10 days**
Urate lowering therapy considerations
- Should not start urate-lowering therapy in patients with asymptomatic hyperuricemia or gout with rare attacks (1 flare/year)
- American College of Rheumatology guidelines for starting ULT:
- Frequent or disabling gout flares (>2/year)
- Gout with CKD (stage 3 or higher)
- Tophus diagnosis on PE or imaging
- Past urolithiasis
- Start at low dose to monitor SE and response to treatment
- Titrate dose every 2-6 weeks to achieve serum urate levels of 6mg/dl, or 5mg/dl in patients with tophi
- Tophus - is deposit of crystalline uric acid at the surface of the joints or in the skin or cartilage, - Tophi look like swollen bulbous growths on joints just under the skin
- Can be treated with urate lowering drugs (allopurinol) or surgical removal
Other tx considerations for gout
- Xanthine oxidase inhibitors (XOI) - works by inhibiting uric acid synthesis. Meds include allopurinol and febuxostat.
- Allopurinol is recommended first line
- Monitor LFTs, renal and blood counts
- Initial dose 100 mg daily in patients with CrCl>60 ml/minute
- Dietary modifications - limit animal protein to no more than 6 oz of lean meat, poultry or fish per day
- Avoid ETOH or use in moderation - ETOH increases the risk of hyperuricemia as it interferes with the removal of uric acid from the body
- Drink plenty of fluids - can help to remove uric acid from the body
Allopurinol AEs
Adverse effects allopurinol:
- May precipitate gout flares (colchicine prophylaxis is recommended for 3 months after achieving urate goal in patients)
- rash, leukopenia, thrombocytopenia, diarrhea, and severe cutaneous reactions
- DRESS syndrome - drug reaction with eosinophilia and systemic symptoms is a potentially life threatening reaction
