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Title: Rheumatology

  • Debbie King FNP
  • Nursing 8800

  • Derived from the Greek words "sklerosis," meaning
    hardness, and "derma," meaning skin, scleroderma
    literally means hard skin. Though it is often
    referred to as if it were a single disease,
    scleroderma is really a symptom of a group of
    diseases that involve the abnormal growth of
    connective tissue, which supports the skin and
    internal organs. It is sometimes used, therefore,
    as an umbrella term for these disorders. In some
    forms of scleroderma, hard, tight skin is the
    extent of this abnormal process. In other forms,
    however, the problem goes much deeper, affecting
    blood vessels and internal organs, such as the
    heart, lungs, and kidneys.
  • Scleroderma is called both a rheumatic
    (roo-MA-tik) disease and a connective tissue
    disease. The term rheumatic disease refers to a
    group of conditions characterized by inflammation
    and/or pain in the muscles, joints, or fibrous
    tissue. A connective tissue disease is one that
    affects tissues such as skin, tendons, and

  • Damage to cells lining the wall of small arteries
  • Build up of tough scar like fibrous tissue
  • Overproduction of collagen in connective tissue
  • Can prove degenerative and life threatening

  • FYI
  • Scleroderma is NOT
  • Contagious
  • Infectious
  • Cancerous
  • Malignant
  • And probably not genetic
  • Research indicates that there is a susceptibility
    gene which raises the likelihood of getting
    scleroderma, but by itself does not cause the

Types of Scleroderma
  • Localized
  • Morphia
  • Linear
  • Systemic
  • Limited (CREST)
  • Diffuse

  • How serious
  • Varies
  • Depends on affected area
  • Depends on extent of affection
  • A mild case can become more serious if not
    diagnosed and properly treated by a qualified
  • Prompt treatment may minimize the symptoms and
    lesson the chance of irreversible damage!!!

Course of disease
  • Highly individualized disease
  • Never the same presentation
  • Is life long chronic disease
  • First few years are rocky
  • Will quiet down after first few years
  • May have periods of remission

Limited (CREST)
  • Systemic
  • Effects wide spread
  • Usually does not affect internal organs
  • Has a positive outlook
  • CREST- term not used much anymore, referred to as
    limited cutaneous systemic sclerosis
  • C-calcinosis, R-Raynaud's, E-Esophageal
    dysfunction, S-Sclerodactyly, T-Telangiectasia

Diffuse Scleroderma
  • Systemic
  • Affects skin, connective tissue, organs
  • May be slow or rapid
  • May overlap with other disease
  • e.g.. Mixed connective tissue disease

Signs and Symptoms
  • Skin
  • Pronounced puffiness (usually first)
  • Tightening
  • Discoloration
  • Thickening
  • Numbness
  • Tingling
  • Telangiectasis
  • Calcinosis
  • Loss of hair
  • Decreased sweat glands

Vascular Changes
  • Hands or feet change color- blanching
  • Reddish spots-- telangiectasis
  • Ulcers on fingertips, knuckles, elbows
  • Visible capillaries around nails

Bone and Muscle Changes
  • Arthritis
  • Brittle bones
  • Bone loss
  • Decreased mobility of fingers
  • Muscles near shoulders and hips weaken
  • General weakness

GI Changes and Effects
  • 80 of patients with Scleroderma are affected in
    this body system
  • Decreased esophageal mobility
  • Increased heartburn
  • Difficulty swallowing
  • Constipation
  • Mal-absorption
  • Watery diarrhea

Respiratory Changes
  • This system can be the most severely affected
  • Dry cough
  • SOB
  • Difficulty taking deep breaths

Other General Signs and Complications
  • Weight loss
  • Fatigue
  • Pain
  • Unexplained swelling
  • Internal organ problems
  • Sjögren's syndrome
  • chronic autoimmune disease in which peoples
    white blood cells attack their moisture-producing

Other General Signs and Complications
  • Auto-immune disorder of liver
  • Thyroid dysfunction
  • Nerve damage
  • Impotence- first sign for many men
  • Brain changes in late disease

  • - most commonly accepted theory
  • Unknown inciting event triggers injury probably
    to cells lining the blood vessels. Autoimmune
    reaction leads to stimulation of fibroblasts
    (cells that make collagen). Net result is
    accumulation of collagen and other connective
    tissue components in parts of the body such as
    skin, lungs ECT

  • Who gets scleroderma
  • 300,000 cases in US, 80-100,000 are systemic form
  • overall incidence rate of SSc in the adult
    population of the United States is approximately
    20 per million per year
  • estimated the prevalence of systemic sclerosis in
    the United States to be around 240 cases per 1
    million adults
  • Develops between ages 25-55 (or any age)
  • Women 3-4 times more than men
  • Prognosis worse in men
  • Regional outbreaks
  • Incidence unknown around the world
  • European decent more common
  • Increasing numbers in African Americans
  • Not directly inherited, some scientist feel a
    slight predisposition in families with history of
    rheumatoid disease

General Outlook
  • No cure
  • There are current treatments to minimize
    irreversible damage
  • Does not always shorten life span with proper
    diagnosis and treatments!
  • Localized- good prognosis with normal life span
  • Diffuse- can be acute or rapid, often affects
  • Cost to US alone was over 1.5 billion a year in
    1998 (sorry no current number was found)

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Head and Neck Manifestations
  • 80 have, 30 present with
  • tight skin, thin lips, vertical perioral furrows
  • dermal and subcutaneous inflammatory process
  • edema precedes epidermal atrophy, loss of

  • Head and Neck Manifestations
  • decreased mouth opening
  • initial complaint 19

General Diagnosing
  • History-including symptoms
  • PE including skin, nails, lungs, tendons/joints
  • Labs
  • ANA (Those with scleroderma typically have a
    speckled, nucleolar, or centromere pattern test
  • Anti-scleroderma 70 antibodies(is positive in up
    to 60)
  • FANA-an auto antibody(positive in most all
  • anti-centromere antibody (Present in 60-80 of
    those with limited cutaneous scleroderma and
    strongly associated with CREST)

General Diagnosing
  • Non-Laboratory Tests
  • Lung function tests may be performed when lung
    involvement is suspected.
  • CT (Computed Tomography) scans may be performed
    to evaluate lung damage.
  • X-rays may be done to detect calcium deposits and
    evaluate lungs.
  • Cardiac testing and monitoring may be performed
    when heart involvement is suspected
  • Nail fold capillary microscope exam
  • Skin biopsy or Kidney biopsy
  • Many will first be diagnosed with mixed
    connective tissue disease
  • Refer to Rheumatologist and Dermatologist

More Labs
  • Special Stains and Immunohistochemistry
  • Direct immunofluorescence
  • usually negative29,30
  • Immunoperoxidase techniques
  • reduced number of CD34-positive cells31
  • Other investigations
  • Serum levels of various enzymes and substrates
    involved in sclerotic process3237
  • have been used as indicator of disease activity
  • Antibodies
  • Anti-cytoplasmic antigen Ro/SSA 35 of cases45
  • Antinuclear almost all cases
  • usually speckled or nucleolar pattern40,4648
  • Autoantibodies to Fc receptor
  • 50 of cases49
  • Antineutrophil cytoplasmic of perinuclear type
  • Small number of cases

GI Complications
  • Most common is GERD-sched frequent scopes!
  • Difficulty swallowing
  • Scarring in lower intestine
  • Perforation of bowel
  • Constipation
  • Diarrhea
  • Mal-absorption
  • Fecal incontinence

GI Complications
  • Diverticulitis
  • Watermelon stomach-dilated blood vessels lining
    the walls of the stomach which may bleed
  • Small intestine bacterial overgrowth syndrome
  • Gastroparesis (the retention of food in the

GI Treatments
  • General GERD Treatments
  • Decrease carbonated beverages
  • Decrease caffeine
  • Avoid regular and decaffeinated coffee
  • Walk regularly
  • Remain upright after meals
  • Avoid bedtime snacks
  • Increase head of bed
  • Chew gum
  • No set dietary restrictions except above

GI Treatments
  • GERD
  • Antacids
  • H-2 Blockers
  • Proton Pump Inhibitors
  • Carafate
  • Dilated strictures in esophagus
  • Dilatation

GI Treatments
  • Swallowing
  • GI stimulants e.g. Reglan
  • EES- may improve heartburn
  • Sandostatin- IM, may improve motion of bowel
  • Constipation
  • Bulking agents
  • Softening agents
  • MiraLAX
  • Zelnorm

GI Treatments
  • Small Intestine Dysfunction
  • E.g.-Bacterial overgrowth-Diarrhea
  • 2-3 weeks on then 1-2 weeks off, one of the
  • Tetracycline
  • Ampicillin
  • Flagyl
  • Vanco
  • Cipro
  • Augmentin
  • Zithromax
  • Bactrim DS
  • Some have such severe infections they need
    constant treatment, which leads to other possible

Lung Complications (will occur in the first 5
  • Pulmonary fibrosis
  • Pulmonary HTN
  • Cor pulmonale
  • Interstitial Lung disease- Scleroderma is the
    number one cause

Lung Complications
  • Frequently occurs in systemic, may be seen in
    limited, but will not be seen in localized
  • Is worse in Men and African Americans
  • Patients with positive Scl-70 antibody are at
    highest risk

Lung Tests
  • O2 Sats
  • PFT
  • High resolution CT
  • Bronchoscope with lavage
  • Biopsy
  • Echo
  • Cardio-pulmonary exercise test

Respiratory Treatments
  • First must ID alveolitis
  • Then suppress with
  • Steroids (low doses only-20mg)
  • Immuno-suppressants
  • Pneumovax
  • Flu vax
  • O2 as needed
  • Rarely transplants
  • Remember to treat GERD as well

Respiratory Treatments
  • Pulmonary Arterial HTN
  • Refer to pulmonary HTN center
  • Endothium receptor antagonist
  • Prostaglandin derivatives
  • CA Channel Blockers

  • Seen in 95 of scleroderma patients
  • Only 5 of population with Raynaud's have
  • Treatments
  • CA Channel blockers
  • Angiotensin II receptor blockers
  • Other BP meds
  • Vasodilators
  • Keep warm, Live in warm climate
  • Exercise and Relaxation
  • Topical antibiotics-PRN

Cardiac Complications
  • Fibrosis
  • Rhythm disturbance
  • Congestive heart failure
  • Pericarditis
  • Pulmonary HTN

Cardiac Treatments
  • Same as with other causes for each problem
  • Refer for all cardiac complications

Kidney Complications
  • Increased protein (if seen-do full renal workup)
  • HTN (monitor BP very often)
  • Renal crisis- may be treated with Ace inhibitors
    , Is now used routinely in these patients
  • Malignant HTN-Refer
  • May be fatal
  • May lead to long term dialysis
  • Once treated, rarely reoccurs, if treated early!!

Kidney Complications
  • WARNING Corticosteroids may lead to renal
    crisis. Prednisone or Medrol are used to treat
    several complications such as
  • Pulmonary Fibrosis
  • Pruritus
  • Joint Pain
  • New findings indicate corticosteroids can be
    implicated in precipitating renal crisis
  • If used-monitor closely

Kidney Complications
  • Seen only in diffuse scleroderma
  • Was leading cause of death until 25 years ago for
    these patients
  • First see increase in BUN and then serum
    creatinine (which suggest chronicity), and
    increase protein in urine
  • Seen in patients with rapidly progression skin
  • Those with positive anti-RNA polymerase
    antibodies appear to be a greater risk

  • Skin fibrosis
  • Refer to Derm
  • Treat with immuno-suppressants, but all have
    major side effects
  • Pruritus and Dryness
  • OTC lotions
  • Antihistamines

  • Localized Scleroderma and Systemic
  • Plaquenil
  • Methotrexate
  • Prednisone
  • Phenytoin
  • Potassium P-aminobenzoate
  • D-Penicillamine
  • PUVA

  • Related to
  • GI bleed
  • Fe deficiency
  • Chronic disease
  • Hemolytic
  • Often treated with Procrit-epoetin

Oral, Facial Complications
  • Dry mouth
  • Increased dental caries
  • Widening of periodontal membranes
  • Facial changes are a real possibility for
    patients with both types of Scleroderma
  • 100 with systemic
  • 20 with localized

Oral, Facial Complications
  • These changes are progressive and not static
  • Most functional problems are due to microstomia
    (small mouth)
  • Eating
  • Talking
  • Kissing
  • ECT

Face Skin Changes
  • Occur over several months to years
  • First is edematous which is painless
  • Second is indurative which is thickened dermis
    and thinned epidermal layer which leads to loss
    of wrinkles, hair follicles, sweat glands, and
    sebaceous glands.
  • Third is atopic-thickened dermis becomes thinner
    and tethering occurs with skin more attached to
    sub Q fat, then clusters of dilated and tortuous
    capillaries increase in a butterfly shape

Joint and Tendon Pain
  • Occurs with all systemic cases and can progress
  • Treat as arthritis
  • Stay active
  • Avoid fatigue
  • Avoid cold
  • PT RRN
  • MEDS
  • NSAIDS, Cox-2 Inhibitor, Analgesics, Narcotics

Complication of Reactive Depression
  • Is not a surprising complication
  • Treatment is the same
  • SSRI
  • Tricyclic Antidepressants
  • ECT

Other General Treatments
  • Relaxin to help inhibit production of collagen
    and lessen existing excess collagen. Helpful with
    skin, lungs, hand flexing, and opening the mouth
  • Iloprost-vasodilator, is a analogue of
    prostaglandin. It also blocks certain cytokines,
    immune factors that appear to play an important
    role in the development of scleroderma. Best
    given IV
  • An ACE may save the kidneys, has even been known
    to bring back kidney function
  • Many investigational drugs
  • Stem cell and bone marrow transplants-used in
    early disease only, with limited benefits.

  • Rheumatologist
  • Dermatologist- often make the diagnosis
  • Primary Care- Is you!
  • Will also see everyone else
  • GI
  • Pulmonology
  • Cardiology
  • Nephrology
  • ECT

Significance to Practice
  • Education of providers is necessary
  • Prevention of complications
  • Remember prompt and proper diagnosis and
    treatment by a qualified provider may minimize
    the symptoms of scleroderma and decrease the
    chance of irreversible damage.

Summary http//www.niams.nih.gov/Health_Info/Scler
  • Greatly misdiagnosed
  • Greatly under diagnosed
  • Early treatment slows progression
  • Early treatment slows complication and improves
    quality of life
  • Further research may change the prognoses of this

  • http//www.clinicaladvisor.com/an-infrequent-cause

Sjögren's Syndrome
  • May or not be associated scleroderma
  • General
  • immune mediated
  • destruction of exocrine glands
  • primary
  • sicca syndrome- isolated d/o lacrimal and
    salivary glands
  • secondary
  • sicca complex- assoc. with other CTD

Sjögren's Syndrome
  • General
  • prevalence
  • 1 population
  • 10-15 of RA patients
  • 9 to 1 FgtM
  • onset 40-60 yrs.
  • increased risk of lymphoma
  • perhaps 44 times risk

Sjögren's Syndrome
  • General
  • clinical manifestations
  • Xerophthalmia (dry eye)
  • Keratoconjunctivitis sicca is characterized by
    inadequate tear film protection of the cornea
    because of either inadequate tear production or
    abnormal tear film constitution, which results in
    excessively fast evaporation or premature
    destruction of the tear film
  • Xerostomia (dry mouth)
  • other areas
  • skin, vagina, genitalia, chronic bronchitis, GI
    tract, renal tubules
  • diagnosis
  • minor salivary gland biopsy
  • labs
  • RF and ANA
  • SS-A/Ro 60
  • SS-B/La 30

Sjögren's Syndrome
  • Head and Neck Manifestations
  • 80 c/o xerostomia (dry mouth), most prominent
  • difficulty chewing, dysphagia, taste changes,
    fissures of tongue and lips, increased dental
    caries, oral candidiasis

Sjögren's Syndrome
  • Head and Neck Manifestations
  • salivary gland enlargement
  • xerostomia may be quantified by salivarv flow
    rate testing

Sjögren's Syndrome
  • Dry Mouth
  • Treatment
  • Pilocarpine
  • is a drug which can stimulate salivary glands to
    make more saliva.
  • clotrimazole/nystatin
  • close dental supervision
  • surveillance for malignancy

Sjögren's Syndrome
  • eye complaints
  • dryness, burning, itching, foreign body sensation
  • keratoconjunctivitis sicca
  • corneal abrasions - rose bengal staining

Sjögren's Syndrome
  • Treatment
  • symptomatic
  • oral fluid intake
  • saliva substitutes
  • artificial tears
  • avoid
  • decongestants
  • antihistamines
  • diuretics
  • anticholinergic

Rheumatoid Arthritis
  • A chronic, progressive, systemic inflammatory
    process that primarily affects the synovial
  • Joints are destroyed over a long course of
  • Deformities are common
  • Leads to emotional and physical trauma

Rheumatoid Arthritis
  • Patho
  • Destroys the joints in two ways
  • Proteolytic enzymes called proteases digest the
    tissue components of affected joints
  • Due to locally released antigens that cause the
    inflammatory cascade
  • They may be autoimmune substances
  • Invasive rheumatoid pannus destroy joints
  • A creation of inflamed synovium.
  • Consists of granulated vascular tissue extending
    from the vascular bed into the joint

Rheumatoid Arthritis
  • Patho continued
  • Also with in the synovial fluid, an inflammatory
    response occurs. Neutrophils infiltrate, and
    secrete a host of proteolytic enzymes in the
    fluid, as well as cytokines and chemotactic
    factors. This accumulation of joint fluid
    distends the joint capsule which leads to pain
  • Aspiration may lead to instant relief

Rheumatoid Arthritis
  • Differential
  • Connective tissue diseases are the differential
  • Osteoarthritis, gout, chronic lyme disease,
    Lupus, parvovirus, polymyalgia rheumatica,
    Sjögren's, sarcoidosis
  • Osteo usually does not affect wrists, fingers
  • Gout is revealed with synovial aspirate of urate
  • Chronic lyme often involves a singular joint and
    have positive serologic markers
  • Lupus arthritic changes are usually not deforming
  • Parvo rash is different than RA
  • Polymyalgia rheumatica are RF negative

Rheumatoid Arthritis
  • Subjective
  • Early
  • Malaise, diffuse arthritis, weight loss,
    anorexia, fever, neuropathic pain in extremities,
    painful eyes and chest pain with deep breaths
  • Classic symptoms
  • Awakens in pain that gets better as the day goes
  • Swelling present in the am and decreases
    throughout the day
  • Pain and swelling go down with moderate activity
  • Late
  • Pain and swelling increase that lead to
    diminished activity and this leads to more pain
    and immobility

Rheumatoid Arthritis
  • Objective
  • Peripheral symmetric polyarthritis and morning
    stiffness- history esp. if lasts more than an
  • Physical exam
  • Cardiac rub, pulmonary friction rub, injected
    sclera with scleritis, loss of sensation,
    decreased range of motion, ecchymotic lesions on
    extremities, rheumatoid nodules may be on elbows

Rheumatoid Arthritis
  • Tests
  • Rheumatoid factor RF
  • Which is an IgM that reacts against IgG
  • Gives not only positive and negative results ,
    also gives insight into the severity of the
  • 25 of people with RA have a negative RF
  • ESR and/or CRP
  • Will be elevated
  • CBC
  • To rule out anemia
  • Look for a leukocytosis
  • Platelet count
  • More elevated with more joint inflammation

Rheumatoid Arthritis
  • More TESTS
  • Joint-fluid analysis
  • With RA will show increased WBC/ml and increased
  • X-rays
  • May be normal in early stages
  • Later will reveal bone erosions- 90 of patients
    show erosion with in the first 2 years
  • ANA
  • to help rule out lupus, if positive with low
    titers may be RA, if high titers is lupus

Rheumatoid Arthritis
  • Management
  • Processes form conservative to aggressive
  • Goal is to reduce pain and inflammation and to
    spare joint function
  • Early on this is possible with out medication
  • Physical and occupation therapies, heat and cold,
    exercise, rest, assertive devices, splints,
    chiropractic adjustment, weight loss, meditation
  • Later on medication is necessary
  • Analgesics, NSAIDS, steroid, GI upset
    treatments, Disease Modifying Anti rheumatic
  • The CRP should be monitored to determine the
    effectiveness of therapy

Rheumatoid Arthritis
  • Medications
  • Analgesics
  • Tylenol, capsaicin cream, aspirin
  • NSAIDs
  • Enteric-coated asa, Sulindac 150 mg BID, Aleve,
    Motrin at 2400mg day
  • May need an H-blocker or a proton pump inhibitors
  • Zantac
  • Prilosec or Nexium
  • Need to monitor liver and kidney function
  • Steroids
  • 7.5mg qd
  • Remember the side effects!!
  • Adrenal insufficiency, hyperglycemia,
    osteoporosis, and skin discoloration
  • Must also give calcium, vitamin D and
    bisphosphonates with prolonged use

Rheumatoid Arthritis
  • Disease-modifying antirheumatic drugs DMARDs -
    are essentially immunosuppressants and include
  • Hydroxychloroquine (Plaquenil
  • Leflunomide (Arava)
  • Cyclosporine (Neoral)
  • Sulfasalzine (Azulfidine)
  • Gold (Ridaura, Solganal, Myochrysine)
  • Methotrexate (Rheumatrex, Trexall)
  • Azathioprine (Imuran)
  • Cyclophosphamide (Cytoxan)
  • Biologics (Actemra, Cimzia, Enbrel, Humira,
    Kineret, Orencia, Remicade, Rituxan, Simponi)
  • Use this class with active disease
  • Treat early with active disease
  • Combo therapy is more effective than mono
  • Side effects include reactivating TB

Rheumatoid Arthritis
  • Older therapies
  • Methotrexate, Imuran, penicillamine, Azulfidine,
  • Methotrexate is most effect, but not used in
    patients over 65, but the risks must not outweigh
    the benefits
  • refer a patient for this drug
  • Cox 2 Inhibitors
  • Only one left in the US is Celebrex
  • Was originally thought to be an amazing class of
    drugs, with no side effects. Until the cardiac
    problems became evident. Never promise
    patients- miracle drugs.

Rheumatoid Arthritis
  • Follow up and referral
  • Routine clinical lab eval and episodic changes in
  • Routine lab every 90 days
  • CBC, platelet count, liver and renal function,
    and fasting glucose
  • Focus recheck visits on the effect of the relief
  • Refer to rheumatologist
  • refer everyone for twice a year visits

Rheumatoid Arthritis
  • Patient education
  • Goals of TX
  • Review the initial management section
  • Address therapeutic and adverse effects of drug
  • Explain this a chronic disease the cover the
    emotional, social, and spiritual sequelae of
    living with this condition
  • Promote self care
  • Educate patient and family to avoid the sick
  • Educate how the caregiver is so important in all
    areas of this condition

Systemic Lupus Erythematosus
  • An inflammatory autoimmune disease
  • Affects many organ systems
  • Spontaneous remissions and exacerbations
    characterize the clinical picture
  • It can be mild, aggressive, or even

Systemic Lupus Erythematosus
  • Epidemiology and cause
  • 40 to 50 cases per 100,000
  • Associated with age, gender, race and genetics
  • 85 are women diagnosed in their 20s or 30s
  • May present as late as 55 years
  • Juvenile cases are not uncommon
  • Descendants of African races are four times more
    likely to develop
  • No cause has been identified, but some triggers
    for exacerbations have been
  • Ultraviolet rays, infections, stress, pregnancy,
    surgery, exposure to cigarette smoke or silica

Systemic Lupus Erythematosus
  • Patho
  • A condition of disordered immunity in which
    mechanisms that normally prevent immune cell
    activation by autoantigens are eliminated
  • Inhibition of suppressor T cells
  • Increase in CD4T helper cells
  • Cytokine production and polyclonal B cell
  • Deregulated intracellular signaling
  • These factors contribute to a significant
    production of autoantibodies that are considered
    a hallmark of the disease

Systemic Lupus Erythematosus
  • Diagnosing
  • must have four of the following
  • arthritis, photosensitivity, oral ulcers, malar
    rash, discoid rash,
  • serositis (inflammation of the pleura or
    pericardia), renal disease, hematologic
    disorders, neurologic disease, Positive ANA,
    immunologic abnormalities

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Systemic Lupus Erythematosus
  • Subjective
  • Complains of malaise, fever, anorexia, weight
    loss, blurred vision, conjunctive swelling,
    sleeplessness, depression. Joints may be
    reported as swollen/painful in the history.
    Shortness of breath and painful inspiration may
    be reported. Vague abdominal problems may be
    reported as well.

Systemic Lupus Erythematosus
  • Objective
  • Skin
  • Malar or Butterfly rash, discoid rash (scarring
    and highly inflammatory lesions), alopecia or
    scalpel exanthems, splinter hemorrhages, redness
    around finger nails, finger tip lesions,
    lymphadenopathy, Raynaud's
  • The malar rash of lupus is red or purplish and
    mildly scaly. Characteristically, it has the
    shape of a butterfly and involves the bridge of
    the nose. Notably, the rash spares the
    naso-labial folds of the face, which contributes
    to its characteristic appearance. It is usually
    macular with sharp edges and not itchy. The rash
    can be transient or progressive with involvement
    of other parts of the facial skin.
  • Discoid lupus lesions are often red, scaly, and
    thick. Usually they do not hurt or itch. Over
    time these lesions can produce scarring and skin
    discoloration (darkly colored and/or lightly
    colored areas). Discoid lesions that occur on the
    scalp may cause hair to fall out. If the lesions
    form scars when they heal, hair loss may be

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Systemic Lupus Erythematosus
  • Musculoskeletal
  • Swollen joints with no pattern, joint pain with
    palpation which is migratory and is the most
    common presenting complaint

Systemic Lupus Erythematosus
  • Objective
  • Neurologic
  • Impaired thought process, peripheral
    paresthesias, decreased deep tendon reflexes
  • Cardiac
  • A systolic murmur, distended jugular veins (with
  • GI
  • Painless or not --oral and nasal lesions,
    subglottic stenosis, right upper quadrant
    tenderness (hepatitis is common), light lower
    quadrant tenderness (right colon enlargement due
    to intestinal vasculitis)

Systemic Lupus Erythematosus
  • Differential Diagnosis
  • Vasculitis, rheumatoid arthritis, scleroderma,
    juvenile RA, chronic active hepatitis, drug
    reactions, drug-induced lupus, polyarteritis.
  • Rule thyroid disease, which could also have been
    caused by the lupus

Systemic Lupus Erythematosus
  • Diagnostic Testing
  • CBC include platelets
  • CMP-checking electrolytes and renal function
  • Serum albumin reduced with nephropathy
  • ANA
  • UA
  • Antibody screens for double stranded DNA
  • Antiphospholipid antibodies
  • Anti-Smith antibodies
  • Single-stranded DNA and nucleoprotein will be
    positive for both Lupus and RA
  • CPK

Labs from the Lupus website
  • Albumin ANA ANCA (antineutrophil cytoplasmic
    antibodies), P-ANCA (perinuclear) C-ANCA
    (cytoplasmic) Anti-Cardiolipin (Anti-Phospholipid)
    Anti-DNA Anti-Ro/SS-A Anti-Sm antibodies Blood
    Urea Nitrogen (BUN) CBC Complement
    studies Cholesterol CPK Creatinine Creatinine
    Clearance Differential Neutrophils, Lymphocytes,
    Monocytes, Eosinophils, Basophils ENA
    (Extractable Nuclear Antigens) Erythrocyte
    Sedimentation Rate (Sed Rate, ESR) Immunoglobulins
    (IG) Iron Rheumatoid Factor White Blood Count
    (WBC, Leukocyte count)

  • Results of labs
  • Anemia or leukopenia or both
  • 90 have an elevated ANA
  • Rising anti-double stranded DNA
  • Rising complement levels
  • Decreasing CH50, C3. C4
  • Elevated ESR and CRP
  • Proteinuria
  • RPR can be falsely positive
  • CPK elevated with muscle involvement

  • Other tests
  • Chest x-ray
  • Renal US
  • Plain films of joints
  • Renal biopsy
  • EEG, echo
  • Ventilation perfusion scans
  • CT of lungs

  • Management
  • Goal of therapy is symptom control and organ
  • Many require very little interventions
  • Joint pain management
  • Corticosteroids
  • Cyclophosphamide
  • Antimalarials
  • Care for fatigue
  • Kidney protection!
  • GERD treatments
  • Pulmonary treatments

Treatments from the Lupus website
  • NSAIDs Antimalarials Corticosteroids Cytotoxics/Im
    munosuppressants CellCept (MMF)
  • New treatments on the horizon Stem Cell
    Transplants Riquent Prasterone Minocycline -
    friend or foe?

  • Follow up and referral
  • See at least 2 times a year if not on meds
  • Check CBC, UA, platelet count, and ANA
  • Depends on how much medical support is required
  • Referral to a Rheumatologist is helpful

  • Education
  • Give national organization info
  • Instruct on the course of the disease
  • Advise a lot of REST
  • Fluids
  • Call with a fever over 101.5
  • Avoid sulfonamide antibiotics
  • Avoid pregnancy until remission for 6 months
  • Prognoses varies with each patient

Giant cell arteritis
  • http//emedicine.medscape.com/article/1201429-over
  • More common in Caucasians than African Americans
  • More common in women
  • Rare under the age of 50
  • 5-27 cases per 100,000 people over 50 in the US
  • is also called temporal arteritis, cranial
    arteritis, and granulomatous arteritis.
  • GCA is a systemic inflammatory vasculitis of
    unknown etiology that affects medium- and
    large-sized arteries.

Giant cell arteritis
  • Can result in a wide variety of systemic,
    neurologic, and ophthalmologic complications.
  • Visual loss is one of the most significant causes
    of morbidity
  • Newly recognized GCA should be considered a true
    neuro-ophthalmic emergency.
  • Prompt treatment with steroids can prevent
    blindness and other vascular sequelae of GCA.15

Giant cell arteritis
  • Criteria for diagnosis
  • Age at onset of 50 years
  • New headache
  • Abnormalities of the temporal arteries
  • Erythrocyte sedimentation rate (ESR) of 50 mm/h
  • Positive results of a temporal artery biopsy
    (vasculitis characterized by a predominance of
    mononuclear infiltrates or granulomas, usually
    with multinucleated giant cells)

Giant cell arteritis
  • Pathophysiology
  • GCA typically involves inflammation of the aortic
    arch and its branches, but almost any artery of
    the body as well as some veins may be affected
    occasionally. The inflammation tends to involve
    the arteries in a segmental or patchy manner,
    although long portions of arteries may be

Polymyalgia rheumatica
  • Polymyalgia rheumatica (PMR) is a clinical
    syndrome characterized by severe aching and
    stiffness in the neck, shoulder girdle, and
    pelvic girdle. It is classified as a rheumatic
    disease, although the etiology is undetermined.

Polymyalgia rheumatica
  • Pathophysiology
  • Polymyalgia rheumatica causes severe pain in the
    proximal muscle groups however, no evidence of
    disease is present at muscle biopsy. Muscle
    strength and electromyographic findings are
    normal. Some evidence suggests the presence of
    cell-mediated injury to the elastic lamina in the
    blood vessels in the affected muscle groups.
  • Polymyalgia rheumatica is closely linked to giant
    cell arteritis (temporal arteritis), but this is
    believed to be a separate disease process.1

Polymyalgia rheumatica
  • Clinical History
  • The patient's history may include the following
  • Pain and stiffness in the proximal muscle groups
    that usually is symmetrical and worse in the
  • Gel phenomenon (stiffness after prolonged
  • Fever (low grade)
  • Weight loss
  • Fatigue
  • Depression
  • No weakness
  • Abrupt onset of symptoms

Polymyalgia rheumatica
  • Frequency in the US
  • One study revealed a prevalence of 1 in 200
    people aged 50 years or older.
  • Mortality/Morbidity
  • Polymyalgia rheumatica is not a life-threatening
    disease, but it does require treatment for 2-4
  • Race
  • Whites are affected more than other ethnic
  • Sex
  • Females are affected twice as often as males.
  • Age
  • Polymyalgia rheumatica usually affects people
    older than 50 years.

Polymyalgia rheumatica
  • Physical
  • The signs and symptoms of polymyalgia rheumatica
    are nonspecific, and objective findings on
    physical examination often are lacking. If
    present, findings may include the following
  • Muscle tenderness
  • Decreased active range of motion of joints
    secondary to pain
  • No muscle atrophy

Polymyalgia rheumatica
  • The following laboratory studies should be
    obtained for suspected polymyalgia rheumatica
  • Erythrocyte sedimentation rate greater than 50
  • Normochromic normocytic anemia in 50 of cases
  • Normal creatinine kinase level
  • Negative finding for rheumatoid factor
  • Mild elevations with liver function test results
  • Mild nonspecific synovitis
  • Negative muscle biopsy findings

Polymyalgia rheumatica
  • Imagining
  • The cause of musculoskeletal symptoms in
    polymyalgia rheumatica (PMR) is not clearly
    defined because joint synovitis may only
    partially explain the diffuse discomfort. MRI
    imaging of the shoulders, hip and extremities of
    patients with PMR has been analyzed. MRI showed
    that subacromial and subdeltoid bursitis of the
    shoulders and iliopectineal bursitis and hip
    synovitis are the predominant and most frequently
    observed lesions in active PMR. The inflammation
    of the bursae associated with glenohumeral
    synovitis, bicipital tenosynovitis and hip
    synovitis may explain the diffuse discomfort and
    morning stiffness.

Polymyalgia rheumatica
  • The goal of therapy is to suppress autoimmune
  • Corticosteroids
  • These agents have anti-inflammatory properties
    and may cause profound and varied metabolic
    effects. They modify the body's immune response
    to diverse stimuli. Based on a systematic
    examination of the peer-reviewed literature,
    which included 30 studies, remission of
    polymyalgia rheumatica seemed to be achieved with
    a 15 mg/d dose of prednisone for most patients. A
    slow tapering of the prednisone, less than 1
    mg/month, was associated with fewer relapses
  • Methotrexate, azathioprine, and other
    immunosuppressive drugs have been used in some
    centers in an effort to limit dosage and duration
    of corticosteroid therapy
  • NSAIDS esp helpful during steroid taper
  • Ensure adequate calcium and vitamin D intake with
    corticosteroid use
  • PT (activity restriction is unnecessary)

  • Epidemiology
  • Estimated prevalence 1.4-2 Male-to-female
    ratio approximately 41 Age of onset typically
    40-60 years in men, seven years later (on
    average) in women rare after menopause

  • Causes and risk factors
  • Urate underexcretion (more common than
    overproduction) may be primary or associated with
    such conditions as renal impairment,
    hypertension, hypothyroidism, or various
    medications (e.g., aspirin). Urate
    overproduction may be primary or associated with
         Dietary factors excessive intake of
    purines, fructose, or alcohol     
    Lymphoma/myeloproliferative disorders, psoriasis,
    high cell-turnover disorders      Medications
    cytotoxic agents or vitamin B12     
    Hypoxanthine-guanine phosphoribo- syltransferase
    deficiency (Lesch-Nyhan syndrome)     
    Metabolic conditions obesity or
    hypertriglyceridemia Hyperuricemia most
    important risk factor      Age at onset of
    first attack inversely related to serum uric acid
    level      Majority of patients with elevated
    serum uric acid levels, however, will not develop
    clinical gout.

  • Documented risk factors in males      Obesity,
    weight gain, and hypertension (metabolic factors)
         High intake of alcohol (especially beer),
    sugars, meats, and seafood      Diuretic use
    (may not be independent of other risk factors,
    such as hypertension and congestive heart
    failure)      High intake of dairy products
    and coffee (more than four cups daily) may be
    protective. History of organ transplantation
    (especially heart or kidney)/use of cyclosporine
    Risk factors for chronic tophaceous gout
    include diuretic use in renal insufficiency/conges
    tive heart failure (especially in women) and long
    history of disease or alcohol abuse.

  • Complications and associated conditions
  • Progressive kidney failure Joint
    erosion/destruction (chronic tophaceous gout)
    Risk for development of type 2 diabetes,
    hypertriglyceridemia, and cardiovascular disease
  • Common triggers for acute event include     
    Infection, acidosis (metabolic)     
    Initiation of chemotherapy, starting/stopping
    allopurinol, diuretic therapy      Trauma,
    surgery, or use of IV contrast media     
    Alcohol use Acute attack typically
    self-limited, with spontaneous resolution in 3-14
    days      Sudden onset of extreme pain,
    tenderness, and joint inflammation      Often
    begins at night or early morning, with pain and
    other symptoms increasing for 24-48 hours     
    90 of initial attacks are monoarticular, most
    commonly the first metatarsophalangeal.
    Subsequent attacks are typically longer, involve
    more joints, and may not resolve without medical
    therapy. Recurrent attacks lead to
    accumulating crystal deposits in joints (chronic
    tophaceous gout), which become stiff and swollen

  • Physical exam
  • Affected joint typically swollen, red, and
    extremely tender Skin may resemble cellulitis,
    with desquamation over inflamed area.
    Characteristics of tophi include     
    Overlying skin is pulled taut.     
    Visible/palpable soft-tissue masses or
    asymptomatic intradermal/subcutaneous nodules
         Typically contain whitish or yellowish

  • Making the diagnosis
  • Demonstration of urate crystals in synovial
    fluid analysis or in tophus by polarized light
    microscopy Presumptive diagnosis can be made
    based on presence of hyperuricemia, documentation
    of risk factors, and suggestive findings on
    history and physical exam. Rule out     
    Septic arthritis      Calcium pyrophosphate
    dihydrate deposition disease (pseudogout)     
    Rheumatoid/psoriatic arthritis      Erosive
    osteoarthritis or sarcoid arthropathy     
    Bacterial cellulitis

  • Testing
  • Synovial fluid analysis/culture needed to
    exclude septic arthritis if patient is febrile
    and has leukocytosis Serum uric acid ( gt6.8
    mg/dL 404 µmol/L is sufficient for crystal
    precipitation level often normal during acute
    attack). X-ray may help confirm. 24-hour
    urine uric acid level as adjunctive test for
         Identifying/excluding overproducers of
    urate      Patients being considered for
    uricosuric therapy Blood culture to rule out

  • Prognosis
  • Risk of recurrence after initial attack 60
    within one year and gt90 within 10 years
    Treatment of acute attack
  • Oral nonsteroidal anti-inflammatory drugs
    (NSAIDs) at maximum dose for one to two weeks
    (drugs of choice for symptomatic relief)
    Colchicine effective but slower to work than
    NSAIDs Corticosteroids, especially in patients
    with contraindications to NSAIDs Rest, ice
    packs, joint elevation (Bed cage may keep
    bedclothes off inflamed joint.) Avoid alcohol
    ensure adequate fluid intake (gt2 L water daily).
    Allopurinol should not be started or
    discontinued during acute attack (avoid sudden
    changes in serum urate levels). Consider
    discontinuation of diuretics.

Foods with estrogenic effects
  • An anti-estrogenic diet is simple and delicious.
    It has three main points Eat down on the food
    chain. Eat foods that are more in tune with our
    genetic makeup such as fruits, vegetables, beans,
    roots, nuts, seeds, eggs, dairy and wild-caught
    fish. Minimize foods treated with chemicals.
    Eat as much organic food as possible, and avoid
    synthetic vitamins. Supplement your diet with
    nutrients that support your hormones. The
    flavones chrysene, apigenin, quercetin, and
    narigenin all inhibit estrogen, as well as the
    indoles indole 3 carbinol, diindolymethane
    (DIM), and indole 3 acetate.
  • Soy,Millet,Barley,Flax seed,Lentils,Kidney
    beans,Lima beans,Rye,Clover,Fennel,Chick peas are
    rich in phytoestrogens, a natural estrogen.

Foods with estrogenic effects
  • Reduce and eliminate ALL beef, pork, lamb, and
    dairy products (they contain an unusable form of
    estrogen for the female body and create estrogen
  • Avoid all caffeine, alcohol, sugars, acid-forming
    foods (red meat, dairy, excess carbohydrates,
    especially refined foods)
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